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  • Print publication year: 2000
  • Online publication date: June 2014

Chapter 19 - Pulmonary vasculitis and pulmonary hemorrhage syndromes



Vasculitis is defined generically as inflammation of a vessel. Hemorrhage is defined generically as the escape of blood from vessels. Were pulmonary vasculitis and pulmonary hemorrhage so directly connected that inflammation of blood vessels led to rupture and then hemorrhage, the classification of the vasculitic and hemorrhagic syndromes would be considerably easier. However, inflamed vessels may leak or not leak; inflammation in vessels may have great or little clinical or pathophysiological significance; and hemorrhage may be due to trauma, vascular anomalies, pulmonary hypertension, or a bleeding diathesis in the absence of inflammation or vascular necrosis.

There are many schemes for classifying vasculitis. One can classify vasculitis by organ involvement, etiology, histology, size of blood vessel, immunological parameters, cellular mediation, or associated systemic disease. Various categorizations are given in Table 1.

In this chapter we separate the large and complex topic into three major groups: antineutrophil cytoplasmic antibody (ANCA)-associated diseases; pulmonary hemorrhage syndromes; and idiopathic vasculitides that rarely affect the lung. Many other entities, including but not limited to pulmonary infections, interstitial lung diseases such as sarcoidosis/necrotizing sarcoid granulomatosis and Langerhans cell histiocytosis, pulmonary hypertension, drugs, transplantation, radiation, systemic processes including connective tissue disease and inflammatory bowel disease, and malignancies, especially lymphoproliferative disorders such as lymphomatoid granulomatosis, cause pulmonary vasculitis and/or hemorrhage. These entities are discussed elsewhere in this text.

McCluskey, RT, Fienberg, R.Vasculitis in primary vasculitides, granulomatoses, and connective tissue diseases. Hum Pathol 1983;14(4):305–15.
Jennette, JC, Falk, RJ.Small-vessel vasculitis. N Engl J Med 1997;337(21):1512–23.
Vuong, PN, Berry, SC.The Pathology of Vessels. France: Springer-Verlag, 2002.
Saleh, A, Stone, JH.Classification and diagnostic criteria in systemic vasculitis. Best Pract Res Clin Rheumatol 2005;19(2):209–21.
Davies, DJ, Moran, JE, Niall, JF, Ryan, GB.Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology?Br Med J (Clin Res Ed) 1982;285(6342):606.
Falk, RJ, Jennette, JC.Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. N Engl J Med 1988;318(25):1651–7.
Tervaert, JW, Goldschmeding, R, Elema, JD, et al. Autoantibodies against myeloid lysosomal enzymes in crescentic glomerulonephritis. Kidney Int 1990;37(2):799–806.
Godman, GC, Churg, J.Wegener's granulomatosis: pathology and review of the literature. AMA Arch Pathol 1954;58(6):533–53.
Falk, RJ, Terrell, RS, Charles, LA, Jennette, JC.Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro. Proc Natl Acad Sci U S A 1990;87(11):4115–9.
Kallenberg, CG.Pathogenesis of ANCA-associated vasculitides. Ann Rheum Dis 2011;70 Suppl 1:i59–63.
Schlieben, DJ, Korbet, SM, Kimura, RE, Schwartz, MM, Lewis, EJ.Pulmonary-renal syndrome in a newborn with placental transmission of ANCAs. Am J Kidney Dis 2005;45(4):758–61.
Jennette, JC, Wilkman, AS, Falk, RJ.Diagnostic predictive value of ANCA serology. Kidney Int 1998;53(3):796–8.
Niles, JL, Pan, GL, Collins, AB, et al. Antigen-specific radioimmunoassays for anti-neutrophil cytoplasmic antibodies in the diagnosis of rapidly progressive glomerulonephritis. J Am Soc Nephrol 1991;2(1):27–36.
Gallicchio, MC, Savige, JA.Detection of anti-myeloperoxidase and anti-elastase antibodies in vasculitides and infections. Clin Exp Immunol 1991;84(2):232–7.
Hagen, EC, Andrassy, K, Csernok, E, et al. Development and standardization of solid phase assays for the detection of anti-neutrophil cytoplasmic antibodies (ANCA). A report on the second phase of an international cooperative study on the standardization of ANCA assays. J Immunol Methods 1996;196(1):1–15.
Hagen, EC, Daha, MR, Hermans, J, et al. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization. Kidney Int 1998;53(3):743–53.
Stone, JH, Talor, M, Stebbing, J, et al. Test characteristics of immunofluorescence and ELISA tests in 856 consecutive patients with possible ANCA-associated conditions. Arthritis Care Res 2000;13(6):424–34.
Choi, HK, Merkel, PA, Walker, AM, Niles, JL.Drug-associated antineutrophil cytoplasmic antibody-positive vasculitis: prevalence among patients with high titers of antimyeloperoxidase antibodies. Arthritis Rheum 2000;43(2):405–13.
Klinger, H.Grenzformen der periarteritis nodosa. Frankfurt Z Path 1931;42:455.
Churg, J, Strauss, L.Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27(2):277–301.
Wegener, F.Über generalisierte, septische Gefasserkrankung. Verh Dtsch Pathol Ges 1936;29:202–9.
Wegener, F.Über eine eigenartiger rhinogene Granulomatose mit besonderer Beiteilingung des Arterien systems und der Nieren. Beitr Pathol Anat 1939;102:36–8.
Wegener, F.Wegener's granulomatosis. Thoughts and observations of a pathologist. Eur Arch Otorhinolaryngol 1990;247(3):133–42.
Carrington, CB, Liebow, A.Limited forms of angiitis and granulomatosis of Wegener's type. Am J Med 1966;41(4):497–527.
Cassan, SM, Coles, DT, Harrison, EG. The concept of limited forms of Wegener's granulomatosis. Am J Med 1970;49(3):366–79.
Mark, EJ, Matsubara, O, Tan-Liu, NS, Fienberg, R.The pulmonary biopsy in the early diagnosis of Wegener's (pathergic) granulomatosis: a study based on 35 open lung biopsies. Hum Pathol 1988;19(9):1065–71.
Travis, WD, Colby, TV, Lombard, C, Carpenter, HA.A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation. Am J Surg Pathol 1990;14(12):1112–25.
Park, YB, Kim, JY, Linton, JA, et al. Clinicopathologic study of Wegener's granulomatosis with special emphasis on early lesions in 10 Korean patients. Yonsei Med J 2001;42(1):46–54.
De Remee, RA, McDonald, TJ, Harrison, EG, Coles, DT.Wegener's granulomatosis. Anatomic correlates, a proposed classification. Mayo Clin Proc 1976;51(12):777–81.
Fauci, AS, Haynes, BF, Katz, P, Wolff, SM.Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98(1):76–85.
Anderson, G, Coles, ET, Crane, M, et al. Wegener's granuloma. A series of 265 British cases seen between 1975 and 1985. A report by a sub-committee of the British Thoracic Society Research Committee. Q J Med 1992;83(302):427–38.
Colby, TV, Fukuoka, J, Ewaskow, SP, Helmers, R, Leslie, KO.Pathologic approach to pulmonary hemorrhage. Ann Diagn Pathol 2001;5(5):309–19.
Fienberg, R, Mark, EJ, Goodman, M, McCluskey, RT, Niles, JL.Correlation of antineutrophil cytoplasmic antibodies with the extrarenal histopathology of Wegener's (pathergic) granulomatosis and related forms of vasculitis. Hum Pathol 1993;24(2):160–8.
Gaudin, PB, Askin, FB, Falk, RJ, Jennette, JC.The pathologic spectrum of pulmonary lesions in patients with anti-neutrophil cytoplasmic autoantibodies specific for anti-proteinase 3 and anti-myeloperoxidase. Am J Clin Pathol 1995;104(1):7–16.
Finkelman, JD, Lee, AS, Hummel, AM, et al. ANCA are detectable in nearly all patients with active severe Wegener's granulomatosis. Am J Med 2007;120(7):643 e9–14.
Gal, AA, Velasquez, A.Antineutrophil cytoplasmic autoantibody in the absence of Wegener's granulomatosis or microscopic polyangiitis: implications for the surgical pathologist. Mod Pathol 2002;15(3):197–204.
Mamianetti, A.[Should Wegener's disease retain its name?]. Medicina (B Aires) 2009;69(6):689–90.
Lefrak, SS, Matteson, EL.Freidrich Wegener: the past and present. Chest 2007;132(6):2065.
Rosen, MJ.Dr. Friedrich Wegener and the ACCP, Revisited. Chest 2007;132(6):1723; discussion -4.
Woywodt, A, Haubitz, M, Haller, H, Matteson, EL.Wegener's granulomatosis. Lancet 2006;367(9519):1362–6.
Jennette, JC.Nomenclature and classification of vasculitis: lessons learned from granulomatosis with polyangiitis (Wegener's granulomatosis). Clin Exp Immunol 2011;164 Suppl 1:7–10.
Falk, RJ, Gross, WL, Guillevin, L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. Arthritis Rheum 2011;63(4):863–4.
Cotch, MF, Hoffman, GS, Yerg, DE, et al. The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum 1996;39(1):87–92.
Mahr, AD. Epidemiological features of Wegener's granulomatosis and microscopic polyangiitis: two diseases or one ‘anti-neutrophil cytoplasm antibodies-associated vasculitis’ entity? APMIS Suppl 2009(127): 41–7.
Koldingsnes, W, Nossent, H.Epidemiology of Wegener's granulomatosis in northern Norway. Arthritis Rheum 2000;43(11):2481–7.
O'Donnell, JL, Stevanovic, VR, Frampton, C, Stamp, LK, Chapman, PT.Wegener's granulomatosis in New Zealand: evidence of a latitude-dependent incidence gradient. Intern Med J 2006;37:242–6.
Hissaria, P, Cai, FZ, Ahern, M, et al. Wegener's granulomatosis: epidemiological and clinical features in a South Australian study. Intern Med J 2008;38(10):776–80.
Gatenby, PA, Lucas, RM, Engelsen, O, Ponsonby, AL, Clements, M.Antineutrophil cytoplasmic antibody-associated vasculitides: could geographic patterns be explained by ambient ultraviolet radiation?Arthritis Rheum 2009;61(10):1417–24.
Lane, SE, Watts, RA, Scott, DG.Seasonal variations in onset of Wegener's granulomatosis: increased in summer?J Rheumatol 2007;34(4):889–90; author reply 90.
Carruthers, DM, Connor, S, Howie, AJ, et al. Percutaneous image-guided biopsy of lung nodules in the assessment of disease activity in Wegener's granulomatosis. Rheumatology (Oxford) 2000;39(7):776–82.
Watts, RA, Scott, DG, Jayne, DR, et al. Renal vasculitis in Japan and the UK – are there differences in epidemiology and clinical phenotype?Nephrol Dial Transplant 2008;23(12):3928–31.
Chen, M, Yu, F, Zhang, Y, Zhao, MH.Clinical [corrected] and pathological characteristics of Chinese patients with antineutrophil cytoplasmic autoantibody associated systemic vasculitides: a study of 426 patients from a single centre. Postgrad Med J 2005;81(961):723–7.
Flores-Suarez, LF, Villa, AR.Spectrum of Wegener granulomatosis in a Mexican population. Ann N Y Acad Sci 2007;1107:400–9.
Fujimoto, S, Watts, RA, Kobayashi, S, et al. Comparison of the epidemiology of anti-neutrophil cytoplasmic antibody-associated vasculitis between Japan and the UK. Rheumatology (Oxford) 2011;50:1916–20.
Hoffman, GS, Kerr, GS, Leavitt, RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116(6):488–98.
Takala, JH, Kautiainen, H, Malmberg, H, Leirisalo-Repo, M.Wegener's granulomatosis in Finland in 1981–2000: clinical presentation and diagnostic delay. Scand J Rheumatol 2008;37(6):435–8.
Vassallo, M, Shepherd, RJ, Iqbal, P, Feehally, I.Age-related variations in presentation and outcome in Wegener's granulomatosis. J R Coll Physicians Lond 1997;31(4):396–400.
Chen, M, Yu, F, Zhang, Y, Zhao, MH.Antineutrophil cytoplasmic autoantibody-associated vasculitis in older patients. Medicine (Baltimore) 2008;87(4):203–9.
Stone, JH, Merkel, PA, Spiera, R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010;363(3):221–32.
Knight, A, Sandin, S, Askling, J.Risks and relative risks of Wegener's granulomatosis among close relatives of patients with the disease. Arthritis Rheum 2008;58(1):302–7.
Cao, Y, Schmitz, JL, Yang, J, et al. DRB1*15 allele is a risk factor for PR3-ANCA disease in African Americans. J Am Soc Nephrol 2011;22(6):1161–7.
Stassen, PM, Cohen-Tervaert, JW, Lems, SP, et al. HLA-DR4, DR13(6) and the ancestral haplotype A1B8DR3 are associated with ANCA-associated vasculitis and Wegener's granulomatosis. Rheumatology (Oxford) 2009;48(6):622–5.
Wieczorek, S, Holle, JU, Epplen, JT.Recent progress in the genetics of Wegener's granulomatosis and Churg-Strauss syndrome. Curr Opin Rheumatol 2010;22(1):8–14.
Mahr, AD, Edberg, JC, Stone, JH, et al. Alpha-antitrypsin deficiency-related alleles Z and S and the risk of Wegener's granulomatosis. Arthritis Rheum 2010;62(12):3760–7.
Elzouki, AN, Segelmark, M, Wieslander, J, Eriksson, S.Strong link between the alpha 1-antitrypsin PiZ allele and Wegener's granulomatosis. J Intern Med 1994;236(5):543–8.
Segelmark, M, Elzouki, AN, Wieslander, J, Eriksson, S.The PiZ gene of alpha 1-antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis. Kidney Int 1995;48(3):844–50.
Falk, RJ, Hogan, S, Carey, TS, Jennette, JC.Clinical course of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and systemic vasculitis. The Glomerular Disease Collaborative Network. Ann Intern Med 1990;113(9):656–63.
Allenbach, Y, Seror, R, Pagnoux, C, Teixeira, L, Guilpain, P, Guillevin, L.High frequency of venous thromboembolic events in Churg-Strauss syndrome, Wegener's granulomatosis and microscopic polyangiitis but not polyarteritis nodosa: a systematic retrospective study on 1130 patients. Ann Rheum Dis 2009;68(4):564–7.
McDonald, TJ, Neel, HB, DeRemee, RA.Wegener's granulomatosis of the subglottis and the upper portion of the trachea. Ann Otol Rhinol Laryngol 1982;91(6 Pt 1):588–92.
Langford, CA, Sneller, MC, Hallahan, CW, et al. Clinical features and therapeutic management of subglottic stenosis in patients with Wegener's granulomatosis. Arthritis Rheum 1996;39(10):1754–60.
Stein, MG, Gamsu, G, Webb, WR, Stulbarg, MS.Computed tomography of diffuse tracheal stenosis in Wegener granulomatosis. J Comput Assist Tomogr 1986;10(5):868–70.
Screaton, NJ, Sivasothy, P, Flower, CD, Lockwood, CM.Tracheal involvement in Wegener's granulomatosis: evaluation using spiral CT. Clin Radiol 1998;53(11):809–15.
Allende, DS, Booth, CN.Wegener's granulomatosis of the breast: a rare entity with daily clinical relevance. Ann Diagn Pathol 2009;13(5):351–7.
Gunnarsson, R, Omdal, R, Kjellevold, KH, Ellingsen, CL.Wegener's granulomatosis of the prostate gland. Rheumatol Int 2004;24(2):120–2.
Bean, SM, Conner, MG.Wegener's granulomatosis of the uterine cervix: a case report and review of the literature. Int J Gynecol Pathol 2007;26(1):95–8.
Nowack, R, Wachtler, P, Kunz, J, Rasmussen, N.Cranial nerve palsy in Wegener's granulomatosis – lessons from clinical cases. J Neurol 2009;256(3):299–304.
Stone, JH.Limited versus severe Wegener's granulomatosis: baseline data on patients in the Wegener's granulomatosis etanercept trial. Arthritis Rheum 2003;48(8):2299–309.
Sinovich, V, Snow, J.Protracted superficial Wegener's granulomatosis. Australas J Dermatol 2003;44(3):207–14.
Fienberg, R.The protracted superficial phenomenon in pathergic (Wegener's) granulomatosis. Hum Pathol 1981;12(5):458–67.
Ghorbel, B, Dhrif, AS, Miled, M, Houman, MH.Protracted superficial Wegener granulomatosis in a child. Dermatol Online J 2007;13(4):14.
Aberle, DR, Gamsu, G, Lynch, D.Thoracic manifestations of Wegener granulomatosis: diagnosis and course. Radiology 1990;174(3 Pt 1):703–9.
Su, T, Li, HC, Chen, M, et al. Invasive pulmonary aspergillosis in patients with antineutrophil cytoplasmic antibody associated vasculitis. J Clin Rheumatol 2009;15(8):380–2.
Farrelly, CA.Wegener's granulomatosis: a radiological review of the pulmonary manifestations at initial presentation and during relapse. Clin Radiol 1982;33(5):545–51.
Cordier, JF, Valeyre, D, Guillevin, L, Loire, R, Brechot, JM.Pulmonary Wegener's granulomatosis. A clinical and imaging study of 77 cases. Chest 1990;97(4):906–12.
Kuhlman, JE, Hruban, RH, Fishman, EK.Wegener granulomatosis: CT features of parenchymal lung disease. J Comput Assist Tomogr 1991;15(6):948–52.
Maskell, GF, Lockwood, CM, Flower, CD.Computed tomography of the lung in Wegener's granulomatosis. Clin Radiol 1993;48(6):377–80.
Beggs, AD, Hain, SF.F-18 FDG-positron emission tomographic scanning and Wegener's granulomatosis. Clin Nucl Med 2002;27(10):705–6.
Papiris, SA, Manoussakis, MN, Drosos, AA, et al. Imaging of thoracic Wegener's granulomatosis: the computed tomographic appearance. Am J Med 1992;93(5):529–36.
Lee, KS, Kim, TS, Fujimoto, K, et al. Thoracic manifestation of Wegener's granulomatosis: CT findings in 30 patients. Eur Radiol 2003;13(1):43–51.
Reuter, M, Schnabel, A, Wesner, F, et al. Pulmonary Wegener's granulomatosis: correlation between high-resolution CT findings and clinical scoring of disease activity. Chest 1998;114(2):500–6.
Hansell, DM, Bankier, AA, MacMahon, H, et al. Fleischner Society: glossary of terms for thoracic imaging. Radiology 2008;246(3):697–722.
Agarwal, R, Aggarwal, AN, Gupta, D.Another cause of reverse halo sign: Wegener's granulomatosis. Br J Radiol 2007;80(958):849–50.
Primack, SL, Hartman, TE, Lee, KS, Muller, NL.Pulmonary nodules and the CT halo sign. Radiology 1994;190(2):513–5.
Kim, Y, Lee, KS, Jung, KJ, et al. Halo sign on high resolution CT: findings in spectrum of pulmonary diseases with pathologic correlation. J Comput Assist Tomogr 1999;23(4):622–6.
Gaeta, M, Blandino, A, Scribano, E, et al. Computed tomography halo sign in pulmonary nodules: frequency and diagnostic value. J Thorac Imaging 1999;14(2):109–13.
Bicknell, SG, Mason, AC.Wegener's granulomatosis presenting as cryptogenic fibrosing alveolitis on CT. Clin Radiol 2000;55(11):890–1.
Gohel, VK, Dalinka, MK, Israel, HL, Libshitz, HI.The radiological manifestations of Wegener's granulomatosis. Br J Radiol 1973;46(546):427–32.
Lenclud, C, De Vuyst, P, Dupont, E, et al. Wegener's granulomatosis presenting as acute respiratory failure with anti-neutrophil-cytoplasm antibodies. Chest 1989;96(2):345–7.
Stokes, TC, McCann, BG, Rees, RT, Sims, EH, Harrison, BD.Acute fulminating intrapulmonary haemorrhage in Wegener's granulomatosis. Thorax 1982;37(4):315–6.
Travis, WD, Carpenter, HA, Lie, JT.Diffuse pulmonary hemorrhage. An uncommon manifestation of Wegener's granulomatosis. Am J Surg Pathol 1987;11(9):702–8.
Merkel, PA, McCarty, D, Sharma, A, Stone, JR.Case records of the Massachusetts General Hospital. Case 31–2008. A 39-year-old man with chest pain, arthralgias, and a mediastinal mass. N Engl J Med 2008;359(15):1603–14.
Fidder, HH, Apter, S, Langevitz, P, et al. Positive antineutrophil cytoplasmic antibodies-associated vasculitis presenting with hemoptysis and a mediastinal mass. Chest 1999;115(5):1473–5.
George, TM, Cash, JM, Farver, C, et al. Mediastinal mass and hilar adenopathy: rare thoracic manifestations of Wegener's granulomatosis. Arthritis Rheum 1997;40(11):1992–7.
Yoshikawa, Y, Watanabe, T.Pulmonary lesions in Wegener's granulomatosis: a clinicopathologic study of 22 autopsy cases. Hum Pathol 1986;17(4):401–10.
Blennerhassett, JB, Borrie, J, Lichter, I, Taylor, AJ.Localized pulmonary Wegener's granuloma simulating lung cancer: report of four cases. Thorax 1976;31(5):576–84.
Leu, HJ.Pathergic granulomatoses. Pathomorphology of classical and limited form of Wegener's granulomatosis, of lymphomatoid granulomatosis and of eosinophilic granulomatous angiitis. Vasa 1979;8(3):203–12.
Katzenstein, AL, Locke, WK.Solitary lung lesions in Wegener's granulomatosis. Pathologic findings and clinical significance in 25 cases. Am J Surg Pathol 1995;19(5):545–52.
Meneses, M, Deck, C, Donoso, S, Contreras, D, Guzman, L.[Wegener's granulomatosis: clinical and pathological report of 22 cases]. Rev Med Chil 1990;118(4):392–9.
Yoshimura, N, Matsubara, O, Tamura, A, Kasuga, T, Mark, EJ.Wegener's granulomatosis. Associated with diffuse pulmonary hemorrhage. Acta Pathol Jpn 1992;42(9):657–61.
Haworth, SJ, Savage, CO, Carr, D, Hughes, JM, Rees, AJ.Pulmonary haemorrhage complicating Wegener's granulomatosis and microscopic polyarteritis. Br Med J (Clin Res Ed) 1985;290(6484):1775–8.
Mark, EJ, Ramirez, JF.Pulmonary capillaritis and hemorrhage in patients with systemic vasculitis. Arch Pathol Lab Med 1985;109(5):413–8.
Myers, JL, Katzenstein, AL.Wegener's granulomatosis presenting with massive pulmonary hemorrhage and capillaritis. Am J Surg Pathol 1987;11(11):895–8.
Odeh, M, Best, LA, Kerner, H, Bassan, H, Oliven, A.Localized Wegener's granulomatosis relapsing as diffuse massive intra-alveolar hemorrhage. Chest 1993;104(3):955–6.
Peters, JE, Salama, AD, Ind, PW.Wegener's granulomatosis presenting as acute systemic vasculitis following 20 years of limited tracheobronchial disease. J Laryngol Otol 2009;123(12):1375–7.
Yousem, SA.Bronchocentric injury in Wegener's granulomatosis: a report of five cases. Hum Pathol 1991;22(6):535–40.
Uner, AH, Rozum-Slota, B, Katzenstein, AL.Bronchiolitis obliterans-organizing pneumonia (BOOP)-like variant of Wegener's granulomatosis. A clinicopathologic study of 16 cases. Am J Surg Pathol 1996;20(7):794–801.
Crissman, JD, Weiss, MA, Gluckman, J.Midline granuloma syndrome: a clinicopathologic study of 13 patients. Am J Surg Pathol 1982;6(4):335–46.
Douglas, AC, Anderson, TJ, MacDonald, M, Simpson, JG.Midline and Wegener's granulomatosis. Ann N Y Acad Sci 1976;278:618–35.
Lie, JT.Wegener's granulomatosis: histological documentation of common and uncommon manifestations in 216 patients. Vasa 1997;26(4):261–70.
Fienberg, R.Necrotizing granulomatosis and angiitis of the lungs and its relationship to chronic pneumonitis of the cholesterol type. Am J Pathol 1953;29(5):913–31.
Fienberg, R.A morphologic and immunohistologic study of the evolution of the necrotizing palisading granuloma of pathergic (Wegener's) granulomatosis. Sem Respir Med 1989;10:126–32.
Travis, WD, Hoffman, GS, Leavitt, RY, Pass, HI, Fauci, AS.Surgical pathology of the lung in Wegener's granulomatosis. Review of 87 open lung biopsies from 67 patients. Am J Surg Pathol 1991;15(4):315–33.
Schwarz, MI, Brown, KK.Small vessel vasculitis of the lung. Thorax 2000;55(6):502–10.
Blockmans, D, Baeyens, H, Van Loon, R, Lauwers, G, Bobbaers, H.Periaortitis and aortic dissection due to Wegener's granulomatosis. Clin Rheumatol 2000;19(2):161–4.
Schildhaus, HU, Von Netzer, B, Dombrowski, F, Pfeifer, U.Atypical manifestation of a cytoplasmic antineutrophil cytoplasmic antibody (PR3-ANCA)-associated vasculitis with involvement of aortic intima and parietal endocardium. Hum Pathol 2002;33(4):441–5.
Shitrit, D, Shitrit, AB, Starobin, D, et al. Large vessel aneurysms in Wegener's granulomatosis. J Vasc Surg 2002;36(4):856–8.
Wagenaar, SS, van den Bosch, JM, Westermann, CJ, Bosman, HG, Lie, JT.Isolated granulomatous giant cell vasculitis of the pulmonary elastic arteries. Arch Pathol Lab Med 1986;110(10):962–4.
Heffner, DK.Wegener's granulomatosis is not a granulomatous disease. Ann Diagn Pathol 2002;6(5):329–33.
Gindre, D, Peyrol, S, Raccurt, M, et al. Fibrosing vasculitis in Wegener's granulomatosis: ultrastructural and immunohistochemical analysis of the vascular lesions. Virchows Arch 1995;427(4):385–93.
Matsubara, O, Yoshimura, N, Doi, Y, Tamura, A, Mark, EJ.Nasal biopsy in the early diagnosis of Wegener's (pathergic) granulomatosis. Significance of palisading granuloma and leukocytoclastic vasculitis. Virchows Arch 1996;428(1):13–9.
Garner, A.Pathology of ‘pseudotumours’ of the orbit: a review. J Clin Pathol 1973;26(9):639–48.
Sternberg, TH, Reynolds, J, Zeilenga, RH.Pathergic granulomatosis; report of two cases formerly designated Wegener's granulomatosis and malignant granuloma of the face. AMA Arch Derm 1957;75(3):368–79.
Collins, JO, Rosenberg, HA, Warren, P.Disseminated pathergic granulomatosis in a 4-month-old infant: a case report. Pediatrics 1967;40(6):975–9.
Boudes, P.Purely granulomatous Wegener's granulomatosis: a new concept for an old disease. Semin Arthritis Rheum 1990;19(6):365–70.
Binkley, GW.Pathergic granulomatosis. Disseminated type with ulceronodular dermatitis and leukopenia: report of two cases. Arch Dermatol 1963;88:637–43.
Yi, ES, Colby, TV.Wegener's granulomatosis. Semin Diagn Pathol 2001;18(1):34–46.
Mark, EJ, Flieder, DB, Matsubara, O.Treated Wegener's granulomatosis: distinctive pathological findings in the lungs of 20 patients and what they tell us about the natural history of the disease. Hum Pathol 1997;28(4):450–8.
Amin, R.Endobronchial involvement in Wegener's granulomatosis. Postgrad Med J 1983;59(693):452–4.
Hervier, B, Pagnoux, C, Renaudin, K, et al. [Endobronchial stenosis in Wegener's granulomatosis]. Rev Med Interne 2006;27(6):453–7.
Polychronopoulos, VS, Prakash, UB, Golbin, JM, Edell, ES, Specks, U.Airway involvement in Wegener's granulomatosis. Rheum Dis Clin North Am 2007;33(4):755–75, vi.
Strange, C, Halstead, L, Baumann, M, Sahn, SA.Subglottic stenosis in Wegener's granulomatosis: development during cyclophosphamide treatment with response to carbon dioxide laser therapy. Thorax 1990;45(4):300–1.
Daum, TE, Specks, U, Colby, TV, et al. Tracheobronchial involvement in Wegener's granulomatosis. Am J Respir Crit Care Med 1995;151(2 Pt 1):522–6.
French, WO, Civin, WH.Cholesterol pneumonitis and Wegener's granulomatosis. AMA Arch Intern Med 1956;97(6):798–802.
Goulart, RA, Mark, EJ, Rosen, S.Tumefactions as an extravascular manifestation of Wegener's granulomatosis. Am J Surg Pathol 1995;19(2):145–53.
Chua, J, Lim, L.Systemic Wegener's granulomatosis with severe orbito-ocular involvement. Singapore Med J 2008;49(10):e259–62.
Allen, CM, Camisa, C, Salewski, C, Weiland, JE.Wegener's granulomatosis: report of three cases with oral lesions. J Oral Maxillofac Surg 1991;49(3):294–8.
Boudny, C, Nievergelt, H, Braathen, LR, Simon, D.Wegener's granulomatosis presenting as pyoderma gangrenosum. J Dtsch Dermatol Ges 2008;6(6):477–9.
Olson, AL, Schwarz, MI.Diffuse alveolar hemorrhage. Prog Respir Res Basel Karger 2007;36:250–63.
Yousem, SA, Lombard, CM.The eosinophilic variant of Wegener's granulomatosis. Hum Pathol 1988;19(6):682–8.
Potter, MB, Fincher, RK, Finger, DR.Eosinophilia in Wegener's granulomatosis. Chest 1999;116(5):1480–3.
Loane, J, Jaramillo, M, Young, HA, Kerr, KM.Eosinophilic angiocentric fibrosis and Wegener's granulomatosis: a case report and literature review. J Clin Pathol 2001;54(8):640–1.
Bambery, P, Sakhuja, V, Behera, D, Deodhar, SD.Pleural effusions in Wegener's granulomatosis: report of five patients and a brief review of the literature. Scand J Rheumatol 1991;20(6):445–7.
Awasthi, A, Malhotra, P, Gupta, N, Gupta, D, Rajwanshi, A.Pitfalls in the diagnosis of Wegener's granulomatosis on fine needle aspiration cytology. Cytopathology 2007;18(1):8–12.
Fekete, PS, Campbell, WG, Bernardino, ME.Transthoracic needle aspiration biopsy in Wegener's granulomatosis. Morphologic findings in five cases. Acta Cytol 1990;34(2):155–60.
Kaneishi, NK, Howell, LP, Russell, LA, Vogt, PJ, Lie, JT.Fine needle aspiration cytology of pulmonary Wegener's granulomatosis with biopsy correlation. A report of three cases. Acta Cytol 1995;39(6):1094–100.
Nolle, B, Specks, U, Ludemann, J, et al. Anticytoplasmic autoantibodies: their immunodiagnostic value in Wegener granulomatosis. Ann Intern Med 1989;111(1):28–40.
Finkielman, JD, Merkel, PA, Schroeder, D, et al. Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis. Ann Intern Med 2007;147(9):611–9.
Muller Kobold, AC, Kallenberg, CG, Tervaert, JW.Monocyte activation in patients with Wegener's granulomatosis. Ann Rheum Dis 1999;58(4):237–45.
Marinaki, S, Kalsch, AI, Grimminger, P, et al. Persistent T-cell activation and clinical correlations in patients with ANCA-associated systemic vasculitis. Nephrol Dial Transplant 2006;21(7):1825–32.
Popa, ER, Stegeman, CA, Bos, NA, Kallenberg, CG, Tervaert, JW.Differential B- and T-cell activation in Wegener's granulomatosis. J Allergy Clin Immunol 1999;103(5 Pt 1):885–94.
Keogh, KA, Ytterberg, SR, Fervenza, FC, et al. Rituximab for refractory Wegener's granulomatosis: report of a prospective, open-label pilot trial. Am J Respir Crit Care Med 2006;173(2):180–7.
Popa, ER, Tervaert, JW.The relation between Staphylococcus aureus and Wegener's granulomatosis: current knowledge and future directions. Intern Med 2003;42(9):771–80.
Choi, HK, Slot, MC, Pan, G, et al. Evaluation of antineutrophil cytoplasmic antibody seroconversion induced by minocycline, sulfasalazine, or penicillamine. Arthritis Rheum 2000;43(11):2488–92.
Hogan, SL, Satterly, KK, Dooley, MA, et al. Silica exposure in anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and lupus nephritis. J Am Soc Nephrol 2001;12(1):134–42.
Jennette, JC.Pathogenic potential of anti-neutrophil cytoplasmic autoantibodies. Lab Invest 1994;70(2):135–7.
Masutani, K, Tokumoto, M, Nakashima, H, et al. Strong polarization toward Th1 immune response in ANCA-associated glomerulonephritis. Clin Nephrol 2003;59(6):395–405.
Pendergraft, WF, Rudolph, EH, Falk, RJ, et al. Proteinase 3 sidesteps caspases and cleaves p21(Waf1/Cip1/Sdi1) to induce endothelial cell apoptosis. Kidney Int 2004;65(1):75–84.
Berger, SP, Seelen, MA, Hiemstra, PS, et al. Proteinase 3, the major autoantigen of Wegener's granulomatosis, enhances IL-8 production by endothelial cells in vitro. J Am Soc Nephrol 1996;7(5):694–701.
Heeringa, P, Huugen, D, Tervaert, JW.Anti-neutrophil cytoplasmic autoantibodies and leukocyte-endothelial interactions: a sticky connection?Trends Immunol 2005;26(11):561–4.
Reinhold-Keller, E, Beuge, N, Latza, U, et al. An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 2000;43(5):1021–32.
Matteson, EL, Gold, KN, Bloch, DA, Hunder, GG.Long-term survival of patients with Wegener's granulomatosis from the American College of Rheumatology Wegener's Granulomatosis Classification Criteria Cohort. Am J Med 1996;101(2):129–34.
Niles, J.Rituximab in induction therapy for anti-neutrophil cytoplasmic antibody (ANCA) vasculitis. Clin Exp Immunol 2011;164 Suppl 1:27–30.
Hogan, SL, Falk, RJ, Chin, H, et al. Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis. Ann Intern Med 2005;143(9):621–31.
Etanercept plus standard therapy for Wegener's granulomatosis. N Engl J Med 2005;352(4):351–61.
Merkel, PA, Lo, GH, Holbrook, JT, et al. Brief communication: high incidence of venous thrombotic events among patients with Wegener granulomatosis: the Wegener's Clinical Occurrence of Thrombosis (WeCLOT) Study. Ann Intern Med 2005;142(8):620–6.
Launay, D, Souza, R, Guillevin, L, et al. Pulmonary arterial hypertension in ANCA-associated vasculitis. Sarcoidosis Vasc Diffuse Lung Dis 2006;23(3):223–8.
Heijl, C, Harper, L, Flossmann, O, et al. Incidence of malignancy in patients treated for antineutrophil cytoplasm antibody-associated vasculitis: follow-up data from European Vasculitis Study Group clinical trials. Ann Rheum Dis 2011;70(8):1415–21.
Knight, A, Askling, J, Ekbom, A.Cancer incidence in a population-based cohort of patients with Wegener's granulomatosis. Int J Cancer 2002;100(1):82–5.
Faurschou, M, Sorensen, IJ, Mellemkjaer, L, et al. Malignancies in Wegener's granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients. J Rheumatol 2008;35(1):100–5.
Stone, JH, Holbrook, JT, Marriott, MA, et al. Solid malignancies among patients in the Wegener's Granulomatosis Etanercept Trial. Arthritis Rheum 2006;54(5):1608–18.
Jennette, JC, Falk, RJ, Andrassy, K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37(2):187–92.
Bosch, X.Microscopic polyangiitis (microscopic polyarteritis) with late emergence of generalised Wegener's granulomatosis. Ann Rheum Dis 1999;58(10):644–7.
Lane, SE, Watts, R, Scott, DG.Epidemiology of systemic vasculitis. Curr Rheumatol Rep 2005;7(4):270–5.
Guillevin, L, Durand-Gasselin, B, Cevallos, R, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 1999;42(3):421–30.
Villiger, PM, Guillevin, L.Microscopic polyangiitis: clinical presentation. Autoimmun Rev 2010;9(12):812–9.
Jennette, JC, Thomas, DB, Falk, RJ.Microscopic polyangiitis (microscopic polyarteritis). Semin Diagn Pathol 2001;18(1):3–13.
Lauque, D, Cadranel, J, Lazor, R, et al. Microscopic polyangiitis with alveolar hemorrhage. A study of 29 cases and review of the literature. Groupe d'Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM“O”P). Medicine (Baltimore) 2000;79(4):222–33.
Tsuchiya, N, Kobayashi, S, Kawasaki, A, et al. Genetic background of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis: association of HLA-DRB1*0901 with microscopic polyangiitis. J Rheumatol 2003;30(7):1534–40.
Tsuchiya, N, Kobayashi, S, Hashimoto, H, Ozaki, S, Tokunaga, K.Association of HLA-DRB1*0901-DQB1*0303 haplotype with microscopic polyangiitis in Japanese. Genes Immun 2006;7(1):81–4.
Bartfai, Z, Gaede, KI, Russell, KA, et al. Different gender-associated genotype risks of Wegener's granulomatosis and microscopic polyangiitis. Clin Immunol 2003;109(3):330–7.
Oh, JS, Lee, CK, Kim, YG, et al. Clinical features and outcomes of microscopic polyangiitis in Korea. J Korean Med Sci 2009;24(2):269–74.
Serra, A, Cameron, JS, Turner, DR, et al. Vasculitis affecting the kidney: presentation, histopathology and long-term outcome. Q J Med 1984;53(210):181–207.
Savage, CO, Winearls, CG, Evans, DJ, Rees, AJ, Lockwood, CM.Microscopic polyarteritis: presentation, pathology and prognosis. Q J Med 1985;56(220):467–83.
Guillevin, L.Treatment of classic polyarteritis nodosa in 1999. Nephrol Dial Transplant 1999;14(9):2077–9.
Akikusa, B, Kondo, Y, Irabu, N, Yamamoto, S, Saiki, S.Six cases of microscopic polyarteritis exhibiting acute interstitial pneumonia. Pathol Int 1995;45(8):580–8.
Pesci, A, Manganelli, P.Respiratory system involvement in antineutrophil cytoplasmic-associated systemic vasculitides: clinical, pathological, radiological and therapeutic considerations. Drugs R D 2007;8(1):25–42.
Nada, AK, Torres, VE, Ryu, JH, Lie, JT, Holley, KE.Pulmonary fibrosis as an unusual clinical manifestation of a pulmonary-renal vasculitis in elderly patients. Mayo Clin Proc 1990;65(6):847–56.
Mansi, IA, Opran, A, Sondhi, D, Ayinla, R, Rosner, F.Microscopic polyangiitis presenting as idiopathic pulmonary fibrosis: is anti-neutrophilic cytoplasmic antibody testing indicated?Am J Med Sci 2001;321(3):201–2.
Park, J, Banno, S, Sugiura, Y, et al. Microscopic polyangiitis associated with diffuse panbronchiolitis. Intern Med 2004;43(4):331–5.
Uji, M, Matsushita, H, Iwata, S.Microscopic polyangiitis after influenza vaccination. Intern Med 2005;44(8):892–6.
Bansal, PJ, Tobin, MC.Neonatal microscopic polyangiitis secondary to transfer of maternal myeloperoxidase-antineutrophil cytoplasmic antibody resulting in neonatal pulmonary hemorrhage and renal involvement. Ann Allergy Asthma Immunol 2004;93(4):398–401.
Schwarz, MI, Mortenson, RL, Colby, TV, et al. Pulmonary capillaritis. The association with progressive irreversible airflow limitation and hyperinflation. Am Rev Respir Dis 1993;148(2):507–11.
Hatama, S, Kumagai, H, Fujiwara, M, Fujishima, M.A case of microscopic polyarteritis nodosa with interstitial pneumonia successfully treated with steroid pulse therapy and immunosuppressive agents. Ren Fail 1998;20(5):737–46.
Jindal, G, Cruz, SD, Punia, RP, Kaur, R.Refractory anemia as a presenting feature of microscopic polyangitis: a rare vasculitis in children. Indian J Pediatr 2011;78:1287–89.
Doyle, MK, Cuellar, ML.Drug-induced vasculitis. Expert Opin Drug Saf 2003;2(4):401–9.
Nadrous, HF, Yu, AC, Specks, U, Ryu, JH.Pulmonary involvement in Henoch-Schonlein purpura. Mayo Clin Proc 2004;79(9):1151–7.
Usui, K, Ochiai, T, Muto, R, et al. Diffuse pulmonary hemorrhage as a fatal complication of Schonlein-Henoch purpura. J Dermatol 2007;34(10):705–8.
Bombardieri, S, Paoletti, P, Ferri, C, et al. Lung involvement in essential mixed cryoglobulinemia. Am J Med 1979;66(5):748–56.
Gayraud, M, Guillevin, L, le Toumelin, P, et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum 2001;44(3):666–75.
Masi, AT, Hunder, GG, Lie, JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33(8):1094–100.
Lanham, JG, Elkon, KB, Pusey, CD, Hughes, GR.Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984;63(2):65–81.
Mukhtyar, C, Guillevin, L, Cid, MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009;68(3):310–7.
Kallenberg, CG.Churg-Strauss syndrome: just one disease entity?Arthritis Rheum 2005;52(9):2589–93.
Sinico, RA, Bottero, P.Churg-Strauss angiitis. Best Pract Res Clin Rheumatol 2009;23(3):355–66.
Sable-Fourtassou, R, Cohen, P, Mahr, A, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 2005;143(9):632–8.
Gibelin, A, Maldini, C, Mahr, A.Epidemiology and etiology of Wegener granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and Goodpasture syndrome: vasculitides with frequent lung involvement. Semin Respir Crit Care Med 2011;32(3):264–73.
Mahr, A, Guillevin, L, Poissonnet, M, Ayme, S.Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum 2004;51(1):92–9.
Pagnoux, C.Churg-Strauss syndrome: evolving concepts. Discov Med 2010;9(46): 243–52.
Martin, RM, Wilton, LV, Mann, RD.Prevalence of Churg-Strauss syndrome, vasculitis, eosinophilia and associated conditions: retrospective analysis of 58 prescription-event monitoring cohort studies. Pharmacoepidemiol Drug Saf 1999;8(3):179–89.
Harrold, LR, Andrade, SE, Go, AS, et al. Incidence of Churg-Strauss syndrome in asthma drug users: a population-based perspective. J Rheumatol 2005;32(6):1076–80.
Keogh, KA, Specks, U.Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med 2003;115(4):284–90.
Guillevin, L, Pagnoux, C, Mouthon, L.Churg-strauss syndrome. Semin Respir Crit Care Med 2004;25(5):535–45.
Manganelli, P, Giacosa, R, Fietta, P, Zanetti, A, Neri, TM.Familial vasculitides: Churg-Strauss syndrome and Wegener's granulomatosis in 2 first-degree relatives. J Rheumatol 2003;30(3):618–21.
Vaglio, A, Martorana, D, Maggiore, U, et al. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum 2007;56(9):3159–66.
Wieczorek, S, Hellmich, B, Gross, WL, Epplen, JT.Associations of Churg-Strauss syndrome with the HLA-DRB1 locus, and relationship to the genetics of antineutrophil cytoplasmic antibody-associated vasculitides: comment on the article by Vaglio et al. Arthritis Rheum 2008;58(1):329–30.
Wieczorek, S, Hellmich, B, Arning, L, et al. Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener's granulomatosis. Arthritis Rheum 2008;58(6):1839–48.
Chumbley, LC, Harrison, EG, DeRemee, RA.Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc 1977;52(8):477–84.
Cottin, V, Khouatra, C, Dubost, R, Glerant, JC, Cordier, JF.Persistent airflow obstruction in asthma of patients with Churg-Strauss syndrome and long-term follow-up. Allergy 2009;64(4):589–95.
Guillevin, L, Cohen, P, Gayraud, M, et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 1999;78(1):26–37.
Wolf, J, Bergner, R, Mutallib, S, Buggle, F, Grau, AJ.Neurologic complications of Churg-Strauss syndrome – a prospective monocentric study. Eur J Neurol 2010;17(4):582–8.
Shimamoto, C, Hirata, I, Ohshiba, S, Fujiwara, S, Nishio, M.Churg-Strauss syndrome (allergic granulomatous angiitis) with peculiar multiple colonic ulcers. Am J Gastroenterol 1990;85(3):316–9.
Memain, N, De, BM, Guillevin, L, Wechsler, B, Meyer, O.Delayed relapse of Churg-Strauss syndrome manifesting as colon ulcers with mucosal granulomas: 3 cases. J Rheumatol 2002;29(2):388–91.
Suzuki, M, Nabeshima, K, Miyazaki, M, et al. Churg-Strauss syndrome complicated by colon erosion, acalculous cholecystitis and liver abscesses. World J Gastroenterol 2005;11(33):5248–50.
Sinico, RA, Di Toma, L, Maggiore, U, et al. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis 2006;47(5):770–9.
Choi, YH, Im, JG, Han, BK, et al. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings. Chest 2000;117(1):117–24.
Kim, JT, Oh, TY, Chang, WH.Rare case of multiple pulmonary artery aneurysms with caval thrombosis – Hughes-Stovin syndrome. Eur J Cardiothorac Surg 2007;31(3):561–2.
Silva, CI, Müller, NL, Fujimoto, K, et al.. Churg-Strauss syndrome: high resolution CT and pathologic findings. J Thorac Imaging 2005;20(2):74–80.
Worthy, SA, Müller, NL, Hansell, DM, Flower, CD.Churg-Strauss syndrome: the spectrum of pulmonary CT findings in 17 patients. AJR Am J Roentgenol 1998;170(2):297–300.
Johkoh, T, Müller, NL, Akira, M, et al. Eosinophilic lung diseases: diagnostic accuracy of thin-section CT in 111 patients. Radiology 2000;216(3):773–80.
Kim, YK, Lee, KS, Chung, MP, et al. Pulmonary involvement in Churg-Strauss syndrome: an analysis of CT, clinical, and pathologic findings. Eur Radiol 2007;17(12):3157–65.
Erzurum, SC, Underwood, GA, Hamilos, DL, Waldron, JA.Pleural effusion in Churg-Strauss syndrome. Chest 1989;95(6):1357–9.
Degesys, GE, Mintzer, RA, Vrla, RF.Allergic granulomatosis: Churg-Strauss syndrome. AJR Am J Roentgenol 1980;135(6):1281–2.
Roufosse, F, Cogan, E, Goldman, M.The hypereosinophilic syndrome revisited. Annu Rev Med 2003;54:169–84.
Dunogue, B, Pagnoux, C, Guillevin, L.Churg-strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment. Semin Respir Crit Care Med 2011;32(3):298–309.
Wiesner, O, Russell, KA, Lee, AS, et al. Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum 2004;50(9):2954–65.
Hauser, T, Mahr, A, Metzler, C, et al. The leucotriene receptor antagonist montelukast and the risk of Churg-Strauss syndrome: a case-crossover study. Thorax 2008;63(8):677–82.
Harrold, LR, Patterson, MK, Andrade, SE, et al. Asthma drug use and the development of Churg-Strauss syndrome (CSS). Pharmacoepidemiol Drug Saf 2007;16(6):620–6.
Le Gall, C, Pham, S, Vignes, S, et al. Inhaled corticosteroids and Churg-Strauss syndrome: a report of five cases. Eur Respir J 2000;15(5):978–81.
Nachman, PH, Henderson, AG.Pathogenesis of lung vasculitis. Semin Respir Crit Care Med 2011;32(3):245–53.
Kiene, M, Csernok, E, Muller, A, et al. Elevated interleukin-4 and interleukin-13 production by T cell lines from patients with Churg-Strauss syndrome. Arthritis Rheum 2001;44(2):469–73.
Hellmich, B, Csernok, E, Gross, WL.Proinflammatory cytokines and autoimmunity in Churg-Strauss syndrome. Ann N Y Acad Sci 2005;1051:121–31.
Schmitt, WH, Csernok, E, Kobayashi, S, et al. Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage. Arthritis Rheum 1998;41(3):445–52.
Tsurikisawa, N, Saito, H, Tsuburai, T, et al. Differences in regulatory T cells between Churg-Strauss syndrome and chronic eosinophilic pneumonia with asthma. J Allergy Clin Immunol 2008;122(3):610–6.
Jakiela, B, Sanak, M, Szczeklik, W., et al. Both Th2 and Th17 responses are involved in the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol 2011;29(1 Suppl 64):S23–34.
Pepper, RJ, Fabre, MA, Pavesio, C., et al. Rituximab is effective in the treatment of refractory Churg-Strauss syndrome and is associated with diminished T-cell interleukin-5 production. Rheumatology (Oxford) 2008;47(7):1104–5.
Brill, R, Churg, J, Beaver, PC.Allergic granulomatosis associated with visceral larva migrans; case report with autopsy findings on Toxocara infection in a child. Am J Clin Pathol 1953;23(12):1208–15.
Imai, H, Nakamoto, Y, Hirokawa, M, Akihama, T, Miura, AB.Carbamazepine-induced granulomatous necrotizing angiitis with acute renal failure. Nephron 1989;51(3):405–8.
Somogyi, A, Muzes, G, Molnar, J, Tulassay, Z.Drug-related Churg-Strauss syndrome?Adverse Drug React Toxicol Rev 1998;17(2–3):63–74.
Bourgarit, A, Le Toumelin, P, Pagnoux, C, et al. Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients. Medicine (Baltimore) 2005;84(5):323–30.
Guillevin, L, Lhote, F, Gayraud, M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore) 1996;75(1):17–28.
Guillevin, L, Cevallos, R, Durand-Gasselin, B, et al. Treatment of glomerulonephritis in microscopic polyangiitis and Churg-Strauss syndrome. Indications of plasma exchanges, Meta-analysis of 2 randomized studies on 140 patients, 32 with glomerulonephritis. Ann Med Interne (Paris) 1997;148(3):198–204.
Goodpasture, EW.The significance of certain pulmonary lesions in relation to the etiology of influenza. Amer J Med Sci 1919;158:863–70.
Stanton, MC, Tange, JD.Goodpasture's syndrome (pulmonary haemorrhage associated with glomerulonephritis). Australas Ann Med 1958;7(2):132–44.
Scheer, R, Grossman, M.Immune aspects of the glomerulonephritis associated with pulmonary hemorrhageAnn Intern Med 1964;60:1009–21.
Duncan, DA, Drummond, KN, Michael, AF, Vernier, RL.Pulmonary hemorrhage and glomerulonephritis. Report of six cases and study of the renal lesion by the fluorescent antibody technique and electron microscopy. Ann Intern Med 1965;62:920–38.
Sturgill, BC, Westervelt, FB.Immunofluorescence studies in a case of Goodpasture's syndrome. JAMA 1965;194(8):914–6.
Simpson, IJ, Doak, PB, Williams, LC, et al. Plasma exchange in Goodpasture's syndrome. Am J Nephrol 1982;2(6):301–11.
Wilson, CB, Dixon, FJ.Anti-glomerular basement membrane antibody-induced glomerulonephritis. Kidney Int 1973;3(2):74–89.
Bolton, WK.Goodpasture's syndrome. Kidney Int 1996;50(5):1753–66.
Watts, RA, Lane, SE, Bentham, G, Scott, DG.Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum 2000;43(2):414–9.
Shah, MK, Hugghins, SY.Characteristics and outcomes of patients with Goodpasture's syndrome. South Med J 2002;95(12):1411–8.
Ball, JA, Young, KR. Pulmonary manifestations of Goodpasture's syndrome. Antiglomerular basement membrane disease and related disorders. Clin Chest Med 1998;19(4):777–91, ix.
Shah, MK.Outcomes in patients with Goodpasture's syndrome and hydrocarbon exposure. Ren Fail 2002;24(5):545–55.
Bombassei, GJ, Kaplan, AA.The association between hydrocarbon exposure and anti-glomerular basement membrane antibody-mediated disease (Goodpasture's syndrome). Am J Ind Med 1992;21(2):141–53.
Donaghy, M, Rees, AJ.Cigarette smoking and lung haemorrhage in glomerulonephritis caused by autoantibodies to glomerular basement membrane. Lancet 1983;2(8364):1390–3.
Luo, W, Wang, XP, Kashtan, CE, Borza, DB.Alport alloantibodies but not Goodpasture autoantibodies induce murine glomerulonephritis: protection by quinary crosslinks locking cryptic alpha3(IV) collagen autoepitopes in vivo. J Immunol 2010;185(6):3520–8.
Pedchenko, V, Bondar, O, Fogo, AB, et al. Molecular architecture of the Goodpasture autoantigen in anti-GBM nephritis. N Engl J Med 2010;363(4):343–54.
Phelps, RG, Jones, V, Turner, AN, Rees, AJ.Properties of HLA class II molecules divergently associated with Goodpasture's disease. Int Immunol 2000;12(8):1135–43.
Phelps, RG, Rees, AJ.The HLA complex in Goodpasture's disease: a model for analyzing susceptibility to autoimmunity. Kidney Int 1999;56(5):1638–53.
Ooi, JD, Holdsworth, SR, Kitching, AR.Advances in the pathogenesis of Goodpasture's disease: from epitopes to autoantibodies to effector T cells. J Autoimmun 2008;31(3):295–300.
Rees, AJ, Peters, DK, Amos, N, Welsh, KI, Batchelor, JR.The influence of HLA-linked genes on the severity of anti-GBM antibody-mediated nephritis. Kidney Int 1984;26(4):445–50.
Rees, AJ, Peters, DK, Compston, DA, Batchelor, JR.Strong association between HLA-DRW2 and antibody-mediated Goodpasture's syndrome. Lancet 1978;1(8071):966–8.
Perl, SI, Pussell, BA, Charlesworth, JA, Macdonald, GJ, Wolnizer, M.Goodpasture's (anti-GBM) disease and HLA-DRw2. N Engl J Med 1981;305(8):463–4.
Persson, U, Hertz, JM, Carlsson, M, et al. Patients with Goodpasture's disease have two normal COL4A3 alleles encoding the NC1 domain of the type IV collagen alpha 3 chain. Nephrol Dial Transplant 2004;19(8):2030–5.
Luo, H, Chen, M, Cui, Z, et al. The association of HLA-DQB1, -DQA1 and -DPB1 alleles with anti- glomerular basement membrane (GBM) disease in Chinese patients. BMC Nephrol 2011;12(1):21.
Teague, CA, Doak, PB, Simpson, IJ, Rainer, SP, Herdson, PB.Goodpasture's syndrome: an analysis of 29 cases. Kidney Int 1978;13(6):492–504.
Lindic, J, Vizjak, A, Ferluga, D, et al. Clinical outcome of patients with coexistent antineutrophil cytoplasmic antibodies and antibodies against glomerular basement membrane. Ther Apher Dial 2009;13(4):278–81.
Primack, SL, Miller, RR, Müller, NL.Diffuse pulmonary hemorrhage: clinical, pathologic, and imaging features. AJR Am J Roentgenol 1995;164(2):295–300.
Hansell, DM.Small-vessel diseases of the lung: CT-pathologic correlates. Radiology 2002;225(3):639–53.
Lombard, CM, Colby, TV, Elliott, CG.Surgical pathology of the lung in anti-basement membrane antibody-associated Goodpasture's syndrome. Hum Pathol 1989;20(5):445–51.
Hellmark, T, Niles, JL, Collins, AB, McCluskey, RT, Brunmark, C.Comparison of anti-GBM antibodies in sera with or without ANCA. J Am Soc Nephrol 1997;8(3):376–85.
Perez-Arellano, JL, Losa Garcia, JE, Garcia Macias, MC, et al. Hemosiderin-laden macrophages in bronchoalveolar lavage fluid. Acta Cytol 1992;36(1):26–30.
Reynolds, J, Moss, J, Duda, MA, et al. The evolution of crescentic nephritis and alveolar haemorrhage following induction of autoimmunity to glomerular basement membrane in an experimental model of Goodpasture's disease. J Pathol 2003;200(1):118–29.
Donald, KJ, Edwards, RL, McEvoy, JD.Alveolar capillary basement membrane lesions in Goodpasture's syndrome and idiopathic pulmonary hemosiderosis. Am J Med 1975;59(5):642–9.
Damoiseaux, J, Vaessen, M, Knapen, Y, et al. Evaluation of the FIDIS vasculitis multiplex immunoassay for diagnosis and follow-up of ANCA-associated vasculitis and Goodpasture's disease. Ann N Y Acad Sci 2007;1109:454–63.
Sinico, RA, Radice, A, Corace, C, Sabadini, E, Bollini, B.Anti-glomerular basement membrane antibodies in the diagnosis of Goodpasture syndrome: a comparison of different assays. Nephrol Dial Transplant 2006;21(2):397–401.
Calderon, EJ, Wichmann, I, Varela, JM, et al. Presence of glomerular basement membrane (GBM) antibodies in HIV- patients with Pneumocystis carinii pneumonia. Clin Exp Immunol 1997;107(3):448–50.
Salama, AD, Dougan, T, Levy, JB, et al. Goodpasture's disease in the absence of circulating anti-glomerular basement membrane antibodies as detected by standard techniques. Am J Kidney Dis 2002;39(6):1162–7.
Short, AK, Esnault, VL, Lockwood, CM.Anti-neutrophil cytoplasm antibodies and anti-glomerular basement membrane antibodies: two coexisting distinct autoreactivities detectable in patients with rapidly progressive glomerulonephritis. Am J Kidney Dis 1995;26(3):439–45.
Bonsib, SM, Goeken, JA, Kemp, JD, et al. Coexistent anti-neutrophil cytoplasmic antibody and antiglomerular basement membrane antibody associated disease = report of six cases. Mod Pathol 1993;6(5):526–30.
Rutgers, A, Slot, M, van Paassen, P, et al. Coexistence of anti-glomerular basement membrane antibodies and myeloperoxidase-ANCAs in crescentic glomerulonephritis. Am J Kidney Dis 2005;46(2):253–62.
Bosch, X, Mirapeix, E, Font, J, et al. Prognostic implication of anti-neutrophil cytoplasmic autoantibodies with myeloperoxidase specificity in anti-glomerular basement membrane disease. Clin Nephrol 1991;36(3):107–13.
Segelmark, M, Butkowski, R, Wieslander, J.Antigen restriction and IgG subclasses among anti-GBM autoantibodies. Nephrol Dial Transplant 1990;5(12):991–6.
Mercola, KE, Hagadorn, JE.Complement-dependent acute immunologic lung injury in an experimental model resembling Goodpasture's syndrome. Exp Mol Pathol 1973;19(2):230–40.
Zou, J, Hannier, S, Cairns, LS, et al. Healthy individuals have Goodpasture autoantigen-reactive T cells. J Am Soc Nephrol 2008;19(2):396–404.
Leinonen, A, Netzer, KO, Boutaud, A, Gunwar, S, Hudson, BG.Goodpasture antigen: expression of the full-length alpha3(IV) chain of collagen IV and localization of epitopes exclusively to the noncollagenous domain. Kidney Int 1999;55(3):926–35.
Jennette, JC, Falk, RJ.Nosology of primary vasculitis. Curr Opin Rheumatol 2007;19(1):10–6.
Krakower, CA, Greenspon, SA.Localization of the nephrotoxic antigen within the isolated renal glomerulus. AMA Arch Pathol 1951;51(6):629–39.
Zhang, Y, Su, SC, Hecox, DB, et al. Central tolerance regulates B cells reactive with Goodpasture antigen alpha3(IV)NC1 collagen. J Immunol 2008;181(9):6092–100.
Szczech, LA, Anderson, A, Ramers, C, et al. The uncertain significance of anti-glomerular basement membrane antibody among HIV-infected persons with kidney disease. Am J Kidney Dis 2006;48(4):e55–9.
Cui, Z, Wang, HY, Zhao, MH.Natural autoantibodies against glomerular basement membrane exist in normal human sera. Kidney Int 2006;69(5):894–9.
Arends, J, Wu, J, Borillo, J, et al. T cell epitope mimicry in antiglomerular basement membrane disease. J Immunol 2006;176(2):1252–8.
Mesnard, L, Keller, AC, Michel, ML, et al. Invariant natural killer T cells and TGF-beta attenuate anti-GBM glomerulonephritis. J Am Soc Nephrol 2009;20(6):1282–92.
Nakamura, A, Yuasa, T, Ujike, A, et al. Fcgamma receptor IIB-deficient mice develop Goodpasture's syndrome upon immunization with type IV collagen: a novel murine model for autoimmune glomerular basement membrane disease. J Exp Med 2000;191(5):899–906.
Liu, K, Li, QZ, Delgado-Vega, AM, et al. Kallikrein genes are associated with lupus and glomerular basement membrane-specific antibody-induced nephritis in mice and humans. J Clin Invest 2009;119(4):911–23.
Clyne, S, Frederick, C, Arndt, F, Lewis, J, Fogo, AB.Concurrent and discrete clinicopathological presentations of Wegener granulomatosis and anti-glomerular basement membrane disease. Am J Kidney Dis 2009;54(6):1116–20.
Denton, M, Magee, C, Niles, J.Rapidly progressive glomerulonephritis. Ann Intern Med 2002;137(5 Part 1):W1.
Arimura, Y, Minoshima, S, Kamiya, Y, et al. A case of Goodpasture's syndrome associated with anti-myeloperoxidase antibodies. Intern Med 1992;31(2):239–43.
Kalluri, R, Danoff, TM, Okada, H, Neilson, EG.Susceptibility to anti-glomerular basement membrane disease and Goodpasture syndrome is linked to MHC class II genes and the emergence of T cell-mediated immunity in mice. J Clin Invest 1997;100(9):2263–75.
Kalluri, R, Wilson, CB, Weber, M, et al. Identification of the alpha 3 chain of type IV collagen as the common autoantigen in antibasement membrane disease and Goodpasture syndrome. J Am Soc Nephrol 1995;6(4):1178–85.
Yang, R, Hellmark, T, Zhao, J, et al. Antigen and epitope specificity of anti-glomerular basement membrane antibodies in patients with goodpasture disease with or without anti-neutrophil cytoplasmic antibodies. J Am Soc Nephrol 2007;18(4):1338–43.
Yang, R, Hellmark, T, Zhao, J, et al. Levels of epitope-specific autoantibodies correlate with renal damage in anti-GBM disease. Nephrol Dial Transplant 2009;24(6):1838–44.
Savage, CO, Pusey, CD, Bowman, C, Rees, AJ, Lockwood, CM.Antiglomerular basement membrane antibody mediated disease in the British Isles 1980–4. Br Med J (Clin Res Ed) 1986;292(6516):301–4.
Pusey, CD.Anti-glomerular basement membrane disease. Kidney Int 2003;64(4):1535–50.
Sanders, JS, Rutgers, A, Stegeman, CA, Kallenberg, CG.Pulmonary: renal syndrome with a focus on anti-GBM disease. Semin Respir Crit Care Med 2011;32(3):328–34.
Johnson, JP, Whitman, W, Briggs, WA, Wilson, CB.Plasmapheresis and immunosuppressive agents in antibasement membrane antibody-induced Goodpasture's syndrome. Am J Med 1978;64(2):354–9.
Levy, JB, Turner, AN, Rees, AJ, Pusey, CD.Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med 2001;134(11):1033–42.
Jaskowski, TD, Martins, TB, Litwin, CM, Hill, HR.Comparison of four enzyme immunoassays for the detection of immunoglobulin G antibody against glomerular basement membrane. J Clin Lab Anal 2002;16(3):143–5.
Dahlberg, PJ, Kurtz, SB, Donadio, JV, et al. Recurrent Goodpasture's syndrome. Mayo Clin Proc 1978;53(8):533–7.
Keller, F, Nekarda, H.Fatal relapse in Goodpasture's syndrome 3 years after plasma exchange. Respiration 1985;48(1):62–6.
Kluth, DC, Rees, AJ.Anti-glomerular basement membrane disease. J Am Soc Nephrol 1999;10(11):2446–53.
Jayne, DR, Marshall, PD, Jones, SJ, Lockwood, CM.Autoantibodies to GBM and neutrophil cytoplasm in rapidly progressive glomerulonephritis. Kidney Int 1990;37(3):965–70.
Lara, AR, Schwarz, MI.Diffuse alveolar hemorrhage. Chest 2010;137(5):1164–71.
Castaner, E, Alguersuari, A, Gallardo, X, et al. When to suspect pulmonary vasculitis: radiologic and clinical clues. Radiographics 2010;30(1):33–53.
De Lassence, A, Fleury-Feith, J, Escudier, E, et al. Alveolar hemorrhage. Diagnostic criteria and results in 194 immunocompromised hosts. Am J Respir Crit Care Med 1995;151(1):157–63.
Imoto, EM, Lombard, CM, Sachs, DP.Pulmonary capillaritis and hemorrhage. A clue to the diagnosis of systemic necrotizing vasculitis. Chest 1989;96(4):927–8.
Anantham, D, Chan, KP, Chuah, KL, Vathsala, A, Eng, P.Pulmonary capillaritis in IgA nephropathy. South Med J 2007;100(6):605–7.
Jennings, CA, King, TE, Tuder, R, Cherniack, RM, Schwarz, MI.Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis. Am J Respir Crit Care Med 1997;155(3):1101–9.
Garrouste, C, Sailler, L, Astudillo, L, et al. [Fulminant alveolar hemorrhage: evolution of giant cell arteritis to ANCA-positive vasculitis?]. Rev Med Interne 2008;29(3):232–5.
Tsai, MH, Chang, YL, Wu, VC, Chang, CC, Huang, TS.Methimazole-induced pulmonary hemorrhage associated with antimyeloperoxidase-antineutrophil cytoplasmic antibody: a case report. J Formos Med Assoc 2001;100(11):772–5.
Nicolls, MR, Terada, LS, Tuder, RM, Prindiville, SA, Schwarz, MI.Diffuse alveolar hemorrhage with underlying pulmonary capillaritis in the retinoic acid syndrome. Am J Respir Crit Care Med 1998;158(4):1302–5.
Bocanegra, TS, Espinoza, LR, Vasey, FB, Germain, BF.Pulmonary hemorrhage in systemic necrotizing vasculitis associated with hepatitis B. Chest 1981;80(1):102–3.
Franks, TJ, Koss, MN.Pulmonary capillaritis. Curr Opin Pulm Med 2000;6(5):430–5.
Gertner, E.Diffuse alveolar hemorrhage in the antiphospholipid syndrome: spectrum of disease and treatment. J Rheumatol 1999;26(4):805–7.
Gertner, E, Lie, JT.Pulmonary capillaritis, alveolar hemorrhage, and recurrent microvascular thrombosis in primary antiphospholipid syndrome. J Rheumatol 1993;20(7):1224–8.
Deane, KD, West, SG.Antiphospholipid antibodies as a cause of pulmonary capillaritis and diffuse alveolar hemorrhage: a case series and literature review. Semin Arthritis Rheum 2005;35(3):154–65.
Milman, N, Pedersen, FM.Idiopathic pulmonary haemosiderosis. Epidemiology, pathogenic aspects and diagnosis. Respir Med 1998;92(7):902–7.
Airaghi, L, Ciceri, L, Giannini, S, et al. Idiopathic pulmonary hemosiderosis in an adult. Favourable response to azathioprine. Monaldi Arch Chest Dis 2001;56(3):211–3.
Rokitansky, K.Über einige der wichtigsten Krankheiten der Arteien. Denkshr dK Akad d Wissensch 1852;4:49.
Virchow, R.Cellular pathology as based upon physiological and pathological histology. Philadelphia: Lippincott. Quoted in Nuzum JW Jr, Nuzum JW Sr. AMA Arch Intern Med 1954;94:942–55.
Kussmaul, A, Maier, R.Über eine bisher nicht beschreibene eigenthümliche areterienerkrankung (periarteritis nodosa), die mit Morbus Brightii und rapid fortschreitender allgemwiner Muskellähmung eingergeht. Dtsch Arch Klin Med 1866;1:484–517.
Ferrari, E.Über poly-arteritis acute nodosa (sogenannte periarteritis nodosa) und ihre beziehungen zur polymyositis und polyneuritis acuta. Beitr Pathol Anat 1903;34:350–86.
Zeek, PM.Periarteritis nodosa and other forms of necrotizing angiitis. N Engl J Med 1953;248(18):764–72.
Lightfoot, RW, Michel, BA, Bloch, DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum 1990;33(8):1088–93.
Pettigrew, HD, Teuber, SS, Gershwin, ME.Polyarteritis nodosa. Compr Ther 2007;33(3):144–9.
Rose, GA, Spencer, H.Polyarteritis nodosa. Q J Med 1957;26(101):43–81.
Watts, RA, Scott, DG.Classification and epidemiology of the vasculitides. Baillieres Clin Rheumatol 1997;11(2):191–217.
Ebert, EC, Hagspiel, KD, Nagar, M, Schlesinger, N.Gastrointestinal involvement in polyarteritis nodosa. Clin Gastroenterol Hepatol 2008;6(9):960–6.
Janssen, HL, van Zonneveld, M, van Nunen, AB, et al. Polyarteritis nodosa associated with hepatitis B virus infection. The role of antiviral treatment and mutations in the hepatitis B virus genome. Eur J Gastroenterol Hepatol 2004;16(8):801–7.
Arkin, A.A Clinical and pathological study of periarteritis nodosa: a report of five cases, one histologically healed. Am J Pathol 1930;6(4):401–26 5.
Pagnoux, C, Seror, R, Henegar, C, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum 2010;62(2):616–26.
Matsumoto, T, Homma, S, Okada, M, et al. The lung in polyarteritis nodosa: a pathologic study of 10 cases. Hum Pathol 1993;24(7):717–24.
Nick, J, Tuder, R, May, R, Fisher, J.Polyarteritis nodosa with pulmonary vasculitis. Am J Respir Crit Care Med 1996;153(1):450–3.
Bonsib, SM.Polyarteritis nodosa. Semin Diagn Pathol 2001;18(1):14–23.
Lee, YJ, Park, SS, Kim, SY, et al. A case of systemic polyarteritis nodosa involving bronchial artery. Sarcoidosis Vasc Diffuse Lung Dis 2010;27(2):164–8.
Mouthon, L.[Causes and mechanisms of systemic vasculitides]. Rev Prat 2008;58(5):487–91.
Balow, JE.Renal vasculitis. Kidney Int 1985;27(6):954–64.
Frohnert, PP, Sheps, SG.Long-term follow-up study of periarteritis nodosa. Am J Med 1967;43(1):8–14.
Guillevin, L, Lhote, F.Treatment of polyarteritis nodosa and Churg-Strauss syndrome: indications of plasma exchanges. Transfus Sci 1994;15(4):371–88.
Segelmark, M, Selga, D.The challenge of managing patients with polyarteritis nodosa. Curr Opin Rheumatol 2007;19(1):33–8.
Guillevin, L, Pagnoux, C.[Treatment of systemic vasculitides]. Rev Prat 2008;58(5):541–4.
Guillevin, L, Mahr, A, Callard, P, et al. Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore) 2005;84(5):313–22.
Agard, C, Mouthon, L, Mahr, A, Guillevin, L.Microscopic polyangiitis and polyarteritis nodosa: how and when do they start?Arthritis Rheum 2003;49(5):709–15.
Horton, BT, Magath, BT, Brown, GE.Arteritis of temporal vessels; report of 7 cases. Proc Staff Meet Mayo Clin 1937;12:548–53.
Jennings, GH.Arteritis of the temporal vesels. Lancet 1938;1:424.
Hu, Z, Yang, Q, Zeng, S., et al. Giant cell arteritis in China: a prospective investigation. Angiology 2002;53(4):457–63.
Gonzalez, CM, Garcia, PC.Epidemiology of the vasculitides. Rheum Dis Clin North Am 2001;27(4):729–49.
Smith, CA, Fidler, WJ, Pinals, RS.The epidemiology of giant cell arteritis. Report of a ten-year study in Shelby County, Tennessee. Arthritis Rheum 1983;26(10):1214–9.
Boesen, P, Sorensen, SF.Giant cell arteritis, temporal arteritis, and polymyalgia rheumatica in a Danish county. A prospective investigation, 1982–1985. Arthritis Rheum 1987;30(3):294–9.
Salvarani, C, Gabriel, SE, O'Fallon, WM, Hunder, GG.The incidence of giant cell arteritis in Olmsted County, Minnesota: apparent fluctuations in a cyclic pattern. Ann Intern Med 1995;123(3):192–4.
Kobayashi, S, Yano, T, Matsumoto, Y, et al. Clinical and epidemiologic analysis of giant cell (temporal) arteritis from a nationwide survey in 1998 in Japan: the first government-supported nationwide survey. Arthritis Rheum 2003;49(4):594–8.
Smetana, GW, Shmerling, RH.Does this patient have temporal arteritis?JAMA 2002;287(1):92–101.
Salvarani, C, Crowson, CS, O'Fallon, WM, Hunder, GG, Gabriel, SE.Reappraisal of the epidemiology of giant cell arteritis in Olmsted County, Minnesota, over a fifty-year period. Arthritis Rheum 2004;51(2):264–8.
Cimmino, MA, Zaccaria, A.Epidemiology of polymyalgia rheumatica. Clin Exp Rheumatol 2000;18(4 Suppl 20):S9–11.
Duhaut, P, Pinede, L, Demolombe-Rague, S, et al. Giant cell arteritis and cardiovascular risk factors: a multicenter, prospective case-control study. Groupe de Recherche sur l'Arterite a Cellules Geantes. Arthritis Rheum 1998;41(11):1960–5.
Salvarani, C, Casali, B, Boiardi, L, et al. Intercellular adhesion molecule 1 gene polymorphisms in polymyalgia rheumatica/giant cell arteritis: association with disease risk and severity. J Rheumatol 2000;27(5):1215–21.
Gonzalez-Gay, MA, Garcia-Porrua, C, Llorca, J, et al. Visual manifestations of giant cell arteritis. Trends and clinical spectrum in 161 patients. Medicine (Baltimore) 2000;79(5):283–92.
Gonzalez-Gay, MA, Barros, S, Lopez-Diaz, MJ, et al. Giant cell arteritis: disease patterns of clinical presentation in a series of 240 patients. Medicine (Baltimore) 2005;84(5):269–76.
Tato, F, Hoffmann, U.Giant cell arteritis: a systemic vascular disease. Vasc Med 2008;13(2):127–40.
Seo, P, Stone, JH.Large-vessel vasculitis. Arthritis Rheum 2004;51(1):128–39.
Wagenaar, SS, Westermann, CJ, Corrin, B.Giant cell arteritis limited to large elastic pulmonary arteries. Thorax 1981;36(11):876–7.
Okubo, S, Kunieda, T, Ando, M, Nakajima, N, Yutani, C.Idiopathic isolated pulmonary arteritis with chronic cor pulmonale. Chest 1988;94(3):665–6.
Masuda, S, Ishii, T, Asuwa, N, Ishikawa, Y, Kiguchi, H.Isolated pulmonary giant cell vasculitis. Pathol Res Pract 1994;190(11):1095–100; discussion 101–4.
Kerr, KM, Auger, WR, Fedullo, PF, et al. Large vessel pulmonary arteritis mimicking chronic thromboembolic disease. Am J Respir Crit Care Med 1995;152(1):367–73.
Lie, JT.Isolated pulmonary Takayasu arteritis: clinicopathologic characteristics. Mod Pathol 1996;9(5):469–74.
Hayreh, SS, Podhajsky, PA, Raman, R, Zimmerman, B.Giant cell arteritis: validity and reliability of various diagnostic criteria. Am J Ophthalmol 1997;123(3):285–96.
Levine, SM, Hellmann, DB.Giant cell arteritis. Curr Opin Rheumatol 2002;14(1):3–10.
Weyand, CM, Goronzy, JJ.Medium- and large-vessel vasculitis. N Engl J Med 2003;349(2):160–9.
Weyand, CM, Goronzy, JJ.Arterial wall injury in giant cell arteritis. Arthritis Rheum 1999;42(5):844–53.
Lie, JT.Disseminated visceral giant cell arteritis: histopathologic description and differentiation from other granulomatous vasculitides. Am J Clin Pathol 1978;69(3):299–305.
Kagata, Y, Matsubara, O, Ogata, S, Lie, JT, Mark, EJ.Infantile disseminated visceral giant cell arteritis presenting as sudden infant death. Pathol Int 1999;49(3):226–30.
Small, P, Brisson, ML.Wegener's granulomatosis presenting as temporal arteritis. Arthritis Rheum 1991;34(2):220–3.
Nishino, H, DeRemee, RA, Rubino, FA, Parisi, JE.Wegener's granulomatosis associated with vasculitis of the temporal artery: report of five cases. Mayo Clin Proc 1993;68(2):115–21.
Dasgupta, B, Hassan, N.Giant cell arteritis: recent advances and guidelines for management. Clin Exp Rheumatol 2007;25(1 Suppl 44):S62–5.
Leavitt, RY, Fauci, AS.Pulmonary vasculitis. Am Rev Respir Dis 1986;134(1):149–66.
Yamada, I, Shibuya, H, Matsubara, O, et al. Pulmonary artery disease in Takayasu's arteritis: angiographic findings. AJR Am J Roentgenol 1992;159(2):263–9.
Park, JH, Chung, JW, Im, JG, et al. Takayasu arteritis: evaluation of mural changes in the aorta and pulmonary artery with CT angiography. Radiology 1995;196(1):89–93.
Takayasu, M.A case with peculiar changes of the retinal central vessels [in Japanese]. Acta of the Opthalmic Society of Japan 1908;12:554–5.
Numano, F.Introductory remarks for this special issue on Takayasu arteritis. Heart Vessels Suppl 1992;7:3–5.
Numano, F, Okawara, M, Inomata, H, Kobayashi, Y.Takayasu's arteritis. Lancet 2000;356(9234):1023–5.
Shimizu, K, Sano, K.Pulseless disease. Journal of Neuropathology and Clinical Neurology 1951;1:37–47.
Arend, WP, Michel, BA, Bloch, DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990;33(8):1129–34.
Gravanis, MB.Giant cell arteritis and Takayasu aortitis: morphologic, pathogenetic and etiologic factors. Int J Cardiol 2000;75 Suppl 1:S21–33; discussion S5–6.
Moriwaki, R, Noda, M, Yajima, M, Sharma, BK, Numano, F.Clinical manifestations of Takayasu arteritis in India and Japan – new classification of angiographic findings. Angiology 1997;48(5):369–79.
Hall, S, Barr, W, Lie, JT, et al. Takayasu arteritis. A study of 32 North American patients. Medicine (Baltimore) 1985;64(2):89–99.
Salazar, M, Varela, A, Ramirez, LA, et al. Association of HLA-DRB1*1602 and DRB1*1001 with Takayasu arteritis in Colombian mestizos as markers of Amerindian ancestry. Int J Cardiol 2000;75 Suppl 1:S113–6.
Vargas-Alarcon, G, Zuniga, J, Gamboa, R, et al. DNA sequencing of HLA-B alleles in Mexican patients with Takayasu arteritis. Int J Cardiol 2000;75 Suppl 1:S117–22.
Numano, F, Ohta, N, Sasazuki, T.HLA and clinical manifestations in Takayasu disease. Jpn Circ J 1982;46(2):184–9.
Subramanyan, R, Joy, J, Balakrishnan, KG.Natural history of aortoarteritis (Takayasu's disease). Circulation 1989;80(3):429–37.
Kerr, GS, Hallahan, CW, Giordano, J, et al. Takayasu arteritis. Ann Intern Med 1994;120(11):919–29.
Lupi-Herrera, E, Sanchez-Torres, G, Marcushamer, J, et al. Takayasu's arteritis. Clinical study of 107 cases. Am Heart J 1977;93(1):94–103.
Ishikawa, K.Natural history and classification of occlusive thromboaortopathy (Takayasu's disease). Circulation 1978;57(1):27–35.
Sharma, S, Kamalakar, T, Rajani, M, Talwar, KK, Shrivastava, S.The incidence and patterns of pulmonary artery involvement in Takayasu's arteritis. Clin Radiol 1990;42(3):177–81.
Nakabayashi, K, Kurata, N, Nangi, N, Miyake, H, Nagasawa, T.Pulmonary artery involvement as first manifestation in three cases of Takayasu arteritis. Int J Cardiol 1996;54 Suppl:S177–83.
Haas, A, Stiehm, ER.Takayasu's arteritis presenting as pulmonary hypertension. Am J Dis Child 1986;140(4):372–4.
Castellani, M, Vanoli, M, Cali, G, et al. Ventilation-perfusion lung scan for the detection of pulmonary involvement in Takayasu's arteritis. Eur J Nucl Med 2001;28(12):1801–5.
Matsubara, O, Yoshimura, N, Tamura, A, et al. Pathological features of the pulmonary artery in Takayasu arteritis. Heart Vessels Suppl 1992;7:18–25.
Inder, SJ, Bobryshev, YV, Cherian, SM, et al. Immunophenotypic analysis of the aortic wall in Takayasu's arteritis: involvement of lymphocytes, dendritic cells and granulocytes in immuno-inflammatory reactions. Cardiovasc Surg 2000;8(2):141–8.
Noris, M, Daina, E, Gamba, S, Bonazzola, S, Remuzzi, G.Interleukin-6 and RANTES in Takayasu arteritis: a guide for therapeutic decisions?Circulation 1999;100(1):55–60.
Eichhorn, J, Sima, D, Thiele, B, et al. Anti-endothelial cell antibodies in Takayasu arteritis. Circulation 1996;94(10):2396–401.
Seko, Y, Minota, S, Kawasaki, A, et al. Perforin-secreting killer cell infiltration and expression of a 65-kD heat-shock protein in aortic tissue of patients with Takayasu's arteritis. J Clin Invest 1994;93(2):750–8.
Hellmann, DB.Immunopathogenesis, diagnosis, and treatment of giant cell arteritis, temporal arteritis, polymyalgia rheumatica, and Takayasu's arteritis. Curr Opin Rheumatol 1993;5(1):25–32.
Al-Aghbari, K, Al-Motarreb, A, Askar, F.Takayasu's arteritis associated with tuberculosis in a young Yemeni woman. Heart Views 2010;11(3):117–20.
Numano, F.Vasa vasoritis, vasculitis and atherosclerosis. Int J Cardiol 2000;75 Suppl 1:S1–8; discussion S17–9.
Ishikawa, K, Maetani, S.Long-term outcome for 120 Japanese patients with Takayasu's disease. Clinical and statistical analyses of related prognostic factors. Circulation 1994;90(4):1855–60.
Hoffman, GS, Leavitt, RY, Kerr, GS, et al. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate. Arthritis Rheum 1994;37(4):578–82.
Valsakumar, AK, Valappil, UC, Jorapur, V, et al. Role of immunosuppressive therapy on clinical, immunological, and angiographic outcome in active Takayasu's arteritis. J Rheumatol 2003;30(8):1793–8.
Behçet, H.Über rezidivierende, aphtose, durch ein Virus verusachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatologische Wochenschr 1937;105:1152–7.
Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet 1990;335(8697):1078–80.
Erkan, F, Gul, A, Tasali, E.Pulmonary manifestations of Behçet's disease. Thorax 2001;56(7):572–8.
Zouboulis, CC.Epidemiology of Adamantiades-Behçet's disease. Ann Med Interne (Paris) 1999;150(6):488–98.
Yurdakul, S, Gunaydin, I, Tuzun, Y, et al. The prevalence of Behçet's syndrome in a rural area in northern Turkey. J Rheumatol 1988;15(5):820–2.
Yamamoto, S, Toyokawa, H, Matsubara, J.A nation-wide survey of Behçet's disease in Japan. Jpn J Opthalmol 1974;18:282–90.
Hirohata, T, Kuratsune, M, Nomura, A, Jimi, S.Prevalence of Behçet's syndrome in Hawaii. With particular reference to the comparison of the Japanese in Hawaii and Japan. Hawaii Med J 1975;34(7):244–6.
Chamberlain, MA.Behçet's syndrome in 32 patients in Yorkshire. Ann Rheum Dis 1977;36(6):491–9.
O'Duffy, JD.Summary of International Symposium on Behçet's Disease. Istanbul, September 29–30, 1977. J Rheumatol 1978;5(2):229–33.
Zouboulis, CC, Kotter, I, Djawari, D, et al. Epidemiological features of Adamantiades-Behçet's disease in Germany and in Europe. Yonsei Med J 1997;38(6):411–22.
Gul, A, Inanc, M, Ocal, L, Aral, O, Konice, M.Familial aggregation of Behçet's disease in Turkey. Ann Rheum Dis 2000;59(8):622–5.
Ohno, S, Ohguchi, M, Hirose, S, et al. Close association of HLA-Bw51 with Behçet's disease. Arch Ophthalmol 1982;100(9):1455–8.
Mizuki, N, Inoko, H, Ohno, S.Pathogenic gene responsible for the predisposition of Behçet's disease. Int Rev Immunol 1997;14(1):33–48.
Karasneh, J, Gul, A, Ollier, WE, Silman, AJ, Worthington, J.Whole-genome screening for susceptibility genes in multicase families with Behçet's disease. Arthritis Rheum 2005;52(6):1836–42.
O'Duffy, JD, Carney, JA, Deodhar, S.Behçet's disease. Report of 10 cases, 3 with new manifestations. Ann Intern Med 1971;75(4):561–70.
Chajek, T, Fainaru, M.Behçet's disease. Report of 41 cases and a review of the literature. Medicine (Baltimore) 1975;54(3):179–96.
Shimizu, T, Ehrlich, GE, Inaba, G.Behçet's disease. Semin Arthritis Rheum 1979;8:223–60.
Lakhanpal, S, Tani, K, Lie, JT, et al. Pathologic features of Behçet's syndrome: a review of Japanese autopsy registry data. Hum Pathol 1985;16(8):790–5.
Matsumoto, T, Uekusa, T, Fukuda, Y.Vasculo-Behçet's disease: a pathologic study of eight cases. Hum Pathol 1991;22(1):45–51.
Hamuryudan, V, Yurdakul, S, Yazici, H.Behçet's syndrome. Rheumatol Eur 1997;26:31–3.
Kaklamani, VG, Vaiopoulos, G, Kaklamanis, PG.Behçet's Disease. Semin Arthritis Rheum 1998;27(4):197–217.
Sakane, T, Takeno, M, Suzuki, N, Inaba, G.Behçet's disease. N Engl J Med 1999;341(17):1284–91.
Uzun, O, Erkan, L, Akpolat, I, et al.. Pulmonary involvement in Behçet's disease. Respiration 2008;75(3):310–21.
Sekine, K, Nakajima, Y, Sawamoto, S, et al. [Vasculo-Behçet's disease with fatal massive hemoptysis]. Nihon Kokyuki Gakkai Zasshi 1999;37(2):135–9.
Peall, AF, Jones, SM.Hemoptysis and Behçet's Syndrome. J Rheumatol 2009;36(4):848–9.
Uzun, O, Akpolat, T, Erkan, L.Pulmonary vasculitis in Behçet disease: a cumulative analysis. Chest 2005;127(6):2243–53.
Dilsen, N, Konice, M, Gazioglu, K.Pleuropulmonary manifestations in Behçet's disease. In Dilsen, N, Konice, M, Ovul, N, eds. Proceedings of an International Symposium on Behçet's disease. Amsterdam: Elsevier Science, 1977. pp. 9–43.
Valesini, G, Pezzi, PP, Catarinelli, G.Clinical manifestations of Behçet's disease in Italy: study of 155 patients at Rome University. In O'Duffy, JD, Kokmen, E, eds. Behçet's Disease: Basic and Clinical Aspects. New York: Marcel Dekker, 1991. pp. 279–89.
Shahram, F, Davatchi, F, Akbarian, M. The 1996 Survey of Behçet's disease in Iran: study of 3153 cases [abstract]. In: VIIIth International Conference on Behçet's Disease 1996 Revue du Rhumatisme, English ed.; 1996. p. 538.
Oshima, Y, Shimizu, T, Yokohari, R, et al. Clinical studies on Behçet's syndrome. Ann Rheum Dis 1963;22(1):36–45.
Greene, RM, Saleh, A, Taylor, AK, et al. Non-invasive assessment of bleeding pulmonary artery aneurysms due to Behçet disease. Eur Radiol 1998;8(3):359–63.
Puckette, TC, Jolles, H, Proto, AV.Magnetic resonance imaging confirmation of pulmonary artery aneurysm in Behçet's disease. J Thorac Imaging 1994;9(3):172–5.
Tunaci, M, Ozkorkmaz, B, Tunaci, A, et al. CT findings of pulmonary artery aneurysms during treatment for Behçet's disease. AJR Am J Roentgenol 1999;172(3):729–33.
Abid, R, Hadj Taieb Abid, F, Chaabane, M, et al. [Superior caval syndrome caused by chronic mediastinitis in Behçet's disease]. J Radiol 1995;76(2–3):155–7.
Okita, Y, Ando, M, Minatoya, K, Kitamura, S, Matsuo, H.Multiple pseudoaneurysms of the aortic arch, right subclavian artery, and abdominal aorta in a patient with Behçet's disease. J Vasc Surg 1998;28(4):723–6.
Abadoglu, O, Osma, E, Ucan, ES, et al. Behçet's disease with pulmonary involvement, superior vena cava syndrome, chyloptysis and chylous ascites. Respir Med 1996;90(7):429–31.
Raz, I, Okon, E, Chajek-Shaul, T.Pulmonary manifestations in Behçet's syndrome. Chest 1989;95(3):585–9.
Gul, A, Yilmazbayhan, D, Buyukbabani, N, et al. Organizing pneumonia associated with pulmonary artery aneurysms in Behçet's disease. Rheumatology (Oxford) 1999;38(12):1285–9.
Min, JK, Hong, YS, Park, SH, et al. Bronchiolitis obliterans organizing pneumonia as an initial manifestation in patients with systemic lupus erythematosus. J Rheumatol 1997;24(11):2254–7.
Ishida, K, Seki, R, Inoue, T, et al. [Clinical aspect of diffuse alveolar hemorrhage syndrome]. Nihon Kokyuki Gakkai Zasshi 2003;41(12):851–6.
Tunaci, A, Berkmen, YM, Gokmen, E.Thoracic involvement in Behçet's disease: pathologic, clinical, and imaging features. AJR Am J Roentgenol 1995;164(1):51–6.
Yazici, H, Yurdakul, S, Hamuryudan, V.Behçet's syndrome. Curr Opin Rheumatol 1999;11(1):53–7.
Sakane, T, Suzuki, N, Nagafuchi, H.Etiopathology of Behçet's disease: immunological aspects. Yonsei Med J 1997;38(6):350–8.
Mege, JL, Dilsen, N, Sanguedolce, V, et al. Overproduction of monocyte derived tumor necrosis factor alpha, interleukin (IL) 6, IL-8 and increased neutrophil superoxide generation in Behçet's disease. A comparative study with familial Mediterranean fever and healthy subjects. J Rheumatol 1993;20(9):1544–9.
Kaneko, S, Suzuki, N, Yamashita, N, et al. Characterization of T cells specific for an epitope of human 60-kD heat shock protein (hsp) in patients with Behçet's disease (BD) in Japan. Clin Exp Immunol 1997;108(2):204–12.
Freysdottir, J, Lau, S, Fortune, F.Gammadelta T cells in Behçet's disease (BD) and recurrent aphthous stomatitis (RAS). Clin Exp Immunol 1999;118(3):451–7.
Frassanito, MA, Dammacco, R, Cafforio, P, Dammacco, F.Th1 polarization of the immune response in Behçet's disease: a putative pathogenetic role of interleukin-12. Arthritis Rheum 1999;42(9):1967–74.
Sugi-Ikai, N, Nakazawa, M, Nakamura, S, Ohno, S, Minami, M.Increased frequencies of interleukin-2- and interferon-gamma-producing T cells in patients with active Behçet's disease. Invest Ophthalmol Vis Sci 1998;39(6):996–1004.
Takeno, M, Kariyone, A, Yamashita, N, et al. Excessive function of peripheral blood neutrophils from patients with Behçet's disease and from HLA-B51 transgenic mice. Arthritis Rheum 1995;38(3):426–33.
Sensi, A, Gavioli, R, Spisani, S, et al. HLA B51 antigen associated with neutrophil hyper-reactivity. Dis Markers 1991;9(6):327–31.
Gul, A, Esin, S, Dilsen, N, et al. Immunohistology of skin pathergy reaction in Behçet's disease. Br J Dermatol 1995;132(6):901–7.
Hirohata, S, Hashimoto, T.Abnormal T cell responses to bacterial superantigens in Behçet's disease (BD). Clin Exp Immunol 1998;112(2):317–24.
Pervin, K, Childerstone, A, Shinnick, T, et al. T cell epitope expression of mycobacterial and homologous human 65-kilodalton heat shock protein peptides in short term cell lines from patients with Behçet's disease. J Immunol 1993;151(4):2273–82.
Direskeneli, H, Eksioglu-Demiralp, E, Yavuz, S, et al. T cell responses to 60/65 kDa heat shock protein derived peptides in Turkish patients with Behçet's disease. J Rheumatol 2000;27(3):708–13.
Erkan, D, Yazici, Y, Sanders, A, Trost, D, Yazici, H.Is Hughes-Stovin syndrome Behçet's disease?Clin Exp Rheumatol 2004;22(4 Suppl 34):S64–8.
Hughes, JP, Stovin, PG.Segmental pulmonary artery aneurysms with peripheral venous thrombosis. Br J Dis Chest 1959;53(1):19–27.
Hamuryudan, V, Yurdakul, S, Moral, F, et al. Pulmonary arterial aneurysms in Behçet's syndrome: a report of 24 cases. Br J Rheumatol 1994;33(1):48–51.
Erkan, F.Pulmonary involvement in Behçet disease. Curr Opin Pulm Med 1999;5(5):314–8.
Lohr, RH, Boland, BJ, Douglas, WW, et al. Organizing pneumonia. Features and prognosis of cryptogenic, secondary, and focal variants. Arch Intern Med 1997;157(12):1323–9.
Le Thi Huong, D, Wechsler, B, Papo, T, et al. Arterial lesions in Behçet's disease. A study in 25 patients. J Rheumatol 1995;22(11):2103–13.
Galateau, F, Loire, R, Capron, F, et al. [Pulmonary lesions in Wegener's disease. Report of the French Anatomo-clinical Research Group. Study of 40 pulmonary biopsies]. Rev Mal Respir 1992;9(4):431–42.
Michel, BA, Arend, WP, Hunder, GG.Clinical differentiation between giant cell (temporal) arteritis and Takayasu's arteritis. J Rheumatol 1996;23(1):106–11.