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Immunoglobulin G4 related chronic sclerosing sialadenitis

  • E L Culver (a1), A Hunt (a2), E Crewe (a2), K A Shah (a3) and P Martinez-Devesa (a2)...

Abstract

Background:

ENT surgeons may be the first specialists to encounter and diagnose patients with salivary gland disease. A new entity involving the salivary glands has recently been described of which ENT surgeons need to be aware: immunoglobulin G4 related chronic sclerosing sialadenitis.

Method:

A literature search of Medline, Embase and Cochrane Library databases was performed, using the search terms ‘IgG4’, ‘hyperIgG4 syndrome’ and ‘IgG4 related chronic sclerosing sialadenitis’.

Results:

Knowledge concerning immunoglobulin G4 related chronic sclerosing sialadenitis is rapidly increasing. This new entity is part of a fibro-inflammatory corticosteroid-responsive systemic disease (immunoglobulin G4 related disease) and has been described in almost every organ. Biopsy of the submandibular gland can be diagnostic. However, the diagnosis can easily be overlooked if: clinical suspicion is not high, one is unaware of the classical morphology and/or immunoglobulin G4 staining is not performed. This paper presents a summary of the current understanding of the disease and its management.

Conclusion:

ENT surgeons should be aware of this new disease entity. Patients with systemic disease should be managed under a multidisciplinary team, with input from clinicians who have an interest in such diseases (such as gastroenterologists and rheumatologists), and input from histopathologists and radiologists.

Copyright

Corresponding author

Address for correspondence: Mr Pablo Martinez-Devesa, Department of Otorhinolaryngology – Head and Neck Surgery, John Radcliffe Hospital, Headington, Oxford OX3 9DU, UK Fax: 01865 231 091, E-mail: Pablo.Martinez-Devesa@ouh.nhs.uk

References

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1Hamano, H, Kawa, S, Horiuchi, A, Unno, H, Furuya, N, Akamatsu, T et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001;344:732–8
2Kamisawa, T, Funata, N, Hayashi, Y, Eishi, Y, Koike, M, Tsuruta, K et al. A new clinicopathological entity of IgG4 related autoimmune disease. J Gastroenterol 2003;38:982–4
3Stone, JH, Zen, Y, Deshpande, V. IgG4-related disease. N Engl J Med 2012;366:539–51
4Geyer, JT, Ferry, JA, Harris, NL, Stone, JH, Zukerberg, LR, Lauwers, GY et al. Chronic sclerosing sialadenitis (Küttner tumor) is an IgG4-associated disease. Am J Surg Pathol 2010;34:202–10
5Takano, KL, Yamamoto, M, Takahashi, H, Hinomura, Y, Imai, K, Himi, T. Clinicopathologic similarities between Mikulicz disease and Küttner tumor. Am J Otolaryngol 2010;31:429–34
6Geyer, JT, Deshpande, V. IgG4-associated sialadenitis. Curr Opin Rheumatol 2011;23:95101
7Paul, R, Shekkar, K, Singh, M. Kuttner tumour: an unusual cause of enlargement of a minor salivary gland in the lip. Br J Oral Maxillofac Surg 2010;48:152–3
8Yamamoto, M, Takahashi, H, Ohara, M, Suzuki, C, Naishiro, Y, Yamamoto, H et al. A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease. Mod Rheumatol 2006;16:335–40
9Blanco, M, Mesko, T, Cura, M, Cabello-Inchausti, B. Chronic sclerosing sialadenitis (Kuttner's tumor): unusual presentation with bilateral involvement of major and minor salivary glands. Ann Diagn Pathol 2003;7:2530
10Chan, JK. Kuttner tumour (chronic sclerosing sialadenitis) of the submandibular gland: an underrecognized entity. Adv Anat Pathol 1998;5:239–51
11Hamano, H, Arakura, N, Muraki, T, Ozaki, Y, Kiyosawa, K, Kawa, S. Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis. J Gastroenterol 2006;41:11971205
12Ghazale, A, Chari, ST, Smyrk, TC, Levy, MJ, Topazian, MD, Takahashi, N et al. Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol 2007;102:1646–53
13De Cocker, JL, D'Arco, F, De Beule, T, Tousseyn, T, Blockmans, D, Hermans, R. IgG4-related systemic disease affecting the parotid and submandibular glands: magnetic resonance imaging features of IgG4-related chronic sclerosing sialadenitis and concomitant lymphadenitis. Clin Imaging 2014;38:195–8
14Smyrk, TC. Pathological features of IgG4-related sclerosing disease. Curr Opin Rheumatol 2011;23:74–9
15Culver, EL, Bateman, AC. General principles of IgG4-related disease. Diag Histopathol 2013;19:111–18
16Deshpande, V, Zen, Y, Chan, J, Yi, EE, Sato, Y, Yoshino, T et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25:1181–92
17Strehl, J, Hartmann, A, Agaimy, A. Numerous IgG4-positive plasma cells are ubiquitous in diverse localised non-specific inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol 2011;64:237–43
18Vitali, C, Bombardieri, S, Jonsson, R, Moutsopoulos, HM, Alexander, EL, Carsons, SE et al. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554–8
19Ambrosetti, A, Zanotti, R, Pattaro, C, Lenzi, L, Chilosi, M, Caramaschi, P et al. Most cases of primary salivary mucosa-associated lymphoid tissue lymphoma are associated either with Sjoegren syndrome or hepatitis C virus infection. Br J Haematol 2004;126:43–9
20Ghazale, A, Chari, S. Is autoimmune pancreatitis a risk factor for pancreatic cancer? Pancreas 2007;35:376
21Kim, T, Grobmyer, SR, Dixon, LR, Allan, RW, Hochwald, SN. Autoimmune pancreatitis and concurrent small lymphocytic lymphoma: not just a coincidence? J Gastrointest Surg 2008;12:1566–70
22Cheuk, W, Yuen, HK, Chan, AC, Shih, LY, Kuo, TT, Ma, MW et al. Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease. Am J Surg Pathol 2008;32:1159–67
23Chari, ST, Smyrk, TC, Levy, MJ, Topazian, MD, Takahashi, N, Zhang, L et al. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol 2006;4:1010–16
24Otsuki, M, Chung, JB, Okazaki, K, Kim, MH, Kamisawa, T, Kawa, S et al. Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan-Korea Symposium on Autoimmune Pancreatitis. J Gastroenterol 2008;43:403–8
25Umehara, H, Okazaki, K, Masaki, Y, Kawano, M, Yamamoto, M, Saeki, T et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 2012;22:2130
26Kamisawa, T, Shimosegawa, T, Okazaki, K, Nishino, T, Watanabe, H, Kanno, A et al. Standard steroid treatment for autoimmune pancreatitis. Gut 2009;58:1504–7
27Hart, PA, Kamisawa, T, Brugge, WR, Chung, JB, Culver, EL, Czajo, L et al. Treatment and long-term sequelae of autoimmune pancreatitis: a multicenter, international analysis. Gut 2013;62:1771–6
28Khosroshahi, A, Carruthers, MN, Deshpande, V, Unizony, S, Bloch, DB, Stone, JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore) 2012;91:5766

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