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Transient left bundle branch block and left ventricular dysfunction in a patient with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome

  • Mark R. Garrelfs (a1), Esther Hoppenreijs (a2) and Ronald B. Tanke (a1)

Abstract

The NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome is a rare novel autoinflammatory disorder. Cardiac involvement has not been previously reported. We present a 12-year-old girl with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome who was diagnosed with severely impaired left ventricular function and complete left bundle branch block during an exacerbation of the disease. Cardiac dysfunction proved to be rapidly reversible after initiation of high-dose methylprednisolone.

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Corresponding author

Author for correspondence: Garrelfs MR, Department of Pediatric Cardiology, Radboud University Medical Centre, Nijmegen, The Netherlands. E-mail: Mark.garrelfs@radboudumc.nl

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Cite this article: Garrelfs MR, Hoppenreijs E, Tanke RB. (2019) Transient left bundle branch block and left ventricular dysfunction in a patient with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome. Cardiology in the Young29: 435–438. doi: 10.1017/S1047951118002342

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References

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1. Grandemange, S, Sanchez, E, Louis-Plence, P, et al. A new autoinflammatory and autoimmune syndrome associated with NLRP1 mutations: NAIAD (NLRP1-associated autoinflammation with arthritis and dyskeratosis). Ann Rheum Dis 2017; 76: 11911198.10.1136/annrheumdis-2016-210021
2. Tissot, C, Singh, Y, Sekarski, N. Echocardiographic evaluation of ventricular function—for the neonatologist and pediatric intensivist. Front Pediatr 2018; 6: 79.
3. Comarmond, C, Cacoub, P. Myocarditis in auto-immune or auto-inflammatory diseases. Autoimmun Rev 2017; 16: 811816.
4. Rich, MM, McGarvey, ML, Teener, JW, et al. ECG changes during septic shock. Cardiology 2002; 97: 187196.
5. Mann, DL. Innate immunity and the failing heart: the cytokine hypothesis revisited. Circ Res 2015; 116: 12541268.10.1161/CIRCRESAHA.116.302317
6. Sergi, C, Shen, F, Lim, DW, et al. Cardiovascular dysfunction in sepsis at the dawn of emerging mediators. Biomed Pharmacother 2017; 95: 153160.
7. Novick, D, Kim, S, Kaplanski, G, et al. Interleukin-18, more than a Th1 cytokine. Semin Immunol 2013; 25: 439448.
8. Raeburn, CD, Dinarello, CA, Zimmerman, MA, et al. Neutralization of IL-18 attenuates lipopolysaccharide-induced myocardial dysfunction. Am J Physiol Heart Circ Physiol 2002, pp 650657.
9. Pomerantz, BJ1, Reznikov, LL, Harken, AH, et al. Inhibition of caspase 1 reduces human myocardial ischemic dysfunction via inhibition of IL-18 and IL-1beta. Proc Natl Acad Sci USA 2001; 98: 28712876.
10. Hedayat, M1, Mahmoudi, MJ, Rose, NR, et al. Proinflammatory cytokines in heart failure: double-edged swords. Heart Fail Rev 2010; 15: 543562.

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