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Rare presentation of eosinophilic granulomatosis polyangiitis with left ventricular endomyocardial fibrosis in a child

Published online by Cambridge University Press:  16 May 2022

Vinoth Doraiswamy Open the ORCID record for Vinoth Doraiswamy [Opens in a new window] ,
Sivakumar Vengudusamy  and
Jayavardhana Arumugam
Vinoth Doraiswamy*
Affiliation:
Department of Cardiology, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore 641004, Tamil Nadu, India
Sivakumar Vengudusamy
Affiliation:
Department of Rheumatology, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore 641004, Tamil Nadu, India
Jayavardhana Arumugam
Affiliation:
Department of Pediatrics, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore 641004, Tamil Nadu, India
*
Address for correspondence: Vinoth Doraiswamy, MD, FNB (Pediatric Cardiology), Consultant Pediatric Cardiologist, Department of Cardiology, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore 641004, Tamil Nadu, India. Tel: +91-422-2570170, +91-422-4345353. E-mail: [email protected]
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Abstract

Eosinophilic granulomatosis polyangiitis represents less than 2% of vasculitis cases in childhood. Children have worse long-term outcomes and higher mortality. Cardiac involvement portends a worse prognosis. We describe here an adolescent girl who presented with heart failure and stroke. Her blood investigations showed eosinophilia and high IgE levels. Cardiac evaluation revealed myocarditis, intracardiac thrombus, and endomyocardial fibrosis, a rare presentation of this disease in childhood.

Keywords

EGPAendomyocardial fibrosismural thrombuscardioembolic stroke
Type
Brief Report
Information
Cardiology in the Young , Volume 33 , Issue 1 , January 2023 , pp. 153 - 155
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Copyright
© The Author(s), 2022. Published by Cambridge University Press

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References

Fina, A, Dubus, JC, Tran, A, et al. Eosinophilic granulomatosis with polyangiitis in children: data from the French RespiRare cohort. Pediatr Pulm 2018; 1–11.Google ScholarPubMed
Eleftheriou, D, Gale, H, Pilkington, C, Fenton, M, Sebire, NJ, Brogan, PA. Eosinophilic granulomatosis with polyangiitis in childhood: retrospective experience from a tertiary referral centre in the UK. Rheumatology (Oxford) 2016; 55: 1263–1272.CrossRefGoogle ScholarPubMed
Gendelman, S, Zeft, A, Spalding, SJ. Childhood-onset eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): a contemporary single-center cohort. J Rheumatol 2013; 40: 929–935.CrossRefGoogle ScholarPubMed
Zwerina, J, Eger, G, Englbrecht, M, Manger, B, Schett, G. Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients. Semin Arthritis Rheum 2008; 39: 108–115.CrossRefGoogle ScholarPubMed
Cereda, AF, Pedrotti, P, De Capitani, L, Giannattasio, C, Roghi, A. Comprehensive evaluation of cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA) with cardiac magnetic resonance. Eur J Intern Med 2017; 39: 51–56.CrossRefGoogle ScholarPubMed
Amaddeo, A, Ventura, A, Marchetti, F, et al. Should cardiac involvement be included in the criteria for diagnosis of Churg Strauss Syndrome? J Pediatr 2012; 160: 707.CrossRefGoogle ScholarPubMed
Moradinejad, MH, Rezaei, A, Ziaee, V. Juvenile Churg–Strauss syndrome as an etiology of myocarditis and ischaemic stroke in adolescents; a case report. Iran J Pediatr 2011; 21: 530–534.Google ScholarPubMed
Albahri, Z, Minxová, L, Lukeš, A, Mawiri, AAL, Štefáčková, Š. Churg-Strauss syndrome in childhood: a case report. J Child Neurol 2014; 29: 98–100.CrossRefGoogle ScholarPubMed
Ito, T, Fujita, S-I, Kanzaki, Y, Sohmiya, K, Hoshiga, M. Eosinophilic granulomatosis with polyangiitis (EGPA) with an unusual manifestation of mid-ventricular obstruction caused by endocardial thrombus. Am J Case Rep 2018; 19: 1197–1203.CrossRefGoogle Scholar
Guillevin, Lïc, Cohen, P, Gayraud, M, Lhote, Fçois, Jarrousse, B, Casassus, P. Churg-Strauss syndrome clinical study and long-term follow-up of 96 patients. Medicine 1999; 78: 26–37.CrossRefGoogle ScholarPubMed

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