Heart transplantation in a patient with eosinophilic granulomatosis with polyangiitis known as Churg-Strauss syndrome

Soyoung Park; Taeeun Kim; Hyo Jung Kim; Bomi Shin; So Young Park; Hyouk-Soo Kwon; Hee-Bom Moon; You Sook Cho; Tae-Bum Kim

doi:10.4168/aard.2015.3.2.159

Journal List > Allergy Asthma Respir Dis > v.3(2) > 1059090

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Park, Kim, Kim, Shin, Park, Kwon, Moon, Cho, and Kim: Heart transplantation in a patient with eosinophilic granulomatosis with polyangiitis known as Churg-Strauss syndrome

Case Report

Allergy, Asthma & Respiratory Disease 2015; 3(2): 159-163.

Published online: 24 March 2015

DOI: https://doi.org/10.4168/aard.2015.3.2.159

Heart transplantation in a patient with eosinophilic granulomatosis with polyangiitis known as Churg-Strauss syndrome

Soyoung Park¹, Taeeun Kim¹, Hyo Jung Kim¹, Bomi Shin², So Young Park², Hyouk-Soo Kwon², Hee-Bom Moon², You Sook Cho², Tae-Bum Kim²

¹Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea.

²Department of Allergy and Clinical Immunology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Correspondence to: Tae-Bum Kim. Department of Allergy and Clinical Immunology, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 138-736, Korea. Tel: +82-2-3010-3280, Fax: +82-2-3010-6969, allergy@medimail.co.kr

Received 15 September 2014 Revised 30 September 2014 Accepted 7 October 2014

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/).

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a rare systemic necrotizing vasculitis affecting small- to medium-sized vessels. EGPA is associated with severe asthma and eosinophilia. The most frequently involved organs are skin and peripheral nerves; however, EGPA may involve other organs, such as the gastrointestinal tract, kidney, and heart. Antineutrophil cytoplasm antibodies (ANCAs)-related abnormal immune reactions are known to be associated with EGPA, but only 30%-40% of patients have a positive marker of ANCA. ANCA-negative patients are at higher risk of cardiac involvement than ANCA-positive patients. Cardiac involvement is one of the leading causes of mortality and could be resistant to conventional treatment. Early treatment with steroid plus cyclophosphamide is important because it could give chances of restoration of cardiac function. For patients undergoing heart transplantation, we should consider the severity of cardiac disease and the presence of systemic diseases, including vasculitis. Here, we report a case of a 25-year-old EGPA patient with cardiac involvement who eventually received heart transplantation for progressive heart failure, although treated with systemic corticosteroid with cyclophosphamide. EGPA patients undergoing heart transplantion are rarely reported worldwide, and this is the first case report in Korea.

Keywords: Eosinophilicgranulomatous vasculitis, Churg-Strauss syndrome, Heart transplantation, Eosinophilia

Figures and Tables

Fig. 1

Initial electrocardiogram shows atrial fibrillation with rapid ventricular response and right bundle branch block.

Fig. 2

(A) Initial chest x-ray: Image shows cardiomegaly, increased pulmonary vascularity and small amount of pleural effusion. (B) Posttransplantation chest x-ray: Cardiomegaly is markedly improved.

Fig. 3

(A) Preoperative echocardiography shows severely dilated left ventricle, global hypokinesia with atrial fibrillation. (B) Postoperative echocardiography shows normal findings with ejection fraction of 57%.

Fig. 4

Trends of blood eosinophil levels and brain natriuretic peptide (BNP). EF, ejection fraction; QOD, every other day; bid, twice a day.

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