Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Matthieu Groh, Gabriella Masciocco, Elizabeth Kirchner, Arnt Kristen, Carlo Pellegrini, Shaïda Varnous, Guillermo Bortman, Mark Rosenberg, Antonio Brucato, Paul Waterworth, Edgardo Bonacina, Fabio Facchetti, Leonard Calabrese, Gina Gregorini, Juan Jose Scali, Randall Starling, Maria Frigerio, Andrea Maria D'Armini, Loïc Guillevin

Research output: Contribution to journalArticle

Abstract

Background Heart involvement is the leading cause of death of patients with eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome) and is more frequent in anti-neutrophil cytoplasm antibody (ANCA)-negative patients. Post-transplant outcome has only been reported once. Methods We conducted a retrospective international multicenter study. Patients satisfying the criteria of the American College of Rheumatology and/or revised Chapel Hill Consensus Conference Nomenclature were identified by collaborating vasculitis and transplant specialists, and the help of the Churg-Strauss Syndrome Association. Results Nine ANCA- patients who received transplants between October 1987 and December 2009 were identified. The vasculitis and cardiomyopathy diagnoses were concomitant for 5 patients and separated by 12 to 288 months for the remaining 4 patients. Despite ongoing immunosuppression, histologic examination of 7 (78%) patients' explanted hearts showed histologic patterns suggestive of active vasculitis. The overall 5-year survival rate was low (57%), but rose to 80% when considering only the 6 patients transplanted during the last decade. After survival lasting 3 to 60 months, 4 (44%) patients died sudden deaths. Conclusions The search for EGPA-related cardiomyopathy is mandatory early in the course of this type of vasculitis. Indeed, prompt treatment with corticosteroids and cyclophosphamide may achieve restore cardiac function. Most patients in this series were undertreated. For patients with refractory EGPA, heart transplantation should be performed, which carries a fair prognosis. No optimal immunosuppressive strategy has yet been identified.

Original languageEnglish
Pages (from-to)842-850
Number of pages9
JournalJournal of Heart and Lung Transplantation
Volume33
Issue number8
DOIs
Publication statusPublished - 2014

Keywords

  • asthma
  • cardiac arrhythmias
  • cardiomyopathy
  • Churg-Strauss
  • heart transplantation
  • hypereosinophilic syndrome
  • immunosuppression
  • myocarditis
  • syndrome

ASJC Scopus subject areas

  • Transplantation
  • Cardiology and Cardiovascular Medicine
  • Pulmonary and Respiratory Medicine
  • Surgery
  • Medicine(all)

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