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

18 Citations (Scopus)

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

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Churg-Strauss Syndrome
Granulomatosis with Polyangiitis
Heart Transplantation
Vasculitis
Transplants
Cardiomyopathies
Cytoplasm
Neutrophils
Antibodies
Immunosuppressive Agents
Sudden Death
Terminology
Cyclophosphamide
Immunosuppression
Multicenter Studies
Cause of Death
Adrenal Cortex Hormones
Survival Rate

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)

Cite this

Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). / Groh, Matthieu; Masciocco, Gabriella; Kirchner, Elizabeth; Kristen, Arnt; Pellegrini, Carlo; Varnous, Shaïda; Bortman, Guillermo; Rosenberg, Mark; Brucato, Antonio; Waterworth, Paul; Bonacina, Edgardo; Facchetti, Fabio; Calabrese, Leonard; Gregorini, Gina; Scali, Juan Jose; Starling, Randall; Frigerio, Maria; D'Armini, Andrea Maria; Guillevin, Loïc.

In: Journal of Heart and Lung Transplantation, Vol. 33, No. 8, 2014, p. 842-850.

Research output: Contribution to journalArticle

Groh, M, Masciocco, G, Kirchner, E, Kristen, A, Pellegrini, C, Varnous, S, Bortman, G, Rosenberg, M, Brucato, A, Waterworth, P, Bonacina, E, Facchetti, F, Calabrese, L, Gregorini, G, Scali, JJ, Starling, R, Frigerio, M, D'Armini, AM & Guillevin, L 2014, 'Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)', Journal of Heart and Lung Transplantation, vol. 33, no. 8, pp. 842-850. https://doi.org/10.1016/j.healun.2014.02.023
Groh, Matthieu ; Masciocco, Gabriella ; Kirchner, Elizabeth ; Kristen, Arnt ; Pellegrini, Carlo ; Varnous, Shaïda ; Bortman, Guillermo ; Rosenberg, Mark ; Brucato, Antonio ; Waterworth, Paul ; Bonacina, Edgardo ; Facchetti, Fabio ; Calabrese, Leonard ; Gregorini, Gina ; Scali, Juan Jose ; Starling, Randall ; Frigerio, Maria ; D'Armini, Andrea Maria ; Guillevin, Loïc. / Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). In: Journal of Heart and Lung Transplantation. 2014 ; Vol. 33, No. 8. pp. 842-850.
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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.",
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T1 - Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

AU - Groh, Matthieu

AU - Masciocco, Gabriella

AU - Kirchner, Elizabeth

AU - Kristen, Arnt

AU - Pellegrini, Carlo

AU - Varnous, Shaïda

AU - Bortman, Guillermo

AU - Rosenberg, Mark

AU - Brucato, Antonio

AU - Waterworth, Paul

AU - Bonacina, Edgardo

AU - Facchetti, Fabio

AU - Calabrese, Leonard

AU - Gregorini, Gina

AU - Scali, Juan Jose

AU - Starling, Randall

AU - Frigerio, Maria

AU - D'Armini, Andrea Maria

AU - Guillevin, Loïc

PY - 2014

Y1 - 2014

N2 - 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.

AB - 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.

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KW - cardiac arrhythmias

KW - cardiomyopathy

KW - Churg-Strauss

KW - heart transplantation

KW - hypereosinophilic syndrome

KW - immunosuppression

KW - myocarditis

KW - syndrome

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