Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
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.
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, INSERM U1016, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France. Electronic address: matthieugroh@hotmail.com.
Matthieu
M
Groh
Department of Cardiology and Heart Transplantation, Ospedale Niguarda, Milan, Italy.
Gabriella
G
Masciocco
Department of Rheumatologic and Immunologic Diseases, Cleveland Clinic, Cleveland, Ohio, USA.
Elizabeth
E
Kirchner
Department of Cardiology, Angiology, and Respiratory Medicine, University Hospital Heidelberg, Heidelberg, Germany.
Arnt
A
Kristen
Division of Cardiac Surgery, Fondazione I.R.C.C.S. Policlinico San Matteo, University of Pavia School of Medicine, Pavia, Italy.
Carlo
C
Pellegrini
Department of Cardiovascular and Thoracic Surgery, Hôpital de la Pitié-Salpêtrière, APHP, Université Pierre-et-Marie-Curie, Paris, France.
Shaïda
S
Varnous
Department of Cardiovascular Surgery, Sanatorio de La Trinidad Mitre, Buenos Aires, Argentina.
Guillermo
G
Bortman
Department of Internal Medicine III (Cardiology and Angiology), University Medical Center Schleswig-Holstein, Campus Kiel, Kiel, Germany.
Mark
M
Rosenberg
Department of Internal Medicine, Ospedale Papa Giovanni XXIII, Bergamo, Italy.
Antonio
A
Brucato
Department of Cardiothoracic Surgery, Wythenshawe Hospital, Manchester, UK.
Paul
P
Waterworth
Department of Pathology, Ospedale Niguarda, Milan, Italy.
Edgardo
E
Bonacina
Department of Pathology, University of Brescia, Spedali Civili Brescia, Brescia, Italy.
Fabio
F
Facchetti
Department of Rheumatologic and Immunologic Diseases, Cleveland Clinic, Cleveland, Ohio, USA.
Leonard
L
Calabrese
Division of Nephrology, Spedali Civili Brescia, Brescia, Italy.
Gina
G
Gregorini
Department of Rheumatology, Autoimmune and Metabolic Bone Diseases, Durand Hospital, Buenos Aires, Argentina.
Juan Jose
JJ
Scali
Heart Failure Center, Heart & Vascular Institute, Cleveland Clinic, Cleveland, Ohio, USA.
Randall
R
Starling
Department of Cardiology and Heart Transplantation, Ospedale Niguarda, Milan, Italy.
Maria
M
Frigerio
Division of Cardiac Surgery, Fondazione I.R.C.C.S. Policlinico San Matteo, University of Pavia School of Medicine, Pavia, Italy.
Andrea Maria
AM
D'Armini
Department of Cardiovascular and Thoracic Surgery, Hôpital de la Pitié-Salpêtrière, APHP, Université Pierre-et-Marie-Curie, Paris, France.
Loïc
L
Guillevin
D000328:Adult; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D009202:Cardiomyopathies / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000401:mortality / Q000601:surgery*; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate; D016896:Treatment Outcome
Churg–Strauss; asthma; cardiac arrhythmias; cardiomyopathy; heart transplantation; hypereosinophilic syndrome; immunosuppression; myocarditis; syndrome