Matthieu Groh1, Gabriella Masciocco2, Elizabeth Kirchner3, Arnt Kristen4, Carlo Pellegrini5, Shaïda Varnous6, Guillermo Bortman7, Mark Rosenberg8, Antonio Brucato9, Paul Waterworth10, Edgardo Bonacina11, Fabio Facchetti12, Leonard Calabrese3, Gina Gregorini13, Juan Jose Scali14, Randall Starling15, Maria Frigerio2, Andrea Maria D'Armini5, Loïc Guillevin6. 1. 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. 2. Department of Cardiology and Heart Transplantation, Ospedale Niguarda, Milan, Italy. 3. Department of Rheumatologic and Immunologic Diseases, Cleveland Clinic, Cleveland, Ohio, USA. 4. Department of Cardiology, Angiology, and Respiratory Medicine, University Hospital Heidelberg, Heidelberg, Germany. 5. Division of Cardiac Surgery, Fondazione I.R.C.C.S. Policlinico San Matteo, University of Pavia School of Medicine, Pavia, Italy. 6. Department of Cardiovascular and Thoracic Surgery, Hôpital de la Pitié-Salpêtrière, APHP, Université Pierre-et-Marie-Curie, Paris, France. 7. Department of Cardiovascular Surgery, Sanatorio de La Trinidad Mitre, Buenos Aires, Argentina. 8. Department of Internal Medicine III (Cardiology and Angiology), University Medical Center Schleswig-Holstein, Campus Kiel, Kiel, Germany. 9. Department of Internal Medicine, Ospedale Papa Giovanni XXIII, Bergamo, Italy. 10. Department of Cardiothoracic Surgery, Wythenshawe Hospital, Manchester, UK. 11. Department of Pathology, Ospedale Niguarda, Milan, Italy. 12. Department of Pathology, University of Brescia, Spedali Civili Brescia, Brescia, Italy. 13. Division of Nephrology, Spedali Civili Brescia, Brescia, Italy. 14. Department of Rheumatology, Autoimmune and Metabolic Bone Diseases, Durand Hospital, Buenos Aires, Argentina. 15. Heart Failure Center, Heart & Vascular Institute, Cleveland Clinic, Cleveland, Ohio, USA.
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.
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.
Authors: Enrico Ammirati; Emanuele Bizzi; Giacomo Veronese; Matthieu Groh; Caroline M Van de Heyning; Jukka Lehtonen; Marc Pineton de Chambrun; Alberto Cereda; Chiara Picchi; Lucia Trotta; Javid J Moslehi; Antonio Brucato Journal: Front Med (Lausanne) Date: 2022-03-07
Authors: Alessandro Giollo; Raluca B Dumitru; Peter P Swoboda; Sven Plein; John P Greenwood; Maya H Buch; Jacqueline Andrews Journal: Int J Cardiovasc Imaging Date: 2020-10-14 Impact factor: 2.357