Sabine Jardel1, Xavier Puéchal2, Alain Le Quellec3, Christian Pagnoux2, Mohamed Hamidou4, François Maurier5, Olivier Aumaitre6, Achille Aouba7, Thomas Quemeneur8, Jean-François Subra9, Vincent Cottin10, Jean Sibilia11, Pascal Godmer12, Patrice Cacoub13, Anne Laure Fauchais14, Eric Hachulla15, Delphine Maucort-Boulch16, Loïc Guillevin2, Jean-Christophe Lega17. 1. National Referral Centre for rare Juvenile Rheumatological and Autoimmune Diseases, Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, UMR 5558, Laboratoire de Biométrie et Biologie Evolutive, CNRS, Claude Bernard University Lyon 1, Lyon, France. 2. National Referral Centre for rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Public - Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France. 3. Department of Internal Medicine, Centre Hospitalier Régional Universitaire de Montpellier, Hôpital St. Eloi, Montpellier 1 University, Montpellier, France. 4. Department of Internal Medicine, Centre Hospitalier Universitaire Hôtel-Dieu, Nantes, France. 5. Department of Internal medicine and Clinical Immunology, Site Belle Isle, Metz, France. 6. Department of Internal Medicine, Centre Hospitalier Universitaire, Hôpital Gabriel Montpied, Clermont-Ferrand, France. 7. Department of Internal Medicine, Centre Hospitalier Universitaire de Caen, Caen University, Caen, France. 8. Department of Internal Medicine, Centre Hospitalier, Valenciennes, France. 9. Department of Internal Medicine, Centre Hospitalier Universitaire d' Angers, Angers, France. 10. Department of Respiratory Medicine, National Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon, France. 11. Department of Rheumatology, National Reference Center for rare Systemic Autoimmune Diseases, Strasbourg University Hospital, Strasbourg University, Strasbourg, France. 12. Department of Internal Medicine, Centre Hospitalier Bretagne-Atlantique, Vannes, France. 13. Sorbonne Universités, UPMC Univ Paris 06, UMR 7211, and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), F-75005, Paris, France; INSERM, UMRS 959, F-75013, Paris, France; CNRS, FRE3632, F-75005, Paris, France; AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, F-75013, Paris, France. 14. Department of Internal Medicine, Hôpital Dupuytren, Centre Hospitalier Universitaire de Limoges Limoges University, Limoges, France. 15. Department of Internal Medicine, Hôpital Claude Huriez, University of Lille, Lille, France. 16. Department of Biostatistics and bioinformatics, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon Claude Bernard University Lyon 1, Lyon, France. 17. National Referral Centre for rare Juvenile Rheumatological and Autoimmune Diseases, Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, UMR 5558, Laboratoire de Biométrie et Biologie Evolutive, CNRS, Claude Bernard University Lyon 1, Lyon, France. Electronic address: jean-christophe.lega@chu-lyon.fr.
Abstract
OBJECTIVE: The aim of the study was to describe the evolution of mortality and cause-specific mortality over time in patients with systemic necrotizing vasculitides (SNV), including polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: Patients with SNV from the French Vasculitis Study Group registry were divided into 5 groups according to the date of diagnosis: <1980, 1980-1989, 1990-1999, 2000-2010, and ≥ 2010. The causes of death were classified as vasculitis, infection, cardiovascular, malignancy, miscellaneous, or unknown. RESULTS: Among the 2217 patients included (PAN 16.1%, GPA 41.7%, EGPA 22.6%, MPA 19.6%), overall incidence of death was 2.26 per 100 person-years. The overall survival improved during each period considered. The 5-year survival rate increased from 72.2% (95% confidence interval [CI] 59.7-87.2) for patients diagnosed before 1980 to 94.5% (95% CI 90.4-98.8) after 2010 (p < 0.001). Periods of diagnosis, age, and male gender were independently associated with a poor survival with a non-significant difference between vasculitis. The incidence of mortality between the 1980s and after 2010 significantly decreased for vasculitis-related (p = 0.03) and cardiovascular-related deaths (p = 0.04). Incidence of death by infection remained stable between the 1980s and the 2000s but no death by infection occurred after 2010. The incidence of death by malignancy remained stable over time. CONCLUSION: Overall survival of SNV patients has improved since the 1980s with the decrease of vasculitis- and cardiovascular-related deaths, but cancer-related mortality remained stable. These results highlight malignancy as the current target to improve the overall prognosis.
OBJECTIVE: The aim of the study was to describe the evolution of mortality and cause-specific mortality over time in patients with systemic necrotizing vasculitides (SNV), including polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). METHODS:Patients with SNV from the French Vasculitis Study Group registry were divided into 5 groups according to the date of diagnosis: <1980, 1980-1989, 1990-1999, 2000-2010, and ≥ 2010. The causes of death were classified as vasculitis, infection, cardiovascular, malignancy, miscellaneous, or unknown. RESULTS: Among the 2217 patients included (PAN 16.1%, GPA 41.7%, EGPA 22.6%, MPA 19.6%), overall incidence of death was 2.26 per 100 person-years. The overall survival improved during each period considered. The 5-year survival rate increased from 72.2% (95% confidence interval [CI] 59.7-87.2) for patients diagnosed before 1980 to 94.5% (95% CI 90.4-98.8) after 2010 (p < 0.001). Periods of diagnosis, age, and male gender were independently associated with a poor survival with a non-significant difference between vasculitis. The incidence of mortality between the 1980s and after 2010 significantly decreased for vasculitis-related (p = 0.03) and cardiovascular-related deaths (p = 0.04). Incidence of death by infection remained stable between the 1980s and the 2000s but no death by infection occurred after 2010. The incidence of death by malignancy remained stable over time. CONCLUSION: Overall survival of SNV patients has improved since the 1980s with the decrease of vasculitis- and cardiovascular-related deaths, but cancer-related mortality remained stable. These results highlight malignancy as the current target to improve the overall prognosis.
Authors: Irena Doubelt; David Cuthbertson; Simon Carette; Sharon A Chung; Lindsy J Forbess; Nader A Khalidi; Curry L Koening; Carol Langford; Carol A McAlear; Larry W Moreland; Paul A Monach; Philip Seo; Ulrich Specks; Robert F Spiera; Jason M Springer; Antoine G Sreih; Kenneth J Warrington; Peter A Merkel; Christian Pagnoux Journal: ACR Open Rheumatol Date: 2021-05-25
Authors: Pierre Jourdain; Benoit Brilland; Ouassim Medhioub; Jeanne Caron; Clément Samoreau; Assia Djema; Renaud Gansey; Jean-Philippe Coindre; Maud Cousin; Anne Sophie Garnier; Nicolas Henry; Samuel Wacrenier; Jeremy Riou; Giorgina Barbara Piccoli; Jean-François Augusto Journal: Kidney Int Rep Date: 2021-01-07