Clinical features and long-term outcomes of patients with systemic polyarteritis nodosa diagnosed since 2005: Data from 196 patients.

Autor: Rohmer J; Department of Internal Medicine, Hôpital Cochin, AP-HP.Centre, Université Paris Cité, Paris, France., Nguyen Y; Department of Internal Medicine, Hôpital Cochin, AP-HP.Centre, Université Paris Cité, Paris, France; Autoimmunity Team, Immunology of Viral Infections and Autoimmune Diseases, INSERM U1184, Université Paris Saclay, Le Kremlin-Bicêtre, France., Trefond L; Department of Internal Medicine, CHU, Clermont Ferrand, France., Agard C; Nantes Université, CHU Nantes, Service de médecine interne, F-44000, Nantes, France., Allain JS; Department of Internal Medicine, CHRU, Rennes, France., Berezne A; Department of Internal Medicine, CH, Annecy, Genevois, France., Charles P; Department of Internal Medicine, Institut Mutualiste Montsouris, Paris, France., Cohen P; Department of Internal Medicine, Hôpital Cochin, AP-HP.Centre, Université Paris Cité, Paris, France., Gondran G; Department of Internal Medicine and dermatology, CHU, Limoges, France., Groh M; Department of Internal Medicine, National Reference Center for Hypereosinophilic Syndromes, Foch Hospital, Suresnes, France; University of Lille, INSERM U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille, France., Huscenot T; Department of Internal Medicine, Hôpital Ambroise Parée, Paris, France., Lacout C; Department of Internal Medicine and Clinical Immunology, CHU, Angers, France., Lazaro E; Department of Internal Medicine, Hôpital Haut Leveque, CHU, Bordeaux, France., London J; Department of Internal Medicine, Hôpital de la Croix Saint Simon, Paris, France., Maurier F; Department of Internal Medicine, CHU, Metz, France., Mekinian A; Department of Internal Medicine, Hôpital Saint Antoine, AP-HP, Sorbonne Université, Paris, France., Mesbah R; Department of Internal Medicine, CH, de Boulogne sur Mer, France., Nubourgh I; Department of Internal Medicine, Université libre de Bruxelles, Belgique., Perard L; Department of Internal Medicine, Hôpital Saint Joseph Saint Luc, Lyon, France., Puéchal X; Department of Internal Medicine, Hôpital Cochin, AP-HP.Centre, Université Paris Cité, Paris, France., Pugnet G; Department of Internal Medicine and clinical immunology, CHU, Toulouse, France., Puyade M; Department of Internal Medicine, CHU, Poitier, France., Queyrel V; Rheumatologie, Hôpital Pasteur, CHU, Nice, France., Roux A; Department of Nephrology, HEGP, Paris, France., Rouzaud D; Department of Internal Medicine, Hôpital Bichat, Paris, France., Durel CA; University Paris-Cité, F-75006, Paris, France., Guillevin L; Department of Internal Medicine, Hôpital Cochin, AP-HP.Centre, Université Paris Cité, Paris, France., Terrier B; Department of Internal Medicine, Hôpital Cochin, AP-HP.Centre, Université Paris Cité, Paris, France; University Paris-Cité, F-75006, Paris, France. Electronic address: benjamin.terrier@aphp.fr.
Jazyk: angličtina
Zdroj: Journal of autoimmunity [J Autoimmun] 2023 Sep; Vol. 139, pp. 103093. Date of Electronic Publication: 2023 Aug 01.
DOI: 10.1016/j.jaut.2023.103093
Abstrakt: Background: The landscape of polyarteritis nodosa (PAN) has substantially changed during the last decades. Recent data regarding causes, characteristics, and prognosis of systemic PAN in the modern era are lacking.
Methods: This retrospective study included patients with systemic PAN referred to the French Vasculitis Study Group between 2005 and 2019. Characteristics, associated conditions and outcomes were collected, and predictors of relapse and death were analyzed.
Results: 196 patients were included. Main clinical symptoms were constitutional (84%), neurological (59%), skin (58%) and musculoskeletal (58%) manifestations. Secondary PAN accounted for 55 (28%) patients, including myelodysplastic syndrome (9%), solid cancer (7%), lymphoma (4%) and autoinflammatory diseases (4%). No patient had active HBV infection. All treated patients (98.5%) received glucocorticoids (GCs), alone (41%) or in combination with immunosuppressants (59%), with remission achieved in 90%. Relapses were independently associated with age >65 years (HR 1.85; 95% CI1.12-3.08), gastrointestinal involvement (1.95; 95% CI1.09-3.52) and skin necrotic lesions (HR 1.95; 95%CI 1.24-3.05). One-, 5- and 10-year overall survival rates were 93%, 87% and 81%, respectively. In multivariate analyses, age >65 years (HR 2.80; 95%CI 1.23-6.37), necrotic purpura (HR 4.16; 95%CI 1.62-10.70), acute kidney injury (HR 4.89; 95% 1.71-13.99) and secondary PAN (HR 2.98; 95%CI 1.29-6.85) were independently associated with mortality.
Conclusion: Landscape of PAN has changed during the last decades, with the disappearance of HBV-PAN and the emergence of secondary PAN. Relapse rate remains high, especially in aged patients with gastrointestinal and cutaneous necrosis, as well as mortality.
(Copyright © 2023. Published by Elsevier Ltd.)
Databáze: MEDLINE
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