Clinical features and long-term outcomes of patients with systemic polyarteritis nodosa diagnosed since 2005 : Data from 196 patients
Copyright © 2023. Published by Elsevier Ltd..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:139 |
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| Enthalten in: |
Journal of autoimmunity - 139(2023) vom: 01. Sept., Seite 103093 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Rohmer, Julien [VerfasserIn] |
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| Links: |
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| Themen: |
Journal Article |
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| Anmerkungen: |
Date Completed 28.08.2023 Date Revised 28.08.2023 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.jaut.2023.103093 |
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NLM360358934 |
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| 100 | 1 | |a Rohmer, Julien |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Clinical features and long-term outcomes of patients with systemic polyarteritis nodosa diagnosed since 2005 |b Data from 196 patients |
| 264 | 1 | |c 2023 | |
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| 500 | |a Date Completed 28.08.2023 | ||
| 500 | |a Date Revised 28.08.2023 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2023. Published by Elsevier Ltd. | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Polyarteritis nodosa | |
| 650 | 4 | |a Predictor of relapse | |
| 650 | 4 | |a Systemic necrotizing vasculitis | |
| 700 | 1 | |a Nguyen, Yann |e verfasserin |4 aut | |
| 700 | 1 | |a Trefond, Ludovic |e verfasserin |4 aut | |
| 700 | 1 | |a Agard, Christian |e verfasserin |4 aut | |
| 700 | 1 | |a Allain, Jean Sebastien |e verfasserin |4 aut | |
| 700 | 1 | |a Berezne, Alice |e verfasserin |4 aut | |
| 700 | 1 | |a Charles, Pierre |e verfasserin |4 aut | |
| 700 | 1 | |a Cohen, Pascal |e verfasserin |4 aut | |
| 700 | 1 | |a Gondran, Guillaume |e verfasserin |4 aut | |
| 700 | 1 | |a Groh, Matthieu |e verfasserin |4 aut | |
| 700 | 1 | |a Huscenot, Tessa |e verfasserin |4 aut | |
| 700 | 1 | |a Lacout, Carole |e verfasserin |4 aut | |
| 700 | 1 | |a Lazaro, Estibaliz |e verfasserin |4 aut | |
| 700 | 1 | |a London, Jonathan |e verfasserin |4 aut | |
| 700 | 1 | |a Maurier, François |e verfasserin |4 aut | |
| 700 | 1 | |a Mekinian, Arsène |e verfasserin |4 aut | |
| 700 | 1 | |a Mesbah, Rafik |e verfasserin |4 aut | |
| 700 | 1 | |a Nubourgh, Isabelle |e verfasserin |4 aut | |
| 700 | 1 | |a Perard, Laurent |e verfasserin |4 aut | |
| 700 | 1 | |a Puéchal, Xavier |e verfasserin |4 aut | |
| 700 | 1 | |a Pugnet, Gregory |e verfasserin |4 aut | |
| 700 | 1 | |a Puyade, Mathieu |e verfasserin |4 aut | |
| 700 | 1 | |a Queyrel, Viviane |e verfasserin |4 aut | |
| 700 | 1 | |a Roux, Arthur |e verfasserin |4 aut | |
| 700 | 1 | |a Rouzaud, Diane |e verfasserin |4 aut | |
| 700 | 1 | |a Durel, Cecile-Audrey |e verfasserin |4 aut | |
| 700 | 1 | |a Guillevin, Loïc |e verfasserin |4 aut | |
| 700 | 1 | |a Terrier, Benjamin |e verfasserin |4 aut | |
| 700 | 0 | |a French Vasculitis Study Group (FVSG) |e verfasserin |4 aut | |
| 700 | 1 | |a Ackermann, Felix |e investigator |4 oth | |
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| 700 | 1 | |a Bussone, Guillaume |e investigator |4 oth | |
| 700 | 1 | |a Catalan, Pilartxo |e investigator |4 oth | |
| 700 | 1 | |a Chasset, François |e investigator |4 oth | |
| 700 | 1 | |a Crabol, Yoann |e investigator |4 oth | |
| 700 | 1 | |a de Moreuil, Claire |e investigator |4 oth | |
| 700 | 1 | |a Hot, Arnaud |e investigator |4 oth | |
| 700 | 1 | |a Humbert, Marc |e investigator |4 oth | |
| 700 | 1 | |a Chiche, Noémie Jourde |e investigator |4 oth | |
| 700 | 1 | |a Le Gallou, Thomas |e investigator |4 oth | |
| 700 | 1 | |a Leroux, Dominique |e investigator |4 oth | |
| 700 | 1 | |a Mesbah, Rafik |e investigator |4 oth | |
| 700 | 1 | |a Mouthon, Luc |e investigator |4 oth | |
| 700 | 1 | |a Pagnoux, Christian |e investigator |4 oth | |
| 700 | 1 | |a Revuz, Sabine |e investigator |4 oth | |
| 700 | 1 | |a Rieu, Laurent |e investigator |4 oth | |
| 700 | 1 | |a Schmidt, Jean |e investigator |4 oth | |
| 700 | 1 | |a Vandergheynst, Frederic |e investigator |4 oth | |
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