Efficacy and safety of treatments in cutaneous polyarteritis nodosa: A French observational retrospective study


      Cutaneous polyarteritis nodosa is a form of medium-sized vessel vasculitis. Despite a disabling and prolonged course, data on treatment efficacy and safety remain scarce.


      We aimed to describe treatment efficacy and safety in patients with cutaneous polyarteritis nodosa.


      This multicenter retrospective, observational study, recorded clinical and biologic data together with treatments received. The primary outcome was the rate of complete response at month 3. Secondary outcomes assessed drug survival and safety.


      We included 68 patients who received a median of 2 therapeutic lines (interquartile range, 1-3). Overall, complete response was achieved in 13 of 42 (31%) patients with colchicine, 4 of 17 (23%) with dapsone, 11 of 25 (44%) with glucocorticoids (GCs) alone, 1 of 9 (11%) with nonsteroidal anti-inflammatory drugs, 11 of 13 (84%) with GCs+azathioprine, and 7 of 15 (47%) with GCs+methotrexate. GCs+azathioprine had the best drug survival (median duration, 29.5 months; interquartile range, 19.5-36.0). Response at month 3 was decreased with peripheral neurologic involvement (odds ratio, 0.19; 95% confidence interval, 0.03-0.81; P = .04). Overall, the rate of treatment-related adverse events was 18%, which led to the discontinuation of treatment in 7% of patients.


      Retrospective study.


      Colchicine seems to confer good benefit-risk balance in cutaneous polyarteritis nodosa without peripheral sensory neuropathy. GCs+azathioprine seem the best treatment in the event of relapse.

      Key words

      Abbreviations used:

      AZA (azathioprine), CR (complete response), cPAN (cutaneous polyarteritis nodosa), CYC (cyclophosphamide), GCs (glucocorticoids), ISs (immunosuppressants), IQR (interquartile range), IVIgs (intravenous immunoglobulins), MTX (methotrexate), NSAIDs (nonsteroidal anti-inflammatory drugs), sPAN (systemic polyarteritis nodosa)
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        • Jennette J.C.
        • Falk R.J.
        • Bacon P.A.
        • et al.
        2012 revised international chapel hill consensus conference nomenclature of vasculitides.
        Arthritis Rheum. 2013; 65: 1-11
        • Pagnoux C.
        • Seror R.
        • Henegar C.
        • et al.
        Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French vasculitis study group database.
        Arthritis Rheum. 2010; 62: 616-626
        • Alibaz-Oner F.
        • Koster M.J.
        • Crowson C.S.
        • et al.
        Clinical spectrum of medium-sized vessel vasculitis.
        Arthritis Care Res. 2017; 69: 884-891
        • Régent A.
        • Mouthon L.
        • Guillevin L.
        • Terrier B.
        Role of therapeutic plasma exchanges in systemic vasculitis.
        Transfus Apher Sci. 2020; 59: 102992
        • Morgan A.J.
        • Schwartz R.A.
        Cutaneous polyarteritis nodosa: a comprehensive review. Cutaneous polyarteritis nodosa.
        Int J Dermatol. 2010; 49: 750-756
        • Shirai T.
        • Shirota Y.
        • Fujii H.
        • Ishii T.
        • Harigae H.
        Four distinct clinical phenotypes of vasculitis affecting medium-sized arteries.
        Scand J Rheumatol. 2019; 48: 308-314
        • Minkowitz G.
        Benign cutaneous polyarteritis nodosa. Relationship to systemic polyarteritis nodosa and to hepatitis B infection.
        Arch Dermatol. 1991; 127: 1520
        • Ozen S.
        The changing face of polyarteritis nodosa and necrotizing vasculitis.
        Nat Rev Rheumatol. 2017; 13: 381-386
        • Criado P.R.
        • Marques G.F.
        • Morita T.C.A.B.
        • de Carvalho J.F.
        Epidemiological, clinical and laboratory profiles of cutaneous polyarteritis nodosa patients: report of 22 cases and literature review.
        Autoimmun Rev. 2016; 15: 558-563
        • Ishiguro N.
        • Kawashima M.
        Cutaneous polyarteritis nodosa: a report of 16 cases with clinical and histopathological analysis and a review of the published work.
        J Dermatol. 2010; 37: 85-93
        • Maillard H.
        • Szczesniak S.
        • Martin L.
        • et al.
        Ann Dermatol Venereol. 1999; 126: 125-129
        • Lobo I.
        • Ferreira M.
        • Silva E.
        • Alves R.
        • Selores M.
        Cutaneous polyarteritis nodosa treated with intravenous immunoglobulins.
        J Eur Acad Dermatol Venereol. 2008; 22: 880-882
        • Furukawa F.
        Cutaneous polyarteritis nodosa: an update.
        Ann Vasc Dis. 2012; 5: 282-288
        • Guillevin L.
        • Lhote F.
        • Gayraud M.
        • et al.
        Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients.
        Medicine (Baltimore). 1996; 75: 17-28
        • Zhou Q.
        • Yang D.
        • Ombrello A.K.
        • et al.
        Early-onset stroke and vasculopathy associated with mutations in ADA2.
        N Engl J Med. 2014; 370: 911-920
        • Guillevin L.
        • Cordier J.F.
        • Lhote F.
        • et al.
        A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis.
        Arthritis Rheum. 1997; 40: 2187-2198
        • Kato A.
        • Hamada T.
        • Miyake T.
        • et al.
        Clinical and laboratory markers associated with relapse in cutaneous polyarteritis nodosa.
        JAMA Dermatol. 2018; 154: 922-926
        • Daoud M.S.
        • Hutton K.P.
        • Gibson L.E.
        Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases.
        Br J Dermatol. 1997; 136: 706-713
        • Buffiere-Morgado A.
        • Battistella M.
        • Vignon-Pennamen M.-D.
        • et al.
        Relationship between cutaneous polyarteritis nodosa (cPAN) and macular lymphocytic arteritis (MLA): blinded histologic assessment of 35 cPAN cases.
        J Am Acad Dermatol. 2015; 73: 1013-1020
        • Chen K.R.
        Cutaneous polyarteritis nodosa: a clinical and histopathological study of 20 cases.
        J Dermatol. 1989; 16: 429-442
        • Flanagan N.
        • Casey E.B.
        • Watson R.
        • Barnes L.
        Cutaneous polyarteritis nodosa with seronegative arthritis.
        Rheumatology. 1999; 38: 1161-1162