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The Clinical Spectrum and Therapeutic Management of Hypocomplementemic Urticarial Vasculitis: Data From a French Nationwide Study of Fifty‐Seven Patients

Marie Jachiet 1, 2, 3 Béatrice Flageul 4 Alban Deroux 5 Alain Le Quellec 6, 7 François Maurier 8 Florence Cordoliani 4 Pascal Godmer Claire Abasq 9 Leonardo Astudillo 10 Pauline Belenotti 11 Didier Bessis 6 Adrien Bigot 12 Marie-Sylvie Doutre 13 Mikael Ebbo 11 Isabelle Guichard 14 Eric Hachulla 15 Emmanuel Heron 16 Géraldine Jeudy 17 Noemie Jourde-Chiche 18 Denis Jullien 19 Christian Lavigne 20 Laurent Machet 21 Marie-Alice Macher 22 Clotilde Martel 23 Sara Melboucy-Belkhir 24 Cécile Morice 25 Antoine Petit 26 Bernard Simorre Thierry Zénone 27 Laurence Bouillet 28 Martine Bagot 26 Véronique Frémeaux-Bacchi 29, 3 Loïc Guillevin 30, 1, 3 Luc Mouthon 30, 1, 3 Nicolas Dupin 1, 3 Selim Aractingi 1, 3 Benjamin Terrier 30, 1, 3
Abstract : OBJECTIVE: Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology that is rarely described in the literature. We undertook this study to analyze the clinical spectrum and the therapeutic management of patients with HUV. METHODS: We conducted a French nationwide retrospective study that included 57 patients with chronic urticaria, histologic leukocytoclastic vasculitis, and hypocomplementemia. We assessed clinical and laboratory data and evaluated the patients' cutaneous and immunologic responses to therapy. We evaluated treatment efficacy by measuring the time to treatment failure. RESULTS: Urticarial lesions were typically more pruritic than painful and were associated with angioedema in 51% of patients, purpura in 35%, and livedo reticularis in 14%. Extracutaneous manifestations included constitutional symptoms (in 56% of patients) as well as musculoskeletal involvement (in 82%), ocular involvement (in 56%), pulmonary involvement (in 19%), gastrointestinal involvement (in 18%), and kidney involvement (in 14%). Patients with HUV typically presented with low C1q levels and normal C1 inhibitor levels, in association with anti-C1q antibodies in 55% of patients. Hydroxychloroquine or colchicine seemed to be as effective as corticosteroids as first-line therapy. In patients with relapsing and/or refractory disease, rates of cutaneous and immunologic response to therapy seemed to be higher with conventional immunosuppressive agents, in particular, azathioprine, mycophenolate mofetil, or cyclophosphamide, while a rituximab-based regimen tended to have higher efficacy. Finally, a cutaneous response to therapy was strongly associated with an immunologic response to therapy. CONCLUSION: HUV represents an uncommon systemic and relapsing vasculitis with various manifestations, mainly, musculoskeletal and ocular involvement associated with anti-C1q antibodies, which were found in approximately half of the patients. The best strategy for treating HUV has yet to be defined.
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Soumis le : vendredi 19 juillet 2019 - 11:53:40
Dernière modification le : mercredi 19 août 2020 - 12:08:22

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Marie Jachiet, Béatrice Flageul, Alban Deroux, Alain Le Quellec, François Maurier, et al.. The Clinical Spectrum and Therapeutic Management of Hypocomplementemic Urticarial Vasculitis: Data From a French Nationwide Study of Fifty‐Seven Patients. Arthritis & rheumatology, Wiley, 2015, 67 (2), pp.527-534. ⟨10.1002/art.38956⟩. ⟨hal-02189276⟩



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