Assistance Publique - Hôpitaux de Paris Paris, France
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Lucas Maisonobe1, Anne-Sophie Korganow2, Alvise Berti3, Alban DEROUX4, Nicolas Dupin5, Selim Aractingi5, Giacomo Emmi6, Frédéric Vandergheynst7, Marc Fabre8, Nicolas Kluger9, Marielle Roux8, Noemie Abisror10, Audrey Benyamine11, Giulia Cassone12, Francois Chasset13, Marie-Sylvie Doutre14, Aurelie Foucher15, Caroline Freguin16, Delphine Gobert17, Yannick Gombeir7, José Hernandez-Rodriguez18, Noémie Legouellec19, Anne-Lise Pinault20, Dimitri Titeca-Beauport21, Marie Jachiet22 and Benjamin Terrier23, 1Assistance Publique - Hopitaux de Paris, Paris, France, 2Strasbourg University Hospital, Strasbourg, France, 3University of Trento, Rochester, MN, 4CHU de Grenoble, Grenoble, France, 5CHU Cochin, Paris, France, 6University of Florence, Florence, Italy, 7Université Libre de Bruxelles, Bruxelles, Belgium, 8Centre Hospitalier Pierre Oudot, Bourgoin Jallieu, France, 9Helsinki University Central Hospital, Helsinki, Finland, 10Internal Medicine Saint Antoine Hospital, Paris, France, 11Hopital Nord Marseille, Marseille, France, 12Universita degli Studi di Modena e Reggio, Modena, Italy, 13Dermatology Department, Tenon Hospital, Paris, France, 14CHU Bordeaux, Bordeaux, France, 15CHU de la Réunion, Saint pierre, France, 16Centre Hospitalier Saint-Brieuc, Saint Brieux, France, 17CHU Sant-Atoine, Paris, France, 18Hospital Clinic de Barcelona, Barcelona, Spain, 19Centre Hospitalier de Valenciennes, Valenciennes, France, 20Centre Hospitalier Epinal, Epinal, France, 21CHU Amiens, Amiens, France, 22CHU Saint-Louis, Paris, France, 23Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France
Background/Purpose: Urticarial vasculitis (UV) is characterized by atypical urticarial lesions and leukocytoclastic vasculitis, sometimes with extra-cutaneous manifestations. First-line treatment is often based on colchicine, hydroxychloroquine, dapsone or low-dose glucocorticoids. In refractory forms, the use of biologics has been anecdotally described as potentially effective. We aimed to describe the efficacy and safety of biologics in patients with UV.
Methods: We conducted a retrospective European multicentre study including patients with hypocomplementemic (HUV) or normocomplementemic (NUV) UV who received at least one biologic, including anti-CD20, anti-IgE and/or anti-IL-1. We analyzed the clinical and biological characteristics as well as the efficacy and safety of the biologics.
Results: Forty-one patients were analyzed, including 24 patients with HUV and 17 with NUV.Fifty-two therapeutic sequences were recorded, including rituximab in 23, anti-IL1 in 16 and anti-IgE in 13 cases. Extra-cutaneous manifestations were present in 90%. The most common manifestations were arthralgia in 66%, arthritis in 27%, ocular inflammation in 24%, gastrointestinal involvement in 15%, renal involvement in 15% and pulmonary involvement in 12%.
The median number of treatments before biologic was 4 (IQR 3-6). A higher proportion of HUV was observed in patients treated with anti-CD20 (83%), while a higher proportion of NUV was observed in patients treated with anti-IL-1 (62%) and omalizumab (77%). All patients with renal involvement were treated with anti-CD20.
Biologics were used in combination with glucocorticoids in 75% of patients, with a median prednisone dose of 18 mg/day (IQR 10-39), hydroxychloroquine in 25%, dapsone in 6% and azathioprine in 8%.
After a median follow-up of 25 (IQR 12-43) months, the cutaneous response was complete in 40%, partial in 37% and considered inadequate in 23%. Extracutaneous response was similar to cutaneous response in 84%. Prednisone dose reduction to < 10 mg/day was achieved in 72% of patients, including discontinuation of glucocorticoids in 34%. Despite different disease profiles for each biologic, cutaneous and global clinical response rates were broadly comparable across all biologics.
Severe adverse events, mainly serious infectious, were observed in 7 patients (17%), including 4 patients treated with rituximab and 3 patients treated with anakinra.
Conclusion: Rituximab, anti-IL-1 and omalizumab are an effective and well-tolerated treatment option for UV that are refractory to conventional therapies. Rituximab was mainly used for HUV while anakinra and omalizumab were mainly used for NUV.
L. Maisonobe: None; A. Korganow: None; A. Berti: None; A. DEROUX: None; N. Dupin: None; S. Aractingi: None; G. Emmi: AstraZeneca, 2, Boehringer-Ingelheim, 2, GlaxoSmithKlein(GSK), 2, Novartis, 2, Sanofi, 2, Sobi, 2; F. Vandergheynst: None; M. Fabre: None; N. Kluger: None; M. Roux: None; N. Abisror: None; A. Benyamine: None; G. Cassone: None; F. Chasset: None; M. Doutre: None; A. Foucher: None; C. Freguin: None; D. Gobert: None; Y. Gombeir: None; J. Hernandez-Rodriguez: None; N. Legouellec: None; A. Pinault: None; D. Titeca-Beauport: None; M. Jachiet: None; B. Terrier: AstraZeneca, 5, CSL Vifor, 2, GlaxoSmithKlein(GSK), 2.
