Off-label Use of Biologics in Urticarial Vasculitis: A European Cohort Study

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

Meeting: ACR Convergence 2023

Keywords: Biologicals, Vasculitis, Cutaneous

Session Information

Date: Sunday, November 12, 2023

Title: (0691–0721) Vasculitis – Non-ANCA-Associated & Related Disorders Poster I

Session Type: Poster Session A

Session Time: 9:00AM-11:00AM

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.

Disclosures: 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.

To cite this abstract in AMA style:

Maisonobe L, Korganow A, Berti A, DEROUX A, Dupin N, Aractingi S, Emmi G, Vandergheynst F, Fabre M, Kluger N, Roux M, Abisror N, Benyamine A, Cassone G, Chasset F, Doutre M, Foucher A, Freguin C, Gobert D, Gombeir Y, Hernandez-Rodriguez J, Legouellec N, Pinault A, Titeca-Beauport D, Jachiet M, Terrier B. Off-label Use of Biologics in Urticarial Vasculitis: A European Cohort Study [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/off-label-use-of-biologics-in-urticarial-vasculitis-a-european-cohort-study/. Accessed .

« Back to ACR Convergence 2023

ACR Meeting Abstracts - https://acrabstracts.org/abstract/off-label-use-of-biologics-in-urticarial-vasculitis-a-european-cohort-study/