Efficacy and safety of rituximab-based treatments in angioedema with acquired C1 inhibitor deficiency. - Université de Lille Accéder directement au contenu
Article Dans Une Revue Journal of Allergy and Clinical Immunology: In Practice Année : 2023

Efficacy and safety of rituximab-based treatments in angioedema with acquired C1 inhibitor deficiency.

Kim Heang Ly
  • Fonction : Auteur
Marc Porneuf
  • Fonction : Auteur
Olivier Fain

Résumé

Angioedema (AE) due to acquired C1-inhibitor (C1-INH) deficiency (AAE–C1-INH) is related to excessive consumption of C1-INH or to anti–C1-INH antibodies, and is frequently associated with lymphoproliferative syndromes or monoclonal gammopathies. Standard of care for prophylactic treatment in this condition is not established. Rituximab may be effective to prevent attacks, especially if the lymphoid hemopathy is controlled, but data are scarce. Objective To evaluate efficacy of rituximab in AAE–C1-INH. Methods A retrospective multicenter study was carried out in France, including patients with AAE–C1-INH treated with rituximab between April 2005 and July 2019. Results Fifty-five patients with AAE–C1-INH were included in the study, and 23 of them had an anti–C1-INH antibody. A lymphoid malignancy was identified in 39 patients, and a monoclonal gammopathy in 9. There was no associated condition in 7 cases. Thirty patients received rituximab alone or in association with chemotherapy (n = 25). Among 51 patients with available follow-up, 34 patients were in clinical remission and 17 patients had active AE after a median follow-up of 3.9 years (interquartile range, 1.5-7.7). Three patients died. The presence of anti–C1-INH antibodies was associated with a lower probability of AE remission (hazard ratio, 0.29 [95% CI, 0.12-0.67]; P = .004). Relapse was less frequent in patients with lymphoma (risk ratio, 0.27 [95% CI, 0.09-0.80]; P = .019) and in patients treated with rituximab and chemotherapy (risk ratio, 0.31 [95% CI, 0.12-0.79]; P = .014). Conclusions Rituximab is an efficient and well-tolerated therapeutic option in AE, especially in lymphoid malignancies and in the absence of detectable anti–C1-INH antibodies.
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Dates et versions

hal-04481995 , version 1 (28-02-2024)

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Galith Kalmi, Yann Nguyen, Stephanie Amarger, Magali Aubineau, Beatrice Bibes, et al.. Efficacy and safety of rituximab-based treatments in angioedema with acquired C1 inhibitor deficiency.. Journal of Allergy and Clinical Immunology: In Practice, 2023, Journal of Allergy and Clinical Immunology: In Practice, ⟨10.1016/j.jaip.2023.10.017⟩. ⟨hal-04481995⟩
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