[Acquired autoimmune haemophilia: Where is the place of rituximab in the treatment strategy? Reflection from a monocentric series of 8 patients and literature review]. / Hémophilie acquise auto-immune : quelle est la place du rituximab dans la stratégie thérapeutique ? Réflexion à partir d'une série monocentrique de 8 patients et revue de la littérature.

INTRODUCTION: Autoimmune acquired haemophilia is a rare autoimmune disease. The purpose of immunosuppressive therapy is to stop the production of autoantibodies that inhibit clotting factors VIII or IX. A corticosteroids-cyclophosphamide combination is recommanded as first-line therapy. From our experience at the University Hospital of Nîmes, we discuss the place of rituximab in the therapeutic arsenal. METHODS: We report a monocentric observational retrospective study. Our data are discussed in light of literature data, in particular cohorts EACH2 and SACHA. RESULTS: Eight patients (7 with FVIII anibodies) were consecutively included from 2005. The average age was 68.5 years with a male predominance (62.5%). Bleeding manifestations were usually spontaneous and superficial. A pathology, mostly autoimmune or neoplastic, was associated in 5/8 patients. A "by-pass" haemostatic treatment was prescribed for 3/8 patients. Rituximab was prescribed for 5/8 patients, three times as first-line therapy, and always associated with corticosteroids. Three patients received a cyclophosphamid/cortisone combination, two were treated exclusively with oral corticosteroids. Remission was obtained in all patients, without subsequent relapse. The average time to obtain remission under rituximab (after the first injection) was 32.5 days (10-143). The results observed in our series of patients are consistent with the data from the literature. CONCLUSIONS: Rituximab appears to be an effective and well-tolerated treatment for autoimmune acquired haemophilia. However, its place remains to be specified.

[Efficacy and tolerance of tocilizumab for corticosteroid sparing in giant cell arteritis and aortitis: Experience of Nimes University Hospital about eleven patients]. / Efficacité et tolérance du tocilizumab en traitement d'épargne cortisonique dans l'artérite à cellules géantes et les aortites : expérience du centre hospitalier de Nîmes à propos de onze patients.

INTRODUCTION: Giant cell arteritis is a large-vessels vasculitis, which treatment consists in a slowly-tappered steroid-therapy. Immunosuppressive agents are sometimes used in case of steroid-dependance. We have conducted an observationnal retrospective study including patients treated with tocilizumab for a giant cell arteritis or an aortitis in the internal medicine department at the Nîmes University Hospital. RESULTS: Eleven patients were included between 2011 and 2016, who had been treated only with prednisone. Tocilizumab was used because of steroid-dependance for nine patients, delirium under steroids for one patient and unefficiency of steroids for an other patient. Infusions of tocilizumab, administred monthly at 8mg/kg, led to clinical and biological remission for all patients. Consequently, prednisone was tappered under 10mg/d for ten patients after six months of treatment with tocilizumab. Eight cases of non-severe infection were reported; also two cases of dyslipidemia, one case of prurit and one case of moderate neutropenia. Two relapses were observed after the end of treatment, in patients treated with less than twelve infusions. CONCLUSION: Tocilizumab could be efficient and well-tolerated in steroid-dependent giant cell arteritis and aortitis. The modalities of its use remain to be precised.