No abstract

No abstract

No abstract

We report the case of a 74-year-old woman admitted to the emergency unit for resting dyspnea. Clinical presentation, cardiac MRI and echocardiography were consistent with cardiac tamponade requiring emergency pericardiocentesis. Cytologic examination of the pericardial fluid revealed the presence of CD68 CD1a S100 foamy histiocytes (Fig. 1). Additional investigations complied with the diagnosis of Erdheim-Chester histiocytosis. Treatment with αIFN was initiated but the patient developed severe neutropenia that contraindicated further administration. The detection of BRAF mutation on histiocytes isolated from the pericardial liquid and CNS involvement (cerebral masses) prompted the administration of vemurafenib, a selective BRAF kinase inhibitor. Four months after the initiation of low-dose vemurafenib, pericarditis almost resolved and cerebral masses decreased by 50% (Fig. 2). To our knowledge, analysis of pericardial fluid allowing the diagnosis of Erdheim-Chester disease and the detection of the BRAF mutation has in fact been rarely described in the literature. This case report and the successful evolution under vemurafenib also support the use of BRAF inhibitors in αIFN-intolerant patients with BRAF mutation, particularly in case of heart and central nervous system involvement.

BackgroundThe RAVE trial has revolutionised the induction treatment of ANCA-Associated Vasculitis (AAV, including GPA, granulomatosis with polyangiitis and MPA, microscopic polyangiitis), by demonstrating non-inferiority of rituximab compared with cyclophosphamide1.ObjectivesTo study AAV patients’ characteristics, rituximab prescription practices and efficacy in AAV induction treatment in 4 Belgian university hospitals. The patient population, selected according to the Belgian reimbursement criteria, is relatively homogeneous and comparable to the one of RAVE trial.Methods57 patients, receiving rituximab as AAV induction therapy since May 2014, were enrolled in an observational retrospective multicenter trial involving 4 Belgian university hospitals. We have focused on the type of AAV (GPA/MPA), ANCA specificity (anti-PR3/MPO), prescriber’s speciality, used reimbursement criteria, organ involvements, severity of the flares (according to BVAS-WG definition) and finally rituximab efficacy in AAV induction treatment by considering the RAVE primary (complete remission without prednisone) and secondary (complete remission with prednisone <10 mg, rates of relapses) outcomes at 6, 12, 18 and 24 months.ResultsThe most frequent subtype of AAV was GPA (84%). The main indication was relapsing disease (54.4%), followed by contra-indication to cyclophosphamide (38.6%). 66.7% of the patients reached complete remission with prednisone <10 mg at 6 months, and 55.3% at 12 months, 40% at 18 months, 25% at 24 months respectively. In the “severe disease” subgroup, 73% reached complete remission with prednisone <10 mg at 6 months, 58.8% at 12 months, 50% at 18 months and 32% at 24 months. The rates of complete remission without steroids were very low at 6, 12, 18 and 24 months (between 0%–6%) but our patients were not asked to follow a glucocorticoid tapering scheduled for complete withdrawal of prednisone after 6 months and were usually maintained under low-dose prednisone. Relapse rates were high between 18 and 24 months both in the total group and in the severe disease subgroup (due to the fact that rituximab is not reimbursed for maintenance treatment in Belgium). The subtype of ANCA was not predictive of the risk of relapse.ConclusionsOur results confirm – in a “real-life” cohort of patients selected according data of RAVE trial – those of RAVE regarding complete remission rates at 6 months with prednisone 10 mg/j. The high prevalence of relapses – in particular after 18 months – underlines the need to optimise maintenance treatment after an induction treatment with rituximab.Reference[1] Specks U, Merkel PA, Seo P, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med2013:369(5):417–27.Disclosure of InterestNone declared