Learn More
BACKGROUND Common variable immunodeficiency (CVID) is a primary immune deficiency defined by defective antibody production. In most series, a small proportion of patients present with opportunistic infections (OIs). METHODS The French DEFI study has enrolled patients with primary hypogammaglobulinemia and allows a detailed clinical and immunologic(More)
OBJECTIVE To describe the history of SSc-associated pulmonary arterial hypertension (SSc-PAH) in patients with New York Heart Association (NYHA) functional class (FC) II dyspnoea at diagnosis. METHODS Data at the time of diagnosis were collected and analysed retrospectively for 77 consecutive patients with SSc-PAH. RESULTS Twelve patients (15.6%)(More)
Anti-endothelial cell antibodies (AECAs) have been identified in patients with systemic sclerosis (SSc) with and without pulmonary arterial hypertension (PAH) and in patients with idiopathic pulmonary arterial hypertension (iPAH). However, their target antigens remain poorly identified. Sera from 24 patients with SSc without PAH, 20 patients with SSc with(More)
Granulomatous disease (GD) will develop in a subset of patients with common variable immunodeficiency (CVID). Little is known about the efficacy of therapeutic agents used for treating this disorder. To evaluate the efficacy of immunosuppressive drugs with the help of a set of clinical, biological and radiological criteria. Clinical and laboratory features(More)
Multicentric Castleman disease (MCD) is a distinct lymphoprolif-erative disorder characterized by lymphadenopathy with angiofol-licular hyperplasia and plasma cell infiltration. Patients typically have fever associated with lymphadenopathy, hepatosplenomegaly, respiratory symptoms, peripheral edema, cytopenia, hypergamma-globulinemia, hypoalbuminemia, and(More)
The DEFI study has collected clinical data and biological specimens from kindreds with CVID. Patients with demonstrated parental consanguinity (cCVID group) were compared to patients without parental consanguinity (ncCVID). A total of 24 of the 436 patients with CVID had consanguineous parents. Age at first symptoms and age at diagnosis were comparable in(More)
OBJECTIVES To assess the value of major histocompatibility complex (MHC) class II antigen (HLA-DR) expression to distinguish anti-synthetase myopathy (ASM) from dermatomyositis (DM). METHODS Muscle biopsies from patients with ASM (n = 33), DM without anti-synthetase antibodies (ASAb) (n = 17), and normal muscle biopsy (n = 10) were first reviewed. ASAb(More)
OBJECTIVE To assess the effect of sildenafil, a phosphodiesterase type 5 inhibitor, on digital ulcer (DU) healing in systemic sclerosis (SSc). METHODS Randomised, placebo-controlled study in patients with SSc to assess the effect of sildenafil 20 mg or placebo, three times daily for 12 weeks, on ischaemic DU healing. The primary end point was the time to(More)
OBJECTIVES To assess the association of gender with clinical expression, health-related quality of life (HRQoL), disability, and self-reported symptoms of depression and anxiety in patients with systemic sclerosis (SSc). METHODS SSc patients fulfilling the American College of Rheumatology and/or the Leroy and Medsger criteria were assessed for clinical(More)
OBJECTIVES To identify clinical, functional and health-related quality of life (HRQoL) correlates of clinically significant symptoms of anxiety and depression in patients with systemic sclerosis (SSc). METHODS Three-hundred-and-eighty-one patients fulfilling the American College of Rheumatology and/or the Leroy and Medsger criteria for SSc were assessed(More)