Anti-fibroblast and anti-endothelial cell autoantibodies in pulmonary arterial hypertension (PAH) in patients with connective tissue diseases (CTD).

Objectives: Pulmonary arterial hypertension (PAH) is a rare disease that may be associated with connective tissue diseases (CTD). Anti-fibroblast (AFA) and anti-endothelial cell autoantibodies (AECA) have been identified in idiopathic and systemic sclerosis (SSc)-associated PAH. The aim was to identify autoantibodies discriminating for PAH associated with systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD) and primary Sjögren's syndrome (SS), and their target antigens.

Job-exposure matrix (JEM) validity on crystalline silica among systemic sclerosis patients.

Background: Systemic sclerosis (SSc) is the connective tissue disease with the highest individual mortality. Crystalline silica is known to be an occupational risk factor for SSc. To assess past crystalline silica exposure, we aimed to study the validity of a job exposure matrix (JEM) to assess occupational exposure to crystalline silica compared to specific occupational interviews in two populations of SSc patients.

Avacopan for anti-neutrophil cytoplasm antibodies-associated vasculitis: a multicenter real-world study.

Objectives: Avacopan, a selective C5aR1 inhibitor, recently emerged as a glucocorticoid (GCs) sparing agent in ANCA-associated vasculitis (AAV). We aim to evaluate the tolerance and efficacy of avacopan given outside randomized clinical trials or with severe kidney involvement.

Methods: In this multicentre retrospective study, we reviewed the clinical charts of patients with AAV and contraindication to high dose of GCs who received avacopan 30 mg b.

High Prevalence of the Lung Ultrasound Interstitial Syndrome in Systemic Sclerosis Patients with Normal HRCT and Lung Function-A Pilot Study.

High-resolution computed tomography (HRCT) may lack sensitivity for the early detection of interstitial lung disease associated with systemic sclerosis (SSc-ILD). Lung ultrasound is an emerging technique for the diagnosis of SSc-ILD. This cross-sectional study aimed to describe the prevalence of ultrasound interstitial syndrome in SSc patients with normal HRCT and pulmonary function tests (PFT).

Anti-U1RNP antibodies are associated with a distinct clinical phenotype and a worse survival in patients with systemic sclerosis.

Objectives: To clarify the impact of anti-U1RNP antibodies on the clinical features and prognosis of patients with SSc.

Methods: We conducted a monocentric case-control, retrospective, longitudinal study. For each patient with SSc and anti-U1RNP antibodies (SSc-RNP), one patient with mixed connective tissue disease (MCTD) and 2 SSc patients without anti-U1RNP antibodies (SSc-RNP) were matched for age, sex, and date of inclusion.