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Sponsor: Assistance Publique - Hôpitaux de Paris
Conditions: Pulmonary Arterial Hypertension, HIV Infection, Congenital Heart Defect, Systemic Sclerosis, Connective Tissue Disease
Interventions: skin biopsy, Blood Sample
Countries: France
The investigators have recently evidenced the presence of antibodies to endothelial cells and fibroblasts in patients with idiopathic or SSc-associated PAH. The investigators also have identified several target antigens of anti-fibroblasts antibodies. The objective of this study is to further investigate for the presence of antibodies to endothelial cells and fibroblasts in patients and characterize the antigen specificity of autoantibodies in patients with different types of non idiopathic and non SSc-associated PAH, such as PAH associated with HIV infection, porto-pulmonary hypertension, congenital heart diseases, systemic lupus erythematosus, mixed connective tissue disease and Sjögren's syndrome
Sex: ALL
Age: 18 Years to —
Healthy volunteers: Yes
Study type: INTERVENTIONAL
Inclusion Criteria: age over 18 * for PAH patients: pre-capillary PAH evidenced by right-heart catheterization * no associated systemic disease for idiopathic PAH patients * for HIV patients, HIV1 infection confirmed by ELISA and western blot * for patients with porto pulmonary hypertension: evidence by endoscopy of esophageal varices, confirmation of hepatic venous pressure gradient over 5 mmHg by catheterization of the hepatic veins * for patients with congenital heart defect: evidence by imaging of atrial or ventricular septal defect, or patent ductus arterious and confirmed by heart catheterization * patients with SSc will fulfill the American College of Rheumatology (ACR) and the LEROY and MEDSGER criteria * patients with MCTD will fulfill the criteria for MCTD * patients with SLE will fulfill the updated and revised ACR criteria * patients with Sjögren's syndrome will fulfill the American-European consensus group criteria * patients with chronic thromboembolic pulmonary hypertension: Lung scintiscan showing segmental mismatched perfusion defects and confirmation by angiography of the occlusion and the chance of success of endarterectomy according to the location of disease * Signed written informed consent * Patients with health insurance Exclusion Criteria: * age under 18 * pregnant women * absence of written informed consent * associated malignant tumor
- Clamart, France
- Lille, France
- Paris, France