Pulmonary Arterial Hypertension | Auto-immunity and Pulmonary Arterial Hypertension

Pulmonary Arterial Hypertension research study

What is the primary objective of this study?

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

Who is eligible to participate?

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

Which medical condition, disease, disorder, syndrome, illness, or injury is researched?

Pulmonary Arterial Hypertension

HIV Infection

Congenital Heart Defect

Systemic Sclerosis

Connective Tissue Disease

Study Interventions

Interventions can include giving participants drugs, medical devices, procedures, vaccines, and other products that are either investigational or already available or noninvasive approaches such as surveys, education, and interviews.

Procedure:skin biopsyThe biopsy site (usually the forearm) will be first cleaned, and then anesthetized with pain relieving (spray, cream, or injection). The skin is then sampled using a punch that takes a core (a small cylindrical fragment of tissue from the area of interest

Other:Blood Samplea blood sample will be collected

Study Arms

Research studies and clinical trials typically have two or more research arms. An arm is a group of people who receive the same treatment in the study.

Disease groupTwo hundred patients with PAH will be included: 50 patients with idiopathic PAH (iPAH), 20 with PAH associated with HIV infection, 20 with porto-pulmonary hypertension, 20 with PAH secondary to congenital heart disorders, 40 with SSc, 20 with SLE, 20 with MCTD and 10 with a PAH associated with a Sjögren's syndrome. Two hundred patients without PAH will also be included: 80 patients with SSc and 20 in each of the following groups: HIV infection, porto-pulmonary hypertension, SLE, congenital heart disorders, MCTD and with Sjögren's syndrome.

Control group 1Two hundred healthy blood donors age and sex-matched with patients with PAH, will be included as controls.

Control group 2Twenty patients with proximal chronic thromboembolic pulmonary hypertension (CTPH) will also be included in a control arm of the study.

Study Status


Start Date: June 15, 2010

Completed Date: May 15, 2017

Phase: N/A

Type: Interventional


Primary Outcome: Immunological markers of prognosis interest in pulmonary arterial hypertension (PAH)

Secondary Outcome: Target antigens of autoantibodies

Study sponsors, principal investigator, and references

Principal Investigator: Luc Mouthon, MD, PhD

Lead Sponsor: Assistance Publique - Hôpitaux de Paris


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