Auto-immunity and Pulmonary Arterial Hypertension

NCT01208792 | Completed

• 1 other identifier: P071209
• Study Type: interventional
• Target: 629
• Locations: 1 country
• Sites: 3

Brief Summary

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

Conditions

Pulmonary Arterial Hypertension; HIV Infection; Congenital Heart Defect; Systemic Sclerosis; Connective Tissue Disease

Keywords

Auto antibodies; Target antigens; 2D-immunoblot; Reactive oxygen species; Pulmonary arterial hypertension; Systemic sclerosis; Human immunodeficiency virus infection; Porto pulmonary hypertension; Congenital heart defect; Systemic lupus erythematosus; Mixed connective tissue disease; Sjögren's syndrome

Outcome Measures

Primary Outcomes (1)

• Immunological markers of prognosis interest in pulmonary arterial hypertension (PAH)

  one year

Secondary Outcomes (2)

• Target antigens of autoantibodies

  one year

• Subpopulations of patients with PAH whose serum is able to induce the production of reactive oxygen species (ROS)

  one year

Study Arms (3)

Disease group

EXPERIMENTAL

Two 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.

Procedure: skin biopsy; Other: Blood Sample

Control group 1

OTHER

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

Other: Blood Sample

Control group 2

OTHER

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

Other: Blood Sample

Interventions

skin biopsy PROCEDURE

The 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

Disease group

Blood Sample OTHER

a blood sample will be collected

Control group 1; Control group 2; Disease group

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• 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

You may not qualify if:

• age under 18
• pregnant women
• absence of written informed consent
• associated malignant tumor

Sponsors & Collaborators

• Assistance Publique - Hôpitaux de Paris: lead
• URC-CIC Paris Descartes Necker Cochin: collaborator

Study Sites (3)

Pneumology Department, Antoine Béclère Hospital

Clamart, 92000, France

Location

Internal Medicine Department, Claude Huriez Hospital

Lille, 59000, France

Location

Internal Medicine Department, Cochin Hospital

Paris, 75014, France

Location

MeSH Terms

Conditions

Pulmonary Arterial Hypertension; HIV Infections; Heart Defects, Congenital; Scleroderma, Systemic; Connective Tissue Diseases; Acquired Immunodeficiency Syndrome; Lupus Erythematosus, Systemic; Mixed Connective Tissue Disease; Sjogren's Syndrome

Interventions

Blood Specimen Collection

Condition Hierarchy (Ancestors)

Hypertension, Pulmonary; Lung Diseases; Respiratory Tract Diseases; Blood-Borne Infections; Communicable Diseases; Infections; Sexually Transmitted Diseases, Viral; Sexually Transmitted Diseases; Lentivirus Infections; Retroviridae Infections; RNA Virus Infections; Virus Diseases; Genital Diseases; Urogenital Diseases; Immunologic Deficiency Syndromes; Immune System Diseases; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Skin and Connective Tissue Diseases; Skin Diseases; Slow Virus Diseases; Autoimmune Diseases; Arthritis, Rheumatoid; Arthritis; Joint Diseases; Musculoskeletal Diseases; Rheumatic Diseases; Xerostomia; Salivary Gland Diseases; Mouth Diseases; Stomatognathic Diseases; Dry Eye Syndromes; Lacrimal Apparatus Diseases; Eye Diseases

Intervention Hierarchy (Ancestors)

Specimen Handling; Clinical Laboratory Techniques; Diagnostic Techniques and Procedures; Diagnosis; Punctures; Surgical Procedures, Operative; Investigative Techniques

Study Officials

• Luc Mouthon, MD, PhD

  Assistance Publique Hopitaux de Paris

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NON RANDOMIZED
• Masking: NONE
• Purpose: DIAGNOSTIC
• Intervention Model: FACTORIAL
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: July 12, 2010
• First Posted: September 24, 2010
• Study Start: June 15, 2010
• Primary Completion: July 15, 2014
• Study Completion: May 15, 2017
• Last Updated: March 27, 2026

Record last verified: 2026-03

Locations

FR (3)