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Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension

¹ Paris-Descartes University, Faculty of Medicine, UPRES-EA 1833, ³ Dermatology Dept, ⁵ Internal Medicine Dept, ⁶ French National Reference Centre for Necrotizing Vasculitis and Systemic Sclerosis, Cochin Hospital, and ⁴ Paris-Descartes University, Dept of Biostatistics, Necker Hospital, Assistance Publique–Hôpitaux de Paris (AP–HP), Paris, and ² UPRES-EA 2705, Pneumology Dept and French National Reference Centre for Pulmonary Hypertension, Antoine Béclère Hospital, AP–HP and Paris-Sud University, Clamart, France.

CORRESPONDENCE: L. Mouthon, Laboratoire d'Immunologie, Pavillon Gustave Roussy, 4e étage, Paris-Descartes University, 8 rue Méchain, 75014, Paris, France. Fax: 33 0144412546. E-mail: luc.mouthon{at}cch.aphp.fr

Keywords: Anti-fibroblast antibodies, pulmonary arterial hypertension, systemic sclerosis

Received: December 28, 2005
Accepted May 29, 2006

The aim of the present study was to investigate the presence of anti-fibroblast antibodies in patients with idiopathic or scleroderma-associated pulmonary arterial hypertension (PAH) and healthy controls.

PAH was documented by right-heart catheterisation (mean pulmonary artery pressure at rest >25 mmHg). Serum immunoglobulin (Ig)G and IgM reactivities of patients with idiopathic PAH (n = 35), scleroderma-associated PAH (n = 10), diffuse (n = 10) or limited cutaneous (n = 10) scleroderma without PAH and age- and sex-matched healthy individuals (n = 65) were analysed by cell-based ELISA and immunoblotting on normal human fibroblasts.

As assessed by ELISA, 14 out of 35 (40%) patients with idiopathic PAH and three out of 10 (30%) patients with scleroderma-associated PAH expressed anti-fibroblast IgG antibodies. IgG from all individuals bound to one major 40-kDa protein band. IgG from patients with idiopathic PAH bound to two 25- and 60-kDa bands with a higher intensity than IgG from other individuals.

In conclusion, immunoglobulin G anti-fibroblast antibodies are present in the serum of patients with pulmonary arterial hypertension. Immunoglobulin G from patients with idiopathic pulmonary arterial hypertension or scleroderma-associated pulmonary arterial hypertension express distinct reactivity profiles with fibroblasts antigens, suggesting distinct target antigens.

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