Antibodies to fibroblasts in idiopathic and scleroderma associated pulmonary hypertension

¹ Paris-Descartes University, Faculty of Medicine, UPRES-EA 1833, Cochin Hospital, Paris, France
² UPRES-EA 2705, Pneumology Dept and French National Reference Center for pulmonary hypertension, Antoine Béclère Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP) and Paris-Sud University, Clamart, France
³ Dermatology dept, Cochin Hospital, AP-HP, Paris
⁴ Dept of Biostatistics, Necker Hospital, AP-HP, Paris-Descartes University
⁵ Paris-Descartes University, Faculty of Medicine, UPRES-EA 1833, Cochin Hospital, Paris, France; and Paris-Descartes University, Faculty of Medicine, Internal Medicine Dept and French National Reference Center for necrotizing vasculitis and systemic sclerosis, Cochin Hospital

^* To whom correspondence should be addressed. E-mail: luc.mouthon{at}cch.aphp.fr.

	Abstract

To test the presence of anti-fibroblast antibodies in patients with idiopathic or scleroderma associated pulmonary arterial hypertension and healthy controls.

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

As assessed by ELISA, 14/35 (40%) patients with idiopathic pulmonary arterial hypertension and 3/10 (30%) patients with scleroderma associated pulmonary arterial hypertension expressed anti-fibroblast IgG antibodies. IgG from all individuals bound to one major 40 kDa protein band. IgG from patients with idiopathic pulmonary arterial hypertension bound to two 25 and 60 kDa bands with a higher intensity than IgG from other individuals.

IgG anti-fibroblast antibodies are present in the serum of patients with pulmonary arterial hypertension. IgG 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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