Autoimmunity and pulmonary hypertension: a perspective

• Published in: The European respiratory journal Vol. 26; no. 6; pp. 1110 - 1118
• Main Authors: Nicolls, M. R, Taraseviciene-Stewart, L, Rai, P. R, Badesch, D. B, Voelkel, N. F
• Format: Journal Article
• Language: English
• Published: Leeds Eur Respiratory Soc 01.12.2005; Maney
• Subjects: Adult; Age Distribution; Aged; Autoimmune Diseases - diagnosis; Autoimmune Diseases - epidemiology; Autoimmunity - immunology; Biological and medical sciences; Biopsy, Needle; Comorbidity; Female; Humans; Hypertension, Pulmonary - epidemiology; Hypertension, Pulmonary - immunology; Hypertension, Pulmonary - pathology; Immunohistochemistry; Male; Medical sciences; Middle Aged; Pneumology; Prevalence; Prognosis; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Risk Assessment; Severity of Illness Index; Sex Distribution; Survival Analysis; Autoimmunity; Auto-antibodies; Respiratory disease; Antibody; Immune tolerance; Cardiovascular disease; Immune regulation; Pulmonary hypertension; Endothelium
• ISSN: 0903-1936; 1399-3003
• DOI: 10.1183/09031936.05.00045705

The association between autoimmunity and pulmonary arterial hypertension (PAH) has been appreciated for >40 yrs, but how autoimmune injury might contribute to the pathogenesis of this disease has only been examined in a case-specific manner. It is becoming increasingly clear that a variety of diverse clinical diseases, ranging from viral infections to connective tissue disorders, can culminate in pulmonary vascular pathology that is indistinguishable. Is there a hitherto unappreciated biology that unites these seemingly unrelated conditions? The answer to this question may come from the increasing body of evidence concerned with the central importance of regulatory T-cells in preventing inappropriate B-cell activity. Two striking similarities between conditions associated with severe angioproliferative pulmonary hypertension are a defect in the CD4 T-cell compartment and auto-antibody production. Pathogenic auto-antibodies targeting endothelial cells are capable of inducing vascular endothelial apoptosis and may initiate the development of PAH. The present review will focus on what is known about autoimmune phenomena in pulmonary arterial hypertension patients, in order to better consider whether an early loss of self-tolerance followed by autoimmune injury could influence the early development of severe angioproliferative pulmonary hypertension.