Treatment of pulmonary hypertension secondary to connective tissue diseases.

Pulmonary hypertension has been identified as one of the well known life threatening complications of connective tissue diseases. In patients with connective tissue diseases pulmonary hypertension occurs with little or no evidence of parenchymal lung disease. As in primary pulmonary hypertension, the pathophysiology is unknown but the clinical course and pathological findings are similar. Scleroderma, particularly in its CREST variant (calcinosis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasias), represents the main connective tissue disease associated with pulmonary hypertension. Prevalence varies from 2.3% to 35% in scleroderma and may reach 50% in the CREST variant. Pulmonary hypertension occurs in 23–53% of patients with mixed connective tissue diseases, in 0.5–14% of cases of systemic lupus erythematosus, and much more rarely in those with rheumatoid arthritis, Sjögren’s syndrome, and dermatopolymyositis. Connective tissue diseases represented around 10% of the 601 cases of pulmonary hypertension referred to our department from 1981 to 1998. Prognosis is very poor and no curative treatment is available. Stupi et al reported a two year survival rate of 40% in patients with CREST syndrome and pulmonary hypertension compared with 88% in patients with CREST syndrome without pulmonary hypertension. There are no consensus guidelines for the treatment of pulmonary hypertension secondary to connective tissue diseases. Treatment recommendations, modelled on those of primary pulmonary hypertension, enable us to propose a specific treatment for these patients who were once considered untreatable. Because of the specificity of pulmonary hypertension evaluation and monitoring it is necessary to refer these patients to a centre with expertise in the management of pulmonary vascular diseases. Indeed, the risk of sudden death exists in these patients and the treatment itself can be dangerous, particularly the high doses of calcium channel blockers which may precipitate heart failure because of their negative inotropic properties.