Pulmonary hypertension is increasingly recognized as a complication of the collagen
vascular diseases. Both isolated and secondary forms of the disease may be appreciated.
Pulmonary hypertension is observed with high prevalence in scleroderma and mixed connective
tissue disease. It is less commonly seen in systemic lupus erythematosus, and is a
rare clinical finding in rheumatoid arthritis. The diagnosis and treatment of pulmonary
hypertension associated with the collagen vascular diseases mirror diagnosis and treatment
for pulmonary hypertension of any etiology. Vasodilator therapy may play a particularly
important role in patients with the isolated form of disease, which is most frequently
associated with scleroderma.
Pulmonary hypertension - collagen vascular disease - scleroderma - mixed connective
tissue disease - systemic lupus erythematosus