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Semin Respir Crit Care Med. 2003 Jun;24(3):287-96.

Pulmonary hypertension in the collagen vascular diseases.

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Yale University School of Medicine, Section of Pulmonary and Critical Care Medicine, New Haven, Connecticut.


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.


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