Pulmonary hypertension (high blood pressure in the lungs) is the leading cause of death and disability in patients with scleroderma. This is, however, the area in which advances in diagnosis and treatment are making rapid progress.

The only drugs approved by the Food & Drug Administration for use in scleroderma are agents recently approved for pulmonary hypertension. These include Flolan® (an intravenous prostacyclin), Treprostinil® (a prostacyclin that is given either subcutaneously or intravenously), Ventavis® (an inhaled prostacyclin), Tracleer ® (a pill taken by mouth) and Revatio® (a pill containing the active ingredient in Viagra).

Other similar drugs are at late stages of preapproval clinical trials and there is great interest in studying the benefits of various combination therapies.

The University of Michigan is home to a world class Pulmonary Hypertension Program. Based in the Division of Cardiology, the Program is directed by Vallerie V. McLaughlin, M.D. – a recognized thought leader in pulmonary hypertension and features the participation of many other expert faculty, including Melvyn P. Rubenfire, M.D. The Program is supported by a staff of nurse experts who are skilled and experienced in managing these complex treatments.

Patients in the Scleroderma Program are routinely assessed for the possible presence of pulmonary hypertension and referred to the Pulmonary Hypertension Program for consultation and continuing care. The Pulmonary Hypertension Program accepts direct referrals as well.

Pulmonary hypertension is considered if there is shortness of breath or fatigue during physical activity. Careful physical examination can provide clues as can non-invasive laboratory testing, including pulmonary function testing, Doppler echocardiograms and simple exercise tests. Correct medical diagnosis and care usually requires a right heart catheterization.

The University of Michigan Pulmonary Hypertension Program has an active interest in developing new treatments. Many of their trials permit participation by patients with scleroderma. For more information please see Research – Clinical Trials.

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