Diagnosis of Pulmonary Arterial Hypertension in a Patient With Systemic Sclerosis

Jörg HW Distler, MD; Marius M Hoeper, MD; Oliver Distler, MD


Nat Clin Pract Rheumatol. 2008;4(3):160-164. 

In This Article


PAH contributes significantly to the morbidity and mortality of patients with SSc. PAH can occur in all disease stages and subsets of SSc, but patients with limited cutaneous SSc or anticentromere antibodies, those of older age, and those with decreasing DLCO (together with normal lung volumes), are at particular risk. Regular screening tests for PAH are warranted in patients with SSc, since clinical symptoms, including dyspnea on exertion, are unspecific and occur late in the course of PAH, and effective therapies are available. The most widely used screening tool is the non-invasive TTE; however, TTE can give false-negative and false-positive results, particularly in patients with borderline RVSP values on echocardiography. Lung function tests including DLCO should, therefore, be used in addition to TTE. In patients with suspected PAH, the diagnosis should be confirmed by right heart catheterization.

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