Scleroderma-Related PAH: The Need for Early Diagnosis and Treatment

Author: Kalanathee Paul-Pletzer, PhD
Date Published: May-2011
Source: Medscape Education Cardiology

Pulmonary arterial hypertension (PAH) associated with scleroderma (systemic sclerosis) is an aggressive disorder with a poor prognosis. Effective therapies are available for PAH but patients with scleroderma-associated PAH have a poorer response to these therapies compared with those with idiopathic PAH (IPAH). Nonetheless, improvement in outcomes has been obtained, but early diagnosis and early institution of PAH-specific therapy are of paramount importance.


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