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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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