To discuss the clinical subtypes, pathogenesis, pathology, diagnostic evaluation, treatment options, and prognosis of pulmonary hypertension in systemic sclerosis (SSc-PH) and highlight its fundamental differences from idiopathic pulmonary arterial hypertension (IPAH).
A Medline search for articles published between January 1969 and June 2010 was conducted using the following keywords: scleroderma, systemic sclerosis, pulmonary hypertension, pulmonary arterial hypertension, pulmonary veno-occlusive disease, pathogenesis, pathology, investigation, treatment, and prognosis. The essential differences from IPAH in pathogenesis and histopathologic findings were highlighted and the limitations of some of the investigations used were emphasized. The differences in response to currently accepted therapy and prognosis were also reviewed.
In scleroderma, pulmonary hypertension can be present in isolation or along with interstitial lung disease and left heart disease. In SSc-PH, the unique histopathologic findings in the lungs include intimal fibrosis, absence of plexiform lesions, and a high prevalence of pulmonary veno-occlusive disease-like lesions. Both "6-minute walk test" and NT-proBNP have their limitations in the evaluation of SSc-PH. For treatment, calcium channel blockers are ineffective and anticoagulation should be used with caution. Currently approved therapies are not as effective and prognosis is much worse in SSc-PH compared with IPAH.
SSc-PH is a complex condition with poorer response to therapy and worse outcome compared with that of IPAH. Recent findings have shed some light about the pathophysiology and pathogenesis of SSc-PH. Further research in this area is warranted to better understand the complex pathogenesis and devise better therapeutic strategies.