New insights into the pathogenesis and treatment of primary pulmonary hypertension

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Abstract
The precise molecular mechanisms underlying PPH have hitherto remained elusive. Clues to the aetiology of the disease have been suggested by environmental stimuli associated with the development of severe pulmonary hypertension, pathologically indistinguishable from PPH. The association with appetite suppressants, in particular the fenfluramine/dexfenfluramine group,3 suggested a role of re-uptake inhibition of serotonin.4 Severe PPH may also follow HIV-1 infection, and PPH is associated with autoimmune thyroid disease and the presence of circulating anti-Ku and antinuclear antibodies.1