Chapter 8 Drug-and Toxin-Induced Pulmonary Arterial Hypertension (original) (raw)

Pulmonary arterial hypertension (PAH) is a progressive disease lead-ing to remodeling of small pulmonary arteries. The exact pathogen-esis of PAH is uncertain; however, several risk factors are likely to contribute. These include inflammatory 'hits', such as a viral infec-tion, and inherited disorders in vascular cell growth, such as a mu-tation in the bone morphogenetic protein receptor type 2 gene. It has also become apparent, first through early associations and later with larger epidemiological studies, that the use of certain drugs and toxins are important in triggering PAH, especially in suscep-tible individuals. One of the earliest observations of drug-related PAH followed use of the stimulant appetite suppressant aminorex fumarate. Similar associations have also been reported, especially in other stimulant anorectics such as the serotonin reuptake inhibi-tor dexfenfluramine, as well as a range of other agents, including illegal substances. Agents are described to cau...