Pulmonary hypertension Historical Perspective: Difference between revisions
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==Historical Perspective== | ==Historical Perspective== | ||
The incidence of PAH associated with anorexigens is cyclical in nature and varies depending on the availability of specific appetite suppressants. The link was first identified in the 1960s when an epidemic of PAH occurred in Switzerland, Austria and Germany that was linked to the anorexigen aminorex fumarate. Use of the anorexigens fenfluramine and dexfenfluramine have also been linked with an increased risk for PAH. | The incidence of PAH associated with anorexigens is cyclical in nature and varies depending on the availability of specific appetite suppressants. The link was first identified in the 1960s when an epidemic of PAH occurred in Switzerland, Austria and Germany that was linked to the anorexigen aminorex fumarate. Use of the anorexigens fenfluramine and dexfenfluramine have also been linked with an increased risk for PAH. | ||
Prior to the development of disease-specific targeted PAH therapies, the median survival for subjects diagnosed with IPAH was approximately 2.8 years. However, 2.8 years likely underestimates current survival as the course of the disease has been favorably altered by therapeutic advances since that report from the 1980s. Prognosis is also dependent on the underlying etiology of the disease. The prognosis for patients with PAH associated with connective tissue disease appears to be worse than for those with IPAH. Estimates for 2-year survival in scleroderma patients with associated PAH are 40% compared with 48% for 3-year survival in patients with IPAH. Survival in patients with HIV-associated PAH is similar to patients with IPAH. With current HIV therapies, most of the deaths in patients with HIV and associated PAH are now attributed to PAH. | |||
==References== | ==References== | ||
Revision as of 18:35, 1 March 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Historical Perspective
The incidence of PAH associated with anorexigens is cyclical in nature and varies depending on the availability of specific appetite suppressants. The link was first identified in the 1960s when an epidemic of PAH occurred in Switzerland, Austria and Germany that was linked to the anorexigen aminorex fumarate. Use of the anorexigens fenfluramine and dexfenfluramine have also been linked with an increased risk for PAH.
Prior to the development of disease-specific targeted PAH therapies, the median survival for subjects diagnosed with IPAH was approximately 2.8 years. However, 2.8 years likely underestimates current survival as the course of the disease has been favorably altered by therapeutic advances since that report from the 1980s. Prognosis is also dependent on the underlying etiology of the disease. The prognosis for patients with PAH associated with connective tissue disease appears to be worse than for those with IPAH. Estimates for 2-year survival in scleroderma patients with associated PAH are 40% compared with 48% for 3-year survival in patients with IPAH. Survival in patients with HIV-associated PAH is similar to patients with IPAH. With current HIV therapies, most of the deaths in patients with HIV and associated PAH are now attributed to PAH.