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Which patients are SaPHe in sarcoidosis-associated pulmonary hypertension?

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Pulmonary hypertension (PH) is a well described and clinically important complication in sarcoidosis. The epidemiology of sarcoidosis-associated pulmonary hypertension (SaPH) varies depending on the characteristics of the population analysed, but… Click to show full abstract

Pulmonary hypertension (PH) is a well described and clinically important complication in sarcoidosis. The epidemiology of sarcoidosis-associated pulmonary hypertension (SaPH) varies depending on the characteristics of the population analysed, but ranges from 3–75%, depending on method of diagnosis (i.e. echocardiography versus right heart catheterisation (RHC)) and severity of underlying sarcoidosis [1–4]. Elevation of pulmonary arterial pressure in sarcoidosis can occur via a number of mechanisms (figure 1) [5, 6] and, as such, SaPH has remained under group 5 (PH with unclear and/or multifactorial mechanisms) in the recent 6th World Symposium on Pulmonary Hypertension [7]. The breadth of pathophysiologic mechanisms that lead to PH is fairly unique to sarcoidosis, which makes SaPH an interesting but complicated entity. Pre-capillary pulmonary hypertension is a rare and heterogenous complication in sarcoidosis. 6-min walking distance is a robust and consistent prognostic factor, but the role of pulmonary arterial hypertension-targeted treatments remains controversial. http://bit.ly/2J27Yps

Keywords: pulmonary hypertension; sarcoidosis associated; patients saphe; sarcoidosis; associated pulmonary; hypertension

Journal Title: European Respiratory Journal
Year Published: 2020

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