Extract
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.
Abstract
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
Footnotes
Conflict of interest: D. Vis has nothing to disclose.
Conflict of interest: A. Boucly reports personal fees and non-financial support from Actelion, personal fees from MSD, outside the submitted work.
Conflict of interest: M. Humbert reports personal fees from Acceleron, Merck, Morphogen IX and United Therapeutics, grants and personal fees from Actelion, Bayer and GSK, outside the submitted work.
Conflict of interest: J. Weatherald reports grants, personal fees and non-financial support from Janssen Inc. and Actelion, personal fees and non-financial support from Bayer, personal fees from Novartis, grants from Alberta Lung Association, Canadian Vascular Network, European Respiratory Society and Canadian Thoracic Society, outside the submitted work.
- Received March 15, 2020.
- Accepted March 16, 2020.
- Copyright ©ERS 2020