WASOG statement on the diagnosis and management of sarcoidosis-associated pulmonary hypertension
Laurent Savale, Marloes P. Huitema, Oksana A. Shlobin, Vasileios Kouranos, Steven D. Nathan, Hiliaro Nunes, Rohit Gupta, Jan C. Grutters, Daniel A. Culver, Marco C. Post, Daniel R. Ouellette, Elyse E. Lower, Tamara Al-Hakim, Athol U. Wells, Marc Humbert, Robert P. Baughman
Abstract
Sarcoidosis-associated pulmonary hypertension (SAPH) is an important complication of advanced sarcoidosis. Over the past few years, there have been several studies dealing with screening, diagnosis and treatment of SAPH. This includes the results of two large SAPH-specific registries. A task force was established by the World Association of Sarcoidosis and Other Granulomatous disease (WASOG) to summarise the current level of knowledge in the area and provide guidance for the management of patients. A group of sarcoidosis and pulmonary hypertension experts participated in this task force. The committee developed a consensus regarding initial screening including who should undergo more specific testing with echocardiogram. Based on the results, the committee agreed upon who should undergo right-heart catheterisation and how to interpret the results. The committee felt there was no specific phenotype of a SAPH patient in whom pulmonary hypertension-specific therapy could be definitively recommended. They recommended that treatment decisions be made jointly with a sarcoidosis and pulmonary hypertension expert. The committee recognised that there were significant defects in the current knowledge regarding SAPH, but felt the statement would be useful in directing future studies.