WASOG statement on the diagnosis and management of sarcoidosis-associated pulmonary hypertension.

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.
Competing Interests: Conflict of interest: L. Savale reports support for the present manuscript from Janssen and Janssen, and MSD. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events received from Janssen and Janssen, and MSD, outside the submitted work. Support for attending meetings and/or travel received from Janssen and Janssen, and MSD, outside the submitted work. Conflict of interest: O. Shlobin reports participation on a Data Safety Monitoring Board or Advisory Board for Bayer, United Therapeutics, Johnson and Johnson, and Altavant, outside the submitted work. Conflict of interest: V. Kouranos reports receiving payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Novartis, and Roche, outside the submitted work. Conflict of interest: S.D. Nathan reports receiving consulting fees from United Therapeutics, Bellerophon, Merck, Bayer, Roche, and Boehringer Ingelheim, outside the submitted work. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from United Therapeutics, Bayer, Roche, and Boehringer Ingelheim, outside the submitted work. Payment for expert testimony received from Roche. Conflict of interest: H. Nunes reports receiving consulting fees from Actelion (now Janssen), outside the submitted work. Conflict of interest: R. Gupta reports receiving grants of contracts from Bayer, outside the submitted work. Conflict of interest: J.C. Grutters reports receiving grants or contracts from SPHINX trial (Actelion) outside the submitted work. J.C. Grutters also reports to be member of the SPHINX trial steering committee. Conflict of interest: D.A. Culver reports participation on a Data Safety Monitoring Board or Advisory Board for Actelion (Janssen), and United Therapeutics, outside the submitted work. Conflict of interest: D. Ouellette reports receiving grants or contracts from US Federal Government/PICORI, and Sanofi, outside the submitted work. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Sunrise 2019, India, outside the submitted work. Payment for expert testimony received from Adam & McGrevey Law Firm, and Spangenberg, Shibley & Liber, outside the submitted work. Unpaid Incoming chair Critical Care Network; CHEST. Conflict of interest: E.E. Lower reports receiving grants or contracts from Bayer, Bellephron, Actelion, Genentech, Mallinckrodt, aTyr, Novartis, and Gilead, outside the submitted work. Conflict of interest: T. Al-Hakim reports support for the present manuscript from Foundation for Sarcoidosis Research, and Bayer Pharmaceuticals. Conflict of interest: A.U. Wells reports participation on a Data Safety Monitoring Board or Advisory Board for Roche, outside the submitted work. A.U. Wells also reports to be President Elect of the World Association of Sarcoidosis and Other Granulomatous Diseases. Conflict of interest: M. Humbert reports receiving grants or contracts from Acceleron, Janssen, and Merck, outside the submitted work. Consulting fees received from Acceleron, Janssen, and Merck, outside the submitted work. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events received from AOP, Janssen, and Merck, outside the submitted work. Participation on a Data Safety Monitoring Board or Advisory Board for Acceleron, Janssen, and Merck. Conflict of interest: R.P. Baughman reports support for the present manuscript from Foundation for Sarcoidosis Research. Grants or contracts received from Bayer, Bellephron, Actelion, Genentech, Mallinckrodt, aTyr, Novartis, and Gilead, outside the submitted work. Consulting fees received from Mallinckrodt. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events received from Mallinckrodt, Boehringer Ingelheim, and United Therapeutics. Participation on a Data Safety Monitoring Board or Advisory Board for Bellephron, United Therapeutics, Mallinckrodt, and Actelion. Conflict of interest: M. Huitema and M.C. Post have nothing to disclose.
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