FSR Physicians’ Treatment Protocol
FSR is committed to addressing the gaps that exist in sarcoidosis research. This includes the lack of information and knowledge around the current disease treatment options. Yet another challenge of “rare disease” is the inherent rarity of specialists and experts that can help guide treatment options. While physicians who specialize in sarcoidosis can be found, many times patients and doctors are challenged by a lack of information. FSR’s Scientific Advisory Board developed a Physicians’ Treatment Protocol that can offer expert information to physicians to help guide their diagnosis and treatment decisions.
Click Here to view the list of commonly prescribed drugs for the treatment of sarcoidosis.
This information can be viewed by physicians, and by patients to share with their doctors.
Sarcoidosis Associated Pulmonary Hypertension (SAPH) Guidelines Development
FSR partnered with WASOG to bring together a panel of pulmonary experts in late 2019 to create a dialogue and education physicians on Sarcoidosis Associated Pulmonary Hypertension (SAPH). This meeting was sponsored and made possible through funding support by Bayer.
SAPH is a recognized complication of advanced pulmonary sarcoidosis. It has been reported to occur in 5-15% of patients attending tertiary sarcoidosis clinics (1-3). In some studies, up to half of patients with persistent dyspnea (shortness of breath) despite immunosuppression for lung disease have SAPH (4;5). Recently, two groups have reported on the clinical features and outcome of patients with SAPH. Both registries required right heart catheterization to confirm the diagnosis and focused on those with precapillary pulmonary hypertension. The French group reported on 170 patients with moderate to severe SAPH (6).
We believe that there is an increasing need to develop guidelines for diagnosis and management of SAPH to aid in clinical trials as well as day to day management of these patients.
In this bonus episode on John Carlin’s Sarc Fighter Podcast, Dr. Robert Baughman moderates the panel as they discuss various aspects of SAPH screening, diagnosis, and management.
- Robert Baughman, Internist at University of Cincinnati with specialties in Pulmonary Diseases, Lung Disease, Interstitial Lung Disease
- Professor Athol Wells, Consultant and chest physician based at Royal Brompton Hospital
- Professor Marc Humbert, Head of the Pulmonology and Intensive Respiratory Care Department at University of Paris
- Steven D. Nathan, Director of the Advanced Lung Disease Program and director of the Lung Transplant Program at Inova Fairfax Hospital
(1) Handa T, Nagai S, Miki S et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest 2006; 129(5):1246-1252.
(2) Keir GJ, Walsh SL, Gatzoulis MA et al. Treatment of sarcoidosis-associated pulmonary hypertension: A single centre retrospective experience using targeted therapies. Sarcoidosis Vasc Diffuse Lung Dis 2014; 31(2):82-90.
(3) Bourbonnais JM, Samavati L. Clinical predictors of pulmonary hypertension in sarcoidosis. Eur Respir J 2008; 32(2):296-302.
(4) Sulica R, Teirstein AS, Kakarla S et al. Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension. Chest 2005; 128(3):1483-1489.
(5) Baughman RP, Engel PJ, Taylor L et al. Survival in sarcoidosis associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest 2010; 138:1078-1085.
(6) Boucly A, Cottin V, Nunes H et al. Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension. Eur Respir J 2017; %19;50(4):50-4-2017.
(7) Baughman RP, Shlobin OA, Wells AU et al. Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry. Respir Med 2018; 139:72-78. doi: 10.1016/j.rmed.2018.04.015. Epub;%2018 May 5.:72-78.
(8) Shlobin OA, Baughman RP. Sarcoidosis-Associated Pulmonary Hypertension. Semin Respir Crit Care Med 2017; 38(4):450-462.
(9) Baughman RP, Culver DA, Cordova FC et al. Bosentan For Sarcoidosis Associated Pulmonary Arterial Hypertension (BoSAPAH): A Double Blind, Placebo Controlled Trial. Am J Resp Crit Care Med 2012; 185:A3639.