Abstract
Background The major reasons to treat sarcoidosis are to lower the morbidity and mortality risk or to improve quality of life (QoL). The indication for treatment varies depending on which manifestation is the cause of symptoms: lungs, heart, brain, skin, or other manifestations. While glucocorticoids (GC) remain the first choice for initial treatment of symptomatic disease, prolonged use is associated with significant toxicity. GC-sparing alternatives are available. The presented treatment guideline aims to provide guidance to physicians treating the very heterogenous sarcoidosis manifestations.
Materials and Methods A European Respiratory Society Task Force (TF) committee composed of clinicians, methodologists, and patients with experience in sarcoidosis developed recommendations based on the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology. The committee developed eight PICO (Patients, Intervention, Comparison, Outcomes) questions and these were used to make specific evidence-based recommendations.
Results The TF committee delivered twelve recommendations for seven PICOs. These included treatment of pulmonary, cutaneous, cardiac, and neurologic disease as well as fatigue. One PICO question regarding small fiber neuropathy had insufficient evidence to support a recommendation. In addition to the recommendations, the committee provided information on how they use alternative treatments, when there was insufficient evidence to support a recommendation.
Conclusions There are many treatments available to treat sarcoidosis. Given the diverse nature of the disease, treatment decisions require an assessment of organ involvement, risk for significant morbidity, and impact on QoL of the disease and treatment.
Message An evidence based guideline for treatment of sarcoidosis is presented. The panel used the GRADE approach and specific recommendations are made. A major factor in treating patients is the risk of loss of organ function or impairment of quality of life.
Footnotes
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Conflict of interest: Dr. Baughman reports grants from Gilead, grants from Genentech, grants from Bayer, grants and personal fees from Novartis, personal fees from Methial, grants from aTYR, grants and personal fees from Mallinckrodt, grants and personal fees from Boehringer Ingleheim, grants from Bellephron, outside the submitted work; .
Conflict of interest: Dr. Valeyre reports personal fees from Boeringer Ingelheim, personal fees from Roche, outside the submitted work; .
Conflict of interest: Dr. Korsten reports personal fees from Glaxo Smith Kline , personal fees from Abbvie , personal fees from Pfizer , personal fees from Chugai , personal fees from Novartis Pharma , personal fees from Sanofi-Aventis , personal fees from Lilly, grants from Glaxo Smith Kline, personal fees from Gilead , outside the submitted work; .
Conflict of interest: Dr. Mathioudakis reports grants from Boehringer Ingelheim, outside the submitted work; .
Conflict of interest: Dr. Wuyts has nothing to disclose.
Conflict of interest: Dr. Wells reports personal fees from Boehringer Ingelheim, personal fees from Roche, during the conduct of the study; .
Conflict of interest: Dr. Rottoli has nothing to disclose.
Conflict of interest: Dr. Hilario Nunes has nothing to disclose.
Conflict of interest: Dr. Lower reports grants from Gilead, grants from Genentech, grants from Bayer, grants and personal fees from Novartis, grants from aTYR, outside the submitted work; .
Conflict of interest: Dr. Judson received grant support for his institution from Mallickrodt
Conflict of interest: Dr. Israël-Biet has nothing to disclose.
Conflict of interest: Dr. Grutters has nothing to disclose.
Conflict of interest: Dr. Drent has nothing to disclose.
Conflict of interest: Dr. Culver reports grants and personal fees from aTyr , grants and personal fees from Mallinkrodt, grants and personal fees from Boehringer-Ingelheim, personal fees from Roche, grants and personal fees from Foundation for Sarcoidosis Research, outside the submitted work; .
Conflict of interest: Dr. Bonella reports personal fees and non-financial support from Boehringer Ingelheim, Roche, Galapagos, BMS, Savara Pharma, outside the submitted work.
Conflict of interest: Dr. Antonio has nothing to disclose.
Conflict of interest: Dr. Martone has nothing to disclose.
Conflict of interest: Dr. rer. nat. Bernd Quadder PhD has nothing to disclose.
Conflict of interest: Dr. Spitzer has nothing to disclose.
Conflict of interest: Dr. Nagavci acts as ERS methodologist.
Conflict of interest: Ms Tonia reports acting as ERS Methodologist .
Conflict of interest: Dr. Rigau reports and he was one of theERS methodologists .
Conflict of interest: Dr. Oullette reports grants from PCORI (Patient-centered outcomes research institute, US Federal), grants from Sanofi, outside the submitted work; and Expert witness work for venous thromboembolic disease.
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- Received November 4, 2020.
- Accepted May 4, 2021.
- Copyright ©The authors 2021. For reproduction rights and permissions contact permissions{at}ersnet.org