ERS clinical practice guidelines on treatment of sarcoidosis
Robert P. Baughman, Dominique Valeyre, Peter Korsten, Alexander G. Mathioudakis, Wim Wuyts, Athol U. Wells, Paola Rottoli, Hiliaro Nunes, Elyse E. Lower, Marc A. Judson, Dominique Israël‐Biet, Jan C. Grutters, Marjolein Drent, Daniel A. Culver, Francesco Bonella, Κατερίνα Αντωνίου, F Martone, Bernd Quadder, Ginger Spitzer, Blin Nagavci, Thomy Tonia, David Rigau, Daniel R. Ouellette
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 remain the first choice for initial treatment of symptomatic disease, prolonged use is associated with significant toxicity. Glucocorticoid-sparing alternatives are available. The presented treatment guidelines aim to provide guidance to physicians treating the very heterogenous sarcoidosis manifestations. METHODS: A European Respiratory Society Task Force 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 Task Force committee delivered 12 recommendations for seven PICOs. These included treatment of pulmonary, cutaneous, cardiac and neurologic disease as well as fatigue. One PICO question regarding small-fibre 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.