Robert P Baughman1, Dominique Valeyre2, Peter Korsten3, Alexander G Mathioudakis4, Wim A Wuyts5, Athol Wells6, Paola Rottoli7, Hiliaro Nunes8, Elyse E Lower9, Marc A Judson10, Dominique Israel-Biet11, Jan C Grutters12,13, Marjolein Drent12,14,15, Daniel A Culver16, Francesco Bonella17, Katerina Antoniou18, Filippo Martone19, Bernd Quadder20, Ginger Spitzer21, Blin Nagavci22, Thomy Tonia23, David Rigau24, Daniel R Ouellette25. 1. Dept of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA bob.baughman@uc.edu. 2. INSERM UMR 1272, Université Sorbonne Paris Nord, AP-HP, Hôpital Avicenne, Bobigny, Groupe Hospitalier Paris-Saint Joseph, Paris, France. 3. Dept of Nephrology and Rheumatology, University Medical Center Göttingen, Göttingen, Germany. 4. North West Lung Centre, Wythenshawe Hospital, Manchester University NHS Foundation Trust, and Division of Infection, Immunity and Respiratory Medicine, The University of Manchester, Manchester, UK. 5. Unit for Interstitial Lung Diseases, Dept of Respiratory Medicine, University Hospitals Leuven, Leuven, Belgium. 6. Royal Brompton Hospital, London, UK. 7. Specialization School of Respiratory Diseases, Dept of Medical, Surgical and Neurological Sciences, Siena University, Siena, Italy. 8. INSERM UMR 1272, Université Sorbonne Paris Nord, Service de Pneumologie, Centre de Référence des Maladies Pulmonaires Rares, AP-HP, Hôpital Avicenne, Bobigny, France. 9. Dept of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA. 10. Dept of Medicine, Albany Medical College, Albany, NY, USA. 11. Université de Paris, Centre de Compétences Maladies Rares Pulmonaires, AP-HP, Hôpital Européen Georges Pompidou, Paris, France. 12. ILD Center of Excellence, Dept of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands. 13. Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, The Netherlands. 14. Dept of Pharmacology and Toxicology, Faculty of Health and Life Sciences, Maastricht University, Maastricht, The Netherlands. 15. ILD Care Foundation Research Team, Ede, The Netherlands. 16. Cleveland Clinic, Cleveland, OH, USA. 17. Center for Interstitial and Rare Lung Diseases, Pneumology Dept, Ruhrlandklinik, University Hospital, University of Essen, Essen, Germany. 18. Dept of Respiratory Medicine, Laboratory of Molecular and Cellular Pneumonology, Medical School, University of Crete, Heraklion, Greece. 19. Amici Contro la Sarcoidosi Italia ONLUS, Bologna, Italy. 20. Deutsche Sarkoidose-Vereinigung eV (DSV), Meerbusch, Germany. 21. Foundation for Sarcoidosis Research, Chicago, IL, USA. 22. Institute for Evidence in Medicine, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. 23. Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland. 24. Cochrane Iberoamerica, Barcelona, Spain. 25. Henry Ford Hospital, Detroit, MI, USA.
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.
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.