Practice Guideline

. 2021 Dec 16;58(6):2004079.

doi: 10.1183/13993003.04079-2020. Print 2021 Dec.

ERS clinical practice guidelines on treatment of sarcoidosis

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 Grutters^{12

13}, Marjolein Drent^{12

14

15}, Daniel A Culver¹⁶, Francesco Bonella¹⁷, Katerina Antoniou¹⁸, Filippo Martone¹⁹, Bernd Quadder²⁰, Ginger Spitzer²¹, Blin Nagavci²², Thomy Tonia²³, David Rigau²⁴, Daniel R Ouellette²⁵

Affiliations

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.

PMID: 34140301
DOI: 10.1183/13993003.04079-2020

Free article

Practice Guideline

ERS clinical practice guidelines on treatment of sarcoidosis

Robert P Baughman et al. Eur Respir J. 2021.

Free article

. 2021 Dec 16;58(6):2004079.

doi: 10.1183/13993003.04079-2020. Print 2021 Dec.

Authors

Affiliations

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.

PMID: 34140301
DOI: 10.1183/13993003.04079-2020

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.

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Conflict of interest statement

Conflict of interest: R.P. Baughman reports grants from Gilead, Genentech, Bayer, aTyr and Bellephron, grants and personal fees for consultancy from Novartis, personal fees for consultancy from Methial, grants and personal fees for consultancy and lectures from Mallinckrodt, grants and personal fees for lectures from Boehringer Ingelheim, outside the submitted work. Conflict of interest: D. Valeyre reports personal fees for advisory board work from Boehringer Ingelheim and Roche, outside the submitted work. Conflict of interest: P. Korsten reports grants and personal fees from GlaxoSmithKline, personal fees from AbbVie, Pfizer, Chugai, Novartis Pharma, Sanofi-Aventis, Lilly and Gilead, outside the submitted work. Conflict of interest: A.G. Mathioudakis reports grants from Boehringer Ingelheim, outside the submitted work. Conflict of interest: W.A. Wuyts has nothing to disclose. Conflict of interest: A. Wells reports personal fees for lectures and advisory board work from Boehringer Ingelheim and Roche, during the conduct of the study. Conflict of interest: P. Rottoli has nothing to disclose. Conflict of interest: H. Nunes has nothing to disclose. Conflict of interest: E.E. Lower reports grants from Gilead, Genentech, Bayer and aTYR, grants and personal fees for consultancy from Novartis, outside the submitted work. Conflict of interest: M.A. Judson received grant support for his institution from Mallickrodt. Conflict of interest: D. Israël-Biet has nothing to disclose. Conflict of interest: J.C. Grutters has nothing to disclose. Conflict of interest: M. Drent has nothing to disclose. Conflict of interest: D.A. Culver reports grants and personal fees from aTyr, Mallinckrodt, Boehringer Ingelheim and Foundation for Sarcoidosis Research, personal fees from Roche, outside the submitted work. Conflict of interest: F. Bonella reports personal fees and non-financial support from Boehringer Ingelheim, Roche, Galapagos, BMS and Savara Pharma, outside the submitted work. Conflict of interest: K. Antoniou has nothing to disclose. Conflict of interest: F. Martone has nothing to disclose. Conflict of interest: B. Quadder has nothing to disclose. Conflict of interest: G. Spitzer has nothing to disclose. Conflict of interest: B. Nagavci acts as an ERS methodologist. Conflict of interest: T. Tonia acts as an ERS methodologist. Conflict of interest: D. Rigau acts as an ERS methodologist. Conflict of interest: D.R. Oullette reports grants from PCORI (Patient-Centred Outcomes Research Institute, US Federal) and Sanofi, outside the submitted work; and has provided expert witness work for venous thromboembolic disease.

Comment in

Comparison of the treatment guidelines for sarcoidosis: common sense in the search for evidence.
Kahlmann V, Moor CC, Miedema JR, Wijsenbeek MS. Kahlmann V, et al. Eur Respir J. 2022 Feb 10;59(2):2103114. doi: 10.1183/13993003.03114-2021. Print 2022 Feb. Eur Respir J. 2022. PMID: 34949705 No abstract available.

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ERS clinical practice guidelines on treatment of sarcoidosis

Affiliations

ERS clinical practice guidelines on treatment of sarcoidosis

Authors

Affiliations

Abstract

Conflict of interest statement

Comment in

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Medical