ERS clinical practice guidelines on treatment of sarcoidosis

Robert P Baughman; Dominique Valeyre; Peter Korsten; Alexander G Mathioudakis; Wim A Wuyts; Athol Wells; Paola Rottoli; Hiliaro Nunes; Elyse E Lower; Marc A Judson; Dominique Israel-Biet; Jan C Grutters; Marjolein Drent; Daniel A Culver; Francesco Bonella; Katerina Antoniou; Filippo Martone; Bernd Quadder; Ginger Spitzer; Blin Nagavci; Thomy Tonia; David Rigau; Daniel R Ouellette

doi:10.1183/13993003.04079-2020

ERS clinical practice guidelines on treatment of sarcoidosis

Robert P Baughman^*, Dominique Valeyre, Peter Korsten, Alexander G Mathioudakis, Wim A Wuyts, Athol Wells, Paola Rottoli, Hiliaro Nunes, Elyse E Lower, Marc A Judson, Dominique Israel-Biet, Jan C Grutters, Marjolein Drent, Daniel A Culver, Francesco Bonella, Katerina Antoniou, Filippo Martone, Bernd Quadder, Ginger Spitzer, Blin NagavciThomy Tonia, David Rigau, Daniel R Ouellette

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

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.

Original language	English
Article number	2004079
Number of pages	31
Journal	European Respiratory Journal
Volume	58
Issue number	6
DOIs	https://doi.org/10.1183/13993003.04079-2020
Publication status	Published - Dec 2021

Keywords

Fatigue
Humans
Quality of Life
Sarcoidosis/diagnosis
CHRONIC CUTANEOUS SARCOIDOSIS
LONG-TERM OUTCOMES
SMALL FIBER NEUROPATHY
LATE GADOLINIUM ENHANCEMENT
F-18 FDG-PET/CT
DOSE METHOTREXATE THERAPY
BRITISH-THORACIC-SOCIETY
LEFT-VENTRICULAR DYSFUNCTION
QUALITY-OF-LIFE
PLACEBO-CONTROLLED TRIAL

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10.1183/13993003.04079-2020

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Baughman, R. P., Valeyre, D., Korsten, P., Mathioudakis, A. G., Wuyts, W. A., Wells, A., Rottoli, P., Nunes, H., Lower, E. E., Judson, M. A., Israel-Biet, D., Grutters, J. C., Drent, M., Culver, D. A., Bonella, F., Antoniou, K., Martone, F., Quadder, B., Spitzer, G., ... Ouellette, D. R. (2021). ERS clinical practice guidelines on treatment of sarcoidosis. European Respiratory Journal, 58(6), Article 2004079. https://doi.org/10.1183/13993003.04079-2020

@article{52b6f7e834844cf09b05febfb7059475,

title = "ERS clinical practice guidelines on treatment of sarcoidosis",

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.",

keywords = "Fatigue, Humans, Quality of Life, Sarcoidosis/diagnosis, CHRONIC CUTANEOUS SARCOIDOSIS, LONG-TERM OUTCOMES, SMALL FIBER NEUROPATHY, LATE GADOLINIUM ENHANCEMENT, F-18 FDG-PET/CT, DOSE METHOTREXATE THERAPY, BRITISH-THORACIC-SOCIETY, LEFT-VENTRICULAR DYSFUNCTION, QUALITY-OF-LIFE, PLACEBO-CONTROLLED TRIAL",

author = "Baughman, {Robert P} and Dominique Valeyre and Peter Korsten and Mathioudakis, {Alexander G} and Wuyts, {Wim A} and Athol Wells and Paola Rottoli and Hiliaro Nunes and Lower, {Elyse E} and Judson, {Marc A} and Dominique Israel-Biet and Grutters, {Jan C} and Marjolein Drent and Culver, {Daniel A} and Francesco Bonella and Katerina Antoniou and Filippo Martone and Bernd Quadder and Ginger Spitzer and Blin Nagavci and Thomy Tonia and David Rigau and Ouellette, {Daniel R}",

note = "Copyright {\textcopyright}The authors 2021. For reproduction rights and permissions contact [email protected].",

year = "2021",

month = dec,

doi = "10.1183/13993003.04079-2020",

language = "English",

volume = "58",

journal = "European Respiratory Journal",

issn = "0903-1936",

publisher = "European Respiratory Society",

number = "6",

}

Baughman, RP, Valeyre, D, Korsten, P, Mathioudakis, AG, Wuyts, WA, Wells, A, Rottoli, P, Nunes, H, Lower, EE, Judson, MA, Israel-Biet, D, Grutters, JC, Drent, M, Culver, DA, Bonella, F, Antoniou, K, Martone, F, Quadder, B, Spitzer, G, Nagavci, B, Tonia, T, Rigau, D & Ouellette, DR 2021, 'ERS clinical practice guidelines on treatment of sarcoidosis', European Respiratory Journal, vol. 58, no. 6, 2004079. https://doi.org/10.1183/13993003.04079-2020

TY - JOUR

T1 - ERS clinical practice guidelines on treatment of sarcoidosis

AU - Baughman, Robert P

AU - Valeyre, Dominique

AU - Korsten, Peter

AU - Mathioudakis, Alexander G

AU - Wuyts, Wim A

AU - Wells, Athol

AU - Rottoli, Paola

AU - Nunes, Hiliaro

AU - Lower, Elyse E

AU - Judson, Marc A

AU - Israel-Biet, Dominique

AU - Grutters, Jan C

AU - Drent, Marjolein

AU - Culver, Daniel A

AU - Bonella, Francesco

AU - Antoniou, Katerina

AU - Martone, Filippo

AU - Quadder, Bernd

AU - Spitzer, Ginger

AU - Nagavci, Blin

AU - Tonia, Thomy

AU - Rigau, David

AU - Ouellette, Daniel R

PY - 2021/12

Y1 - 2021/12

N2 - 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.

AB - 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.

KW - Fatigue

KW - Humans

KW - Quality of Life

KW - Sarcoidosis/diagnosis

KW - CHRONIC CUTANEOUS SARCOIDOSIS

KW - LONG-TERM OUTCOMES

KW - SMALL FIBER NEUROPATHY

KW - LATE GADOLINIUM ENHANCEMENT

KW - F-18 FDG-PET/CT

KW - DOSE METHOTREXATE THERAPY

KW - BRITISH-THORACIC-SOCIETY

KW - LEFT-VENTRICULAR DYSFUNCTION

KW - QUALITY-OF-LIFE

KW - PLACEBO-CONTROLLED TRIAL

U2 - 10.1183/13993003.04079-2020

DO - 10.1183/13993003.04079-2020

M3 - Article

C2 - 34140301

SN - 0903-1936

VL - 58

JO - European Respiratory Journal

JF - European Respiratory Journal

IS - 6

M1 - 2004079

ER -

ERS clinical practice guidelines on treatment of sarcoidosis

Abstract

Keywords

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