ERS clinical practice guidelines on treatment of sarcoidosis

Key points

• Sarcoidosis is treated to lower the risk of death or organ failure or to improve quality of life; about 5% of patients die from the disease, most often from pulmonary or cardiac involvement. The ERS Task Force made 12 recommendations for 7 clinical questions using GRADE.
• For untreated patients with major pulmonary involvement at higher risk of death or permanent disability, glucocorticoids are recommended to improve or preserve FVC and quality of life (strong recommendation, low quality evidence).
• If pulmonary disease continues or glucocorticoid side effects are unacceptable, adding methotrexate is suggested, and if disease still continues, adding infliximab.
• Glucocorticoids are recommended for cardiac sarcoidosis with heart block, dysrhythmias or cardiomyopathy, and for clinically significant neurosarcoidosis (strong recommendations, very low quality evidence).
• For troublesome fatigue, a pulmonary rehabilitation programme and/or inspiratory muscle training for 6-12 weeks is suggested, and an 8-week trial of D-methylphenidate or armodafinil may be considered.

Overview

This ERS guideline, developed by clinicians, methodologists and patients, covers treatment of pulmonary, cutaneous, cardiac and neurological sarcoidosis and sarcoidosis-associated fatigue. Glucocorticoids remain the first choice for symptomatic disease, but prolonged use causes significant toxicity, so glucocorticoid-sparing alternatives are important. No recommendation was possible for small-fibre neuropathy, and ocular disease, pulmonary hypertension and transplantation were not evaluated.

Skin and neurological disease

For cosmetically important active skin lesions not controlled by local treatment, oral glucocorticoids may be considered, and infliximab may be added if disease continues despite glucocorticoids or other immunosuppressants. In neurosarcoidosis that continues despite glucocorticoids, methotrexate is suggested, and infliximab if disease continues after a second-line agent such as methotrexate, azathioprine or mycophenolate mofetil.

Relapse and treatment duration

Relapse is common when treatment is withdrawn: 20%-80% of patients relapse after stopping glucocorticoids given for 2 years, systemic therapy had to be restarted in 80% after stopping methotrexate in chronic disease, and more than half relapsed after stopping infliximab at 6-12 months. The need for continued treatment should therefore be re-evaluated every 1-2 years.

FAQ

Does everyone with sarcoidosis need treatment?

No. Treatment is aimed at patients at risk of death or organ failure or with impaired quality of life, depending on which organ causes symptoms.

What if glucocorticoids are not enough or cause side effects?

Methotrexate is suggested as an add-on, followed by infliximab if disease continues.

How is sarcoidosis-related fatigue managed?

With a 6-12-week pulmonary rehabilitation programme and/or inspiratory muscle training, and possibly an 8-week trial of D-methylphenidate or armodafinil if fatigue is not related to disease activity.

Source

Baughman RP, Valeyre D, Korsten P, et al. ERS clinical practice guidelines on treatment of sarcoidosis. Eur Respir J 2021;58:2004079. DOI: 10.1183/13993003.04079-2020. Copyright ©The authors 2021. Summary prepared by Medpresso from the original publication; it is not a substitute for the full text or for medical advice.