Sarcoidosis: an update on current pharmacotherapy options and future directions

ABSTRACT

Introduction: Sarcoidosis is a systemic disease of unknown etiology characterized by the development of non-caseating epitheloid granulomas. The lungs are the most commonly involved organ (>90% of cases), followed by the lymph nodes, the skin, and the eyes.

Areas covered: This review summarizes current pharmacotherapy options and future directions for the development of new therapies. Glucocorticoids are the first-line therapy for sarcoidosis. For patients with the most severe forms of sarcoidosis (who will need glucocorticoids for long periods) and for those intolerant or refractory, immunosuppressive drugs are used as sparing agents. The management of extrathoracic sarcoidosis must be tailored to the specific organ or organs involved; however, there is limited data from controlled trials to guide the treatment of these patients. The emergence of biological therapies has increased the therapeutic armamentarium available to treat sarcoidosis, with monoclonal anti-TNF agents being the most promising, but their use is still limited by a lack of licensing and costs.

Expert commentary: The treatment of sarcoidosis is still not totally standardized. New effective therapies are urgently needed to enable the reduction or replacement of long-term therapy with glucocorticoids in patients with sarcoidosis.

KEYWORDS:

• Sarcoidosis
• therapy
• glucocorticoids
• methotrexate
• azathioprine
• leflunomide
• infliximab
• adalimumab

Article highlights

• Not all patients with sarcoidosis should be considered candidates for systemic therapy.

• Glucocorticoids are the first-line therapy in patients for whom a decision is made to treat.

• Methotrexate is the first choice as second-line, glucocorticoid-sparing therapy.

• Anti-TNF agents are third-line therapies after the failure of glucocorticoids and methotrexate.

• An increasing number of reported cases of sarcoidosis induced by biological agents are reported.

• With the lack of available data, avoid switching to infliximab biosimilars in patients currently treated with the infliximab reference product.

This box summarizes key points contained in the article.

Acknowledgments

The authors wish to thank David Buss for editorial assistance.

Declaration of interest

P Brito-Zerón is supported by ‘Ajut per a la Recerca Josep Font’ (Hospital Clinic-Barcelona 2012). R P Baughmanhas received research grants from Genentech, Mallinckrodt, Janssen, and Celgene regarding treatment for sarcoidosis. He has spoken on behalf of Mallinckrodt. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Additional information

Funding

This paper is supported by grant Fondo de Investigaciones Sanitarias INT15/00085.