The pharmacotherapeutics of sarcoidosis

ABSTRACT

Introduction

Sarcoidosis is a multi-system, inflammatory, and granulomatous disease that can damage multiple organs. Several drugs have been used to treat sarcoidosis, but few have randomized-controlled trials (RCTs) to understand their efficacy. This lack of RCTs and the heterogenous nature of sarcoidosis makes for a challenge to the provider caring for these patients.

Areas covered

Glucocorticoids remain the backbone of treatment of sarcoidosis. The side effect profile of glucocorticoids has resulted in the search for other sarcoid disease modifying drugs. This paper reviews the pharmacology, history, efficacy data, and adverse effects of alternative treatments. Most alternative sarcoidosis immune modulating treatments lack RCTs to evaluate their relative efficacy.

Expert opinion

Because of the variability of disease presentation and progression, the treatment of sarcoidosis is best managed by expert clinicians with a firm understanding of the pharmacology, pharmacokinetics, monitoring requirements, counter-indications, and adverse effects of agents used. More RCTs that compare agents in well-defined sarcoid subgroups are clearly needed.

KEYWORDS:

• Sarcoidosis
• sarcoid
• therapy
• glucocorticoid
• methotrexate
• azathioprine
• leflunomide
• mycophenolate mofetil
• hydroxychloroquine
• TNF inhibitors

Article highlights

• Given the large variability in clinical presentation, treatment decisions for patients with sarcoidosis are highly nuanced and requires extensive clinical experience.

• Specific acute clinical manifestations of sarcoidosis such as Lofgren’s syndrome typically do not require therapy and can be observed for an extended period.

• Sarcoidosis with cardiac disease or neurologic disease almost uniformly require prompt early initiation of aggressive immunosuppression.

• The presence of specific chest radiographic patterns or presence of extrapulmonary sarcoidosis help the clinician in deciding when to initiate therapy.

• Knowledge of the various typical clinical patterns of sarcoidosis are a useful guide to therapy, but close monitoring of individual patients and their clinical trajectory is key.

Declaration of interest

The authors have no 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 this manuscript. This includes employment, consultations, honoraria, stock ownership or options, grants or patients received or pending royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This manuscript was not funded.