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Treatment of pulmonary sarcoidosis: Disease refractory to glucocorticoid therapy

Talmadge E King, Jr, MD
Section Editor
Kevin R Flaherty, MD, MS
Deputy Editor
Helen Hollingsworth, MD


Sarcoidosis is a multisystem disease of unknown etiology characterized by tissue infiltration with noncaseating granulomas. The granulomas may occur in any organ, but the most frequently affected sites are the lungs, lymph nodes, skin, eyes, and liver. Patients with pulmonary sarcoidosis typically present without symptoms, but with an abnormal chest radiograph obtained for an unrelated reason. When symptomatic, patients usually report dyspnea, cough, or nonspecific chest discomfort. Spontaneous resolution of the disease is common, but progressive and disabling organ failure can occur in up to 10 percent of patients.

Glucocorticoids are the most commonly used medication for the treatment of pulmonary sarcoidosis, although no medications have been approved by the US Food and Drug Administration (FDA) for the treatment of sarcoidosis [1-5]. However, some patients with sarcoidosis cannot tolerate or do not respond to glucocorticoids. Several alternative approaches have been proposed, such as the use of immunosuppressive, cytotoxic, and antimalarial drugs [6]. In addition, irradiation has been used for neurosarcoidosis and organ transplantation has been performed successfully for end-stage hepatic, renal, cardiac, or pulmonary disease complicating sarcoidosis. Unfortunately, limited data exist regarding the indications and efficacy of these approaches in the management of pulmonary sarcoidosis.

The treatment of pulmonary sarcoidosis with alternatives to glucocorticoids will be reviewed here. The diagnosis, treatment approach, and use of glucocorticoids for sarcoidosis affecting the lungs and other organs are discussed separately. (See "Clinical manifestations and diagnosis of pulmonary sarcoidosis" and "Treatment of pulmonary sarcoidosis: Initial therapy with glucocorticoids" and "Neurologic sarcoidosis", section on 'Treatment' and "Sarcoid arthropathy", section on 'Treatment' and "Renal disease in sarcoidosis" and "Gastrointestinal and hepatic sarcoidosis", section on 'Treatment' and "Cardiac sarcoidosis", section on 'Treatment'.)


Progressive and disabling respiratory impairment complicates pulmonary sarcoidosis in up to 10 percent of patients. For these patients, intensification of therapy with alternatives to glucocorticoids typically involves immunosuppressive agents. Other patients may be intolerant of glucocorticoids and require immunosuppressive therapy in that basis. Many of these patients may benefit from referral to a sarcoidosis center.

Indications — Patients with sarcoidosis who may benefit from agents other than, or in addition to, glucocorticoids generally have one or more of the following features:


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