Treatment Options for Pulmonary Sarcoidosis and Steroid-Induced Diabetes

Robert Fox, MD, PhD

Disclosures

October 05, 2007

Question

What are the treatment options for a patient with pulmonary sarcoidosis who has had repeat failed treatment with methotrexate, infliximab, and etanercept?

Amir Alvi, MBBS, MRCP

Response from the Expert

 

Robert Fox, MD, PhD
Professor/Member Scripps Memorial Hospital and Research Foundation; Rheumatologist, Scripps Memorial Hospital, LaJolla, California

 

Sarcoidosis is a systemic inflammatory disorder of unknown etiology. Although any organ may be involved, the lungs are most frequently affected. The clinical course of the disease is highly variable, with up to two thirds of untreated patients experiencing spontaneous remission within 12-24 months of onset of symptoms. Treating patients with sarcoidosis can be difficult; however, treatment options include leflunomide or a combination of methotrexate and leflunomide.[1] For these patients it is important to determine whether the lung lesions are "active" sarcoidosis, which are progressive in nature or represent residual fibrotic scarring, because residual fibrosis may not be responsive to therapy. High-resolution chest computed tomographic (CT) scans, bronchoscopy, and serologic markers may help make this distinction. Also, other lesions that masquerade conditions, such as tuberculosis or atypical mycobacterial infections, need to be excluded. In the presence of documented noncaseating granulomatous disease that is life-threatening, cytotoxic agents may be warranted.

Comment

Although the treatment of sarcoidosis is controversial, corticosteroids remain the cornerstone of therapy. Immunosuppressive, cytotoxic, and immunomodulatory agents have, however, emerged as viable therapeutic options for patients failing or experiencing adverse effects from corticosteroids. The majority of published data on treatment options are with methotrexate; however, some data showing favorable responses have been noted with leflunomide, azathioprine, and antimalarial and antimicrobial agents, as well as tumor necrosis factor-alpha inhibitors.[1] As in rheumatoid arthritis patients (with and without lung involvement), combination therapy with these agents may be required.

Because angiotensin-converting enzyme (ACE) is used as a marker for sarcoid activity, additonal treatment options include ACE inhibitors. Remission of cutaneous and lymphatic sarcoidosis in a patient treated with an ACE inhibitor was recently reported in a patient treated for congestive heart failure and hypertension[2]; the remission has continued over 4 years of follow-up. Because this is a report of only 1 case, there is a possibility of sampling error. Whether the patient's remission in this case was a serendipitous spontaneous remission that happened to occur during ACE inhibitor therapy or whether ACE inhibitor therapy can play a role in the treatment of sarcoidosis needs to be determined in a large clinical trial.

Minocycline has been reported useful in anecdotal cases.[3] Limited studies with antimicrobial treatment have demonstrated improvement in sarcoidosis. Consequently, it has been suggested that assessment of patients with sarcoidosis should include screening for the presence of microbes, and antimicrobial treatment should be considered in cases resistant to corticosteroids.[3]

In summary, sarcoidosis can be difficult to manage. When therapy is required, corticosteroids are considered standard, but studies demonstrating their ability to modify the long-term outcome in this disease are lacking. Often, the myriad of adverse side effects from corticosteroids necessitate the addition of immunosuppressants, cytotoxic agents, or biologic therapies to maintain disease remission. Unfortunately, optimal therapeutic regimens have not been described. Patients who do not respond to therapy often experience progressive fibrotic changes and end-organ damage, which ultimately may result in significant morbidity or death.[4]

Comments

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