eMedicine Specialties > Pulmonology > Interstitial Lung Diseases

Sarcoidosis: Treatment & Medication

Author: Nader Kamangar, MD, FACP, FCCP, FAASM, Assistant Professor of Medicine, Director of Hospitalist/Intensivist Program, Division of Pulmonary, Critical Care and Sleep Medicine, David Geffen School of Medicine at University of California Los Angeles; Associate Director, Combined Pulmonary and Critical Care Fellowship Program, Cedars-Sinai/Olive View-UCLA/West Los Angeles Veterans Affairs Medical Center
Coauthor(s): Andrew F Shorr, MD, MPH, Assistant Professor, Department of Internal Medicine, Division of Pulmonary and Critical Care, Uniformed Services University of the Health Sciences
Contributor Information and Disclosures

Updated: Apr 17, 2009

Treatment

Medical Care

Few reliable studies on disease indications and optimal treatment exist. Most patients (>75%) require only symptomatic therapy (nonsteroidal anti-inflammatory drugs). Approximately 10% of patients need treatment for extrapulmonary disease, while 15% of patients require treatment for persistent pulmonary disease.

• Corticosteroids are the mainstay of therapy.
  • Generally, prednisone given daily and then tapered over a 6-month course is adequate for pulmonary disease. Earlier recommendations suggested an initial dose of 1 mg/kg/d of prednisone; however, more recent expert opinions endorse a lower dose (eg, 40 mg/d), which is tapered to every other day long-term therapy over several weeks. Most patients who require long-term steroids can be treated using 10-15 mg of prednisone every other day.
  • Data suggest that corticosteroid use may be associated with increased relapse rates.
  • Occasionally, certain patients cannot tolerate or do not respond to corticosteroids.
  • High-dose inhaled corticosteroids may be an option, but conclusive data are lacking.
• Noncorticosteroid agents are being used more frequently. Common indications for the initiation of such agents include steroid-resistant disease, intolerable adverse effects, or patient desire not to take corticosteroids.
  • Methotrexate (MTX) has been a successful alternative to prednisone and is a steroid-sparing agent.⁶
  • Chloroquine and hydroxychloroquine are antimalarial drugs with immunomodulating properties, which have been used for cutaneous lesions, hypercalcemia, neurological sarcoidosis, and bone lesions. Chloroquine has also been shown to be efficacious for the treatment and maintenance of chronic pulmonary sarcoidosis.^7,8
  • Cyclophosphamide has been rarely used with modest success as a steroid-sparing treatment in patients with refractory sarcoidosis.^9,10
  • Azathioprine is another second-line therapy, which is best used as a steroid-sparing agent rather than as a single-drug treatment for sarcoidosis.¹¹
  • Chlorambucil is an alkylating agent that may be beneficial in patients with progressive disease unresponsive to corticosteroids or when corticosteroids are contraindicated.¹²
  • Cyclosporine is a fungal cyclic polypeptide with lymphocyte-suppressive properties that may be of limited benefit in skin sarcoidosis or in progressive sarcoid resistant to conventional therapy.¹³
  • Infliximab^2,3 and thalidomide¹⁴ have been used for refractory sarcoidosis, particularly for cutaneous disease. Infliximab appears to be an effective treatment for patients with systemic manifestations such as lupus pernio, uveitis, hepatic sarcoidosis, and neurosarcoidosis. Callejas-Rubio et al reported inconsistent results with TNF-inhibitor therapy.¹⁵
  • Tetracyclines have shown promise for the treatment of cutaneous sarcoidosis.¹⁶
• For pulmonary disease, asymptomatic PFT and/or CXR abnormalities are not an indication for treatment. In patients with minimal symptoms, serial reevaluation is prudent. Significant respiratory symptoms associated with PFT and CXR abnormalities likely require therapy. For such patients, treatment is indicated if objective evidence of recent deterioration in lung function exists. Corticosteroids can result in small improvements in the functional vital capacity and in the radiographic appearance in patients with more severe stage II and III disease.
• One recent study demonstrated an approach that may minimize the use of corticosteroids without harming the patient. This is accomplished by withholding therapy unless the patient shows at least a 15% decline in one spirometric measure associated with increasing symptoms or, if asymptomatic, withholding therapy unless the patient shows worsening PFTs and a change in CXR.
• For extrapulmonary sarcoidosis involving such critical organs as the heart, liver, eyes, kidneys, or central nervous system, corticosteroid therapy is indicated.
• Topical corticosteroids are effective for ocular disease.
• Inhaled corticosteroids are occasionally used, in particular in patients with endobronchial disease.

Surgical Care

For patients with advanced sarcoid-induced pulmonary fibrosis, lung transplantation remains the only hope for long-term survival.

Lung transplantation is a viable option for patients with stage IV sarcoidosis. Transplantation in such patients should be strongly considered when the forced vital capacity falls below 50% predicted and/or the forced expiratory volume in 1 second falls below 40% predicted.¹⁷

Medication

Because medical treatment focuses on anti-inflammatory therapies, corticosteroids remain the foundation of treatment.

Nonsteroidal anti-inflammatory drugs (NSAIDs)

NSAIDs are indicated for the treatment of arthralgias and other rheumatic complaints. Patients with stage I sarcoidosis often require only occasional treatment with NSAIDs.

Ibuprofen (Motrin, Ibuprin, Advil)

Ibuprofen and other NSAIDs are useful in the management of joint complaints. They are not indicated for treatment of significant pulmonary disease.

Ketoprofen (Actron, Orudis, Oruvail)

For relief of mild to moderate pain and inflammation.
Small initial dosages are indicated in small and elderly patients and in those with renal or liver disease.

Corticosteroids

The cornerstone of therapy; have potent immunologic effects that ameliorate many signs and symptoms.

Prednisone (Deltasone, Orasone, Sterapred)

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.
Response may be rapid but often is seen over 12-16 wk.

Antimetabolites

Given the adverse side-effect profile of corticosteroids, methotrexate has recently received significant attention as either a corticosteroid alternative or a corticosteroid-sparing agent.

Methotrexate (Folex PFS, Rheumatrex)

Antimetabolite that interferes with folate metabolism. Has been very successful in treating rheumatoid arthritis. The effects often take months to manifest, so it should be used initially with corticosteroids. As the drug's level increases, corticosteroids can be tapered.

Antimalarial agents

Previously employed for the treatment of rheumatoid arthritis. Literature supporting its use in sarcoidosis is limited to case series. Has a relatively benign side-effect profile.

Hydroxychloroquine (Plaquenil)

May be most useful in the management of osseous involvement. Inhibits chemotaxis of eosinophils, locomotion of neutrophils, and impairs complement-dependent antigen-antibody reactions.

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