Sarcoidosis Treatment & Management

  • Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Zab Mosenifar, MD   more...
 
Updated: Jul 29, 2011
 

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.[11]
  • 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.[12, 13]
  • Cyclophosphamide has been rarely used with modest success as a steroid-sparing treatment in patients with refractory sarcoidosis.[14, 15]
  • Azathioprine is another second-line therapy, which is best used as a steroid-sparing agent rather than as a single-drug treatment for sarcoidosis.[16]
  • Chlorambucil is an alkylating agent that may be beneficial in patients with progressive disease unresponsive to corticosteroids or when corticosteroids are contraindicated.[17]
  • 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.[18]
  • Infliximab[2, 3] and thalidomide[19] 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.[20]
  • Tetracyclines have shown promise for the treatment of cutaneous sarcoidosis.[21]

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.

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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.[22]

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Contributor Information and Disclosures
Author

Nader Kamangar, MD, FACP, FCCP, FCCM  Associate Professor of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of California, Los Angeles, David Geffen School of Medicine, Olive View-UCLA Medical Center; Associate Program Director, Pulmonary and Critical Care Multi-Campus Fellowship Program, Cedars-Sinai/West Los Angeles Veterans Affairs/Los Angeles Kaiser Permanente/Olive View-UCLA Medical Center; Site Director, Pulmonary/Critical Care Fellowship Program, Olive View-UCLA Medical Center

Nader Kamangar, MD, FACP, FCCP, FCCM is a member of the following medical societies: American Academy of Sleep Medicine, American Association of Bronchology, American College of Chest Physicians, American College of Physicians, American Lung Association, American Medical Association, American Thoracic Society, California Thoracic Society, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

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

Andrew F Shorr, MD, MPH is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP  Professor of Genomics and Personalized Medicine Research, Internal Medicine, and Pediatrics, Associate Director, Center for Genomics and Personalized Medicine Research, Director of Research, Section on Pulmonary, Critical Care, Allergy and Immunologic Diseases, Wake Forest University School of Medicine

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society, and Sigma Xi

Disclosure: See below for list of all activities None None

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Om Prakash Sharma, MD, FRCP, FCCP, DTM&H  Professor, Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Southern California Keck School of Medicine

Om Prakash Sharma, MD, FRCP, FCCP, DTM&H is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Osler Society, American Thoracic Society, New York Academy of Medicine, and Royal Society of Medicine

Disclosure: Nothing to disclose.

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD  Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Professor and Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society

Disclosure: Nothing to disclose.

References

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Stage I sarcoidosis.
Stage II sarcoidosis.
Stage III sarcoidosis.
Noncaseating granuloma.
Table 1. Prognosis
StageRemission (%)Asymptomatic at 5 y (%)CXR Clearing (%)Mortality (%)
Stage I60-9095540
Stage II40-70583111
Stage III10-20251018
Stage IV0N/A0N/A
Table 2. Results of Multicenter Trial Sponsored by the British Thoracic Society
CharacteristicsGroup L*Group S†P
Dyspnea score (range 1-4)0.240.47NS
Fibrosis score (range 0-16)0.831.47NS
FEV1‡ (% predicted)95.986.90.05
VC§ (% predicted)99.890.80.02
DLCOII (% predicted)84.377.7NS
Weight gain (kg)+3.26+0.990.02
*Long-term steroids



†Short bursts of steroids



‡Forced expiratory volume in 1 second



§Ventilatory capacity



II Diffusing capacity of lung for carbon monoxide



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