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General Information about Sarcoidosis

Sarcoidosis Week

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology.  It is characterized by tissue infiltration of mononuclear phagocytes, lymphocytes and noncaseating granulomas.  Systemic evaluation is necessary to confirm the diagnosis and to detect any other potential organ involvement.  As with most chronic disorders a multimodality approach including clinical, radiological and histopathological evaluation is recommended. The lung is the most commonly affected organ.

The clinical presentation varies widely.  30% of patients present with non-specific constitutional symptoms such as fever, fatigue, weight loss, and malaise. 30-50% of patients are asymptomatic at the time of diagnosis. 

Evaluation includes a detailed history including occupational and environmental exposures.  Routine screening tests must include; chest radiograph (90% of patients have pulmonary involvement),  PFT's including DLCO, serum chemistries, calcium, liver enzymes, creatinine, ACE level, CBC with differential and urinalysis.  Patients should also have an EKG and PPD.  Finally, depending on the organ involved a bronchoscopy with biopsies, BAL with a CD4 CD8 ratio or biopsy of an affected organ should be considered. 

Clinico-radiologic findings alone are NOT adequate to make the diagnosis of sarcoidosis.  Clinical and radiologic findings supported by histologic evidence of noncaseating granulomas and exclusion of other causes of granulomas/local sarcoidosis reactions must occur before a definitive diagnosis is made.

Most patients can be divided into 2 groups, acute and chronic.  The acute form can present as a classical Lofgren's syndrome, characterized by fever, bilateral hilar adenopathy and erythema nodosum.  The chronic form, more insidious in onset, typically presents with symptoms of lupus pernio, uveitis, hypercalcemia, progressive pulmonary sarcoidosis/fibrosis, cystic bone lesions, neurosarcoidosis or myocardial involvement. In patients with suspected cardiac involvement, cardiac MRI has been shown to be of value.  It is more specific and sensitive than echocardiography.  PET scans are being increasingly used for evaluating systemic inflammatory activity but are not useful for diagnosis

The treatment of sarcoidosis is variable and in some instances controversial.   Not all patients with sarcoidosis require treatment.  Asymptomatic patients with normal PFT's, no vital organ involvement can be monitored with PFTs/DLCO and blood work.  Many of these patients will spontaneously remit within 3 years.  Those with a more chronic presentation are less likely go into spontaneous remission and treatment should be offered treatment to palliate symptoms, improve quality of life and prevent end organ disease. 
In those in whom systemic therapy is indicated glucocorticoids remain the first choice.  However the exact starting dose and length of treatment remains undetermined.  Overall general recommendations suggest starting prednisone at a dose of 20-40 mg per day weaning to less than 10 mg a day if the patient continues to do well.  If unable to wean to less than 10 mg a day steroid sparing agents are either added to prednisone or replace prednisone in a stable patient. The toxicity of long term corticosteroids needs to be factored in to the treatment plan.

The antimetabolites are the most commonly used steroid sparing agents.  Methotrexate has the most evidence in terms of effectiveness (5-15mg/wk.); however other agents to be considered include azathioprine (50-150 mg daily- effective in neurosarcoidosis), leflunomide (10-20 mg daily) and mycophenolate (500-1500 mg BID).   If the patient responds they can maintained on an antimetabolite with or without prednisone. Patients' must be constantly monitored for any liver damage or bone marrow suppression.  Once again length of treatment remains undetermined but generally 1-2 years.

In the last decade biologic agents have changed the landscape in treatment of sarcoidosis.  The anti-TNF monoclonal antibodies have been reported as effective in patients with refractory neurologic, ocular and skin manifestations.  For chronic pulmonary disease there are case series reporting benefit with both infliximab and Adalimumab.  However not all TNF agents are effective.  Etanercept and Golimumab have not been shown to be effective in sarcoidosis.  Duration of therapy is unknown however it appears patients tapered off after a year of therapy often relapse and need maintenance for years.  Consensus opinion is that these drugs should be gradually withdrawn by decreasing intervals between doses.

Malignancy, TB, disseminated fungal infections and MS are contraindications to anti- TNF therapy.  In this instance Rituximab or Athcar Gel can be used. Rituximab is a monoclonal antibody that depletes CD20+B cells which appears to have immunomodulatory effects.  Athcar gel is an adrenocorticotropic hormone (ACTH) analogue found to be effective when given twice a week.

Sarcoidosis remains a challenging disease to diagnose and treat. Treatment remains a mixture of evidence-based recommendations and clinical judgment. There is good evidence based support for treatment of pulmonary sarcoidosis but treatment regimens for extra-pulmonary disease are currently unclear and consensus based rather than evidence based. During this sarcoidosis awareness week we also hope to point the significant need for more research in this area.



Quick Facts About Sarcoidosis

  1. Prevalence estimated at close to 200,000 in the United States

  2. Spontaneous remission occurs in 60-70% of patients; disease mortality is 1-5% (most common cause of death is respiratory failure) [Hunninghake et al. (1999)]. Among African Americans, the most affected U.S. group, the estimated lifetime risk of developing sarcoidosis might be as high as 2 percent.

  3. There is no cure for sarcoidosis, but the disease may get better on its own over time or with drug therapy. Drug treatments are used to relieve symptoms, reduce the inflammation of the affected tissues, reduce the impact of granuloma development, and may prevent the development of lung fibrosis or other irreversible organ damage.

  4. There is no evidence that pharmacological therapy prevents disease progression