Sarcoidosis

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General Information about Sarcoidosis

Sarcoidosis Week

Who is at risk?

Sarcoidosis occurs worldwide, in both sexes, and can develop at any age, with most patients between 20 and 40 years, with another peak over age 50 years.  Certain groups appear to be at increased risk, including northern European Whites and African Americans. Estimates of prevalence are very variable, ranging from 1 to 40 cases /100,000, and an estimated 2.5% lifetime prevalence in African Americans, who may also experience more debilitating disease.  How an individual’s genetic makeup affects disease is under active investigation. Familial clustering and “outbreaks” have been reported. 

Causes

The cause of sarcoidosis remains unknown. Sarcoid is thought to result from an abnormal immune response to some trigger, such a virus, bacteria, mold, or other occupational or environmental exposure, with the formation of small clusters of immune cells called granulomas in affected organs such as the lung. Intense research has studied the causes of sarcoidosis as in the NIH/NHLBI multicenter study ACCESS (A Case Control Etiologic Study of Sarcoidosis).  An on-going NIH/NHLBI multicenter study GRADS (Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis) is focusing on the role of genetics and the microbial lung environment. 

Symptoms

The symptoms and signs of patients with sarcoidosis are very variable; ranging from none to multiple symptoms. As sarcoidosis usually affects the lungs, respiratory symptoms such as shortness of breath and cough are common. Other common symptoms include fatigue, fever, swollen lymph nodes, weight loss, skin rashes or lesions, and eye symptoms.   

Diagnosis

Sarcoidosis can be difficult to diagnose given the disease variability. Clinicians should conduct a “head-to-toe” assessment, which typically includes a chest CT Scan to obtain a more detailed picture of the lungs and lymph nodes. A piece of tissue from the lungs or another affected organ such as skin is usually needed to confirm the diagnosis. A lung biopsy can be obtained from an outpatient procedure called a bronchoscopy. Alternative diseases such as infection or lymphoma should be excluded.

Treatment and Prognosis

Sarcoidosis can go away on its own, and no treatment is needed for asymptomatic or mildly affected patients. For serious cases, which typically involve the lungs and possibly other organs such as the heart, skin or eyes, corticosteroids are usually prescribed to reduce inflammation.  Other agents such as methotrexate or TNF-alpha antibody therapies may be administered. Response to treatment should be monitored, with specific tests depending on the organs involved. Chronic progressive lung disease and/or complications involving other organs can occur and be fatal. Lung transplant can be performed for severely affected patients.

 

 


 

Quick Facts About Sarcoidosis

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

  • 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)]

  • 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.

  • There is no evidence that pharmacological therapy prevents disease progression