Over the past 6 months, Tom, a 40-year old, African American man, developed a low-grade fever, joint pain of the knees and ankles, fatigue, cough, and shortness of breath.  Multiple red and tender nodules were noted on his lower extremities.  His chest x-ray revealed bilateral mediastinal lymph node enlargement.  Subsequent lymph node biopsy revealed non-caseous granuloma.  The diagnosis of sarcoidosis was established.  He was treated with prednisone to control his symptoms.

Sarcoidosis is an autoimmune and inflammatory disease characterized by the formation of granulomas which are small nodules with clumps of immune and inflammatory cells found in one or more organs of the body.  Cytokineses such as tumor necrosis factor (TNF) also plays a significant role in granuloma formation.  The precise cause of sarcoidosis is unknown. Granuloma formation is the result of an exaggerated immune response to undefined environmental triggers such as infection or chemicals, particularly in genetically predisposed individuals.  Progressive granuloma can interfere with an organ’s structure and function, while chronic inflammation can lead to fibrosis and scarring of organ tissue.

Sarcoidosis is most common in Scandinavians but can occur in all parts of the world.  In the United States, it is more common in African-Americans than Caucasians.  Furthermore, African-Americans with sarcoidosis tend to have more severe symptoms and chronic disease progression.  Sarcoidosis usually begins between the ages of 20 and 50, and occurs more often in women than men.  Children are rarely affected.

Some patients develop tender and nodules (erythema nodosum) and arthritis of the lower extremities.  Sarcoidosis can also affect almost any organ in the body, including the liver, spleen, and even spinal cord and brain.  Roughly 50% of people with sarcoidosis will go into remission within a year of displaying symptoms even without any specific treatment.  However, an estimated 10-15% of cases become progressive with severe chronic disease.   Ninety-percent or more of people diagnosed with this disease have lung (pulmonary sarcoidosis) and intrathoracic lymph node involvement.  Patients can experience fatigue, coughing, shortness of breath, or chest pain.  Depending on the type of lung involvement, pulmonary sarcoidosis is classified among four different stages.  The prognosis of the last stage is poor and requires aggressive treatment.

Corticosteroids are very effective in reducing inflammation and are frequently the first drugs used in treating sarcoidosis with mild to moderate disease activity.  Other treatments such as Hydroxychloroquine, Methotrexate, Leflunomide or Azathioprine may be used in moderate sarcoidosis to decrease the long-term side effects of high dose steroids.  Anti-TNF factor biologics, especially Remicade (infliximab) or Humira (adalimumab) have proven to be effective in treatment of severe sarcoidosis affecting vital organs.

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