I. Pathology

  1. Noncaseating granulomata formed by T cells & monocytes
  2. Organs usually affected: lung, skin, eye, lymph nodes

II. Epidemiology

  1. Slight female predominance and black more common than white
  2. Usually presents age 20-40
  3. Nonsmokers get it more often!

III. Clinical features

  1. Clinical manifestations are secondary to structural distortion of tissues from the granulomas; can get permanent loss of function, however, from prolonged involvement & scarring
  2. Clinical course is variable; can be acute/self-limiting but usually have insidious onset over months
  3. Consitutional sx: fever, fatigue, malaise, anorexia, weight loss
  4. Polyarthritis
  5. Pulmonic sx: cough, dyspnea, chest pain (most common sx); interstitial lung disease is the most common type of pulmonic involvement
  6. Peripheral, including thoracic, nontender lymphadenopathy (very common)
  7. Mild anemia due to BM involvement
  8. Skin involvement, esp. erythema nodosum (25% of pts)
  9. Eye involvement (25% of pts): sx include blurred vision, tearing, photophobia; can cause blindness
  10. Nasal stuffiness (20% of pts)

IV. Treatment

  1. Corticosteroids have traditionally been the mainstay of treatment
    1. In a systematic review of 8 randomized trials, oral corticosteroids were found to be ass'd with radiologic improvement c/w placebo; also improvements in pulmolnary function and sx (the latter two limited to pst with "stage 2" or "stage 3" disease); data on inhaled steroids is limited and inconclusive (JAMA 287:1301, 2002--AFP)