Description

• Chronic, multisystem disorder characterized by local accumulation of T lymphocytes and mononuclear phagocytes forming noncaseating epithelioid granulomas
• Symptoms mainly due to organ dysfunction due to disruption of local tissue architecture:
  • Predominance of lung symptoms
• ACE and vitamin D levels (and thus calcium levels) may be increased due to secretion from granulomatous tissue
• Prevalence 10–20/100,000 in the U.S. and Europe
• Affects almost all races and geographic locations
  • People of African descent higher predominance
  • 2.4% lifetime risk to blacks in the U.S., whites 0.85%
• Symptoms typically begin in patients 10–40 yr of age

Etiology

Unclear, but appears to be an overly robust cell-mediated immune response to unidentified self- or nonself antigen(s). Familial clustering suggests genetic component