= rare debilitating multisystem inflammatory + autoimmune disorder characterized by recurrent episodes of inflammation + destruction of cartilage in joints, ears, nose, larynx, airways

Etiology: acquired metabolic disorder (? abnormal acid mucopolysaccharide metabolism) / hypersensitivity / autoimmune process (antibodies directed against type II collagen)

Histo: loss of cytoplasm in chondrocytes; plasma cell and lymphocyte infiltration

Age: 40–60 years (no age predilection)

• nasal chondritis = saddle-nose deformity
• bilateral auricular chondritis = swollen + tender ears, cauliflower ears; ocular inflammation
• hearing loss ← obstruction of external auditory meatus / audiovestibular damage)
• cough, hoarseness, dyspnea ← collapse of trachea
• nonerosive seronegative inflammatory polyarthritis = arthralgia
• Head
  • calcification of pinna of ear
• Chest
  • manubriosternal / costochondral arthropathy (30%)
• Respiratory tract (in up to 70%)
  • ectasia + collapsibility (← cartilaginous destruction) of trachea and mainstem bronchi with focal thickening (← mucosal edema) + luminal narrowing (← fibrosis)
  • bronchiectasis
  • generalized + localized emphysema
  • Cardiovascular (in 15–46%)
    Site: elastic elements of cardiac valves + aorta
    Histo: cystic degeneration of collagen, destruction of elastic fibers, lymphocytic infiltration, decreased content of acid mucopolysaccharides
  • aortic aneurysm (4–10%), mostly in ascending aorta, may be multiple / dissecting
  • aortic / mitral valve insufficiency (8%)
  • aortitis + systemic vasculitis (13%)
  • obliterating vasculitis in medium-sized + large arteries
• Bone
  • periarticular osteoporosis
  • erosive changes in carpal bones resembling rheumatoid arthritis
  • soft-tissue swelling around joints + styloid process of ulna
  • erosive irregularities in sacroiliac joints
  • disk space erosion + increased density of articular plates

Rx: corticosteroids, immunosuppression

Prognosis: 74% 5-year survival rate; 55% 10-year survival rate; median survival time of 11 years; airway complications account for >50% of deaths