Ann IM 1998;129:114

Pathophys:Autoimmune

M/F = 1:3. Associated with systemic vasculitis (10%), RA (7%), SLE (5%), Hashimoto’s (4%), other fibrosing syndromes (9%)

Sx:Onset age 13-84 yr, median age is 51 yr; arthritis (52%); ocular (51%); laryngotracheal sx (48%); saddle nose (29%); hearing loss (26%); vertigo (13%)

Si:Auricular chondritis (85%); nasal chondritis (54%); laryngotracheal sx (48%); fever (40%); skin changes (28%); episcleritis

5-yr survival = 74%, 10-yr = 55%; fluctuating progressive course

Systemic vasculitis death (5%), pneumonia (5%), airway collapse (2%)

Lab:

Hem:ESR elevated (82%), anemia (55%)

Urine:Hematuria (26%), proteinuria (14%)

Rx: Steroids, 60% respond (Nejm 1978;299:1203); possibly methotrexate, dapsone, cyclophosphamide, azathioprine