Relapsing Polychondritis

186_mediumEPISODIC, PROGRESSIVE, AUTOIMMUNE INFLAMATION OF CARTILAGE

+ TYPE III COLLAGEN ANTIBODIES

HIGH ESR, ANEMIA

ASSOC. WITH HASHIMOTOS, SJOGREN’S, SCLERODERMA

A CLINICAL Dx

FEMALE>MALE (AGE 35-45)

USUALLY SELF LIMITING

Bx SHOWS ACUTE AND CHRONIC INFLAMMATION, NECROSIS, AND CALCIFICATION

NEED 3/6 TO MAKE THE Dx:

1. PAINFUL RECURRENT AURICULAR CHONDRITIS = THE MOST COMMON Sx

CARTILAGE IS EVENTUALLY DESTROYED—“FELTY FEEL”-(CHARACTERISTICALLY SPARES THE EAR LOBES—UNLIKE PSEUDOMONAL PERICHONDRITIS)

2. NASAL SEPTAL CHONDRITIS (75%)–POSSIBLE SADDLE NOSE DEFORMITY

3. NON-ERODING POLYARTHRITIS (50%)

4. OCULAR INFLAMMATION (THINK VS. COGAN’S) CHECK MHATP

5. LARYNGOTRACHEAL CHONDRITIS –CAN LEAD TO MALACIC AIRWAY (PRIMARILY AFFECTS THE SUBGLOTTIS–SUBGLOTTIC STENOSIS)–THE PRIMARY CAUSE OF RELATED MORTALITY

6. COCHLEAR OR VESTIBULAR LESIONS–30% HL–PRIMARILY SNHL-STERIODS CAN IMPROVE–SECONDARILY CARTILAGENOUS ETD-OME-CHL

Rx: STEROIDS, ASA, NSAIDS, DAPSONE, CYCLOPHOSPHAMIDE, AZOTHIAPRINE

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