Hx
- Autoimmune-like episodic or progressive inflammation of cartilage and other connective tissue:
- Ear, Eye, Nose, Large airway, Joint, Heart, Kidney & skin are all involved
- Often comorbid disease
- M=F, 50yo ± 10 years, Caucasians
Diagnosis
- At least three of McAdam's diagnostic criteria
- ≥ 1 the above clinical findings with positive histolgy Dx
- Chondritis at two or more separate anatomic locations with response to steroids and/or dapsone
McAdams criteria
- Bilateral auricular chondritis
- Nonerosive, seronegative polyarthritis
- Nasal chondritis
- Ocular inflammation: conjunctivitis, keratitis, scleritis/episcleritis, uveitis
- Respiratory tract chondritis: laryngeal and/or tracheal cartilages
- Cochlear and/or vestibular dysfunction: neurosensory hearing loss, tinnitus and/or vertigo
Management
- Dapsone- 50- 200mg OD
- Steroids
- Cyclophosphamide
- Azathioprine
- Cyclosporine
References
Kent PD, Michet CJ Jr, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol 2004; 16:56.