Relapsing Polychondritis

What is is?
It's an immune mediated condition associated with inflammation in cartilaginous structures like the ear, nose and respiratory tract. A third of cases are associated with other disease, most commonly CTD, systemic vasculitis and MDS. It is frequently undiagnosed for a long time. The most common manifestations at time of presentation are:
-auricular inflammation- pinna, sparing the lobule. Ears become floppy when cartilage is damaged.
-nose pain, stuffiness, saddle nose deformity
-joints: parasternal is typical, peripheral joint disease is non-erosive
-eye: anything from uveitis (our pt had anterior uveitis), episcleritis, scleritis, conjunctivitis, or CN palsies

The diagnostic criteria:
- b/l auricular chondritis
- non-erosive seronegative inflammatory polyarthritis
- nasal chondritis
- respiratory tract chondritis
- ocular inflammation
- cochlear and or vestibular dysfunction

You need 3 of the above criteria, OR
2 criteria plus response to steroids, OR
1 criteria + +histology.

Treatment is high dose steroid, often prednisone 60mg po daily. Remember to look after their bones (calcium, vitamin D, alendronate, get a dexa scan) as they will be on high dose for a while. You taper when you have control over symptoms. Some needs chronic low dose steroids.

The disease is life-threatening when it involves your trachea and there is collapse, necessitating tracheostomy.

(Katharine Cheung MD, 9/3/10)