Tuesday, August 11, 2020

A case of relapsing polychondritis

From a case report and mini review in the American Journal of Medicine:

McAdam and the Damiani/Levine diagnostic criteria. 12 RPC is diagnosed if 3 of 6 clinical findings are present: 1) auricular chondritis; 2) nonerosive inflammatory arthritis; 3) nasal chondritis; 4) ocular inflammation, including conjunctivitis, keratitis, scleritis, episcleritis, or uveitis; 5) laryngotracheal chondritis; and 6) cochlear or vestibular damage presenting as sensorineural hearing loss, tinnitus, or vertigo. A diagnosis of RPC also can be made if a patient meets one of 6 criteria AND has compatible cartilage biopsy histology or meets 2 of 6 criteria AND improves clinically after receiving corticosteroids or dapsone. 2

RPC is a rare inflammatory disease with a peak age of onset between ages 40 and 50 years and an estimated incidence of 3.5 cases per million people per year. 3 Cases have been diagnosed across all racial groups. Men and women are equally affected. 3 RPC is defined by abrupt-onset inflammation of the cartilaginous ear, nose, joints, laryngotracheobronchial tree, or heart valves. The disease usually follows an indolent, relapsing-remitting course but may also present fulminantly and threaten vision and organ function. 4 …

Up to one-third of cases of RPC present prior to, during, or after another disease. 6 The most commonly associated syndrome is systemic vasculitis, followed by rheumatoid arthritis and systemic lupus erythematosus.

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