relapsing polychondritis Flashcards
what is the targeted autoAb?
type II collagen
what is the HLA association?
HLA-DR4
signs/symptoms
ears -> spares earlobes, can lead to cauliflower ear, and hearing loss
nose -> may lead to saddle nose deformity, decreased sense of smell
laryngeal/tracheal –> hoarseness, wheezing, risk of pneumonia
+ eye involvement or poly arthritis
1 and #2 cause of death
1 Infection (Pneumonia) #2 vasculitis followed by arterial aneurysm, airway collapse, renal failure, malignancy
what is the disease course?
chronic, recurrent episodes of inflammation with chondrolysis and structural collapse as cartilage is replaced by granulation tissue and fibrosis
what is MAGIC syndrome?
relapsing polychondritis + behcet
Tx
prednisone