Ear
Inflammatory / infectious / autoimmune / systemic disorders
Relapsing polychondritis

Author: Nat Pernick, M.D. (see Authors page)

Revised: 12 February 2018, last major update October 2013

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Relapsing polychondritis [title] ear pathology

Cite this page: Pernick, N. Relapsing polychondritis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/earrelapsingpolychondritis.html. Accessed October 16th, 2018.
Definition / general
  • Also called polychondropathia
  • Uncommon systemic episodic or relapsing disease with progressive degeneration of cartilage throughout the body
  • Probable autoimmune process (antibodies to type II collagen) associated with other autoimmune disorders
  • Whites, no gender preference, usually symptomatic in 40s to 60s although affects all ages
  • 90% have involvement of auricular cartilage, usually bilateral, with swelling, erythema and tenderness
  • Earlobes are typically spared
  • Variable relapsing of disease
  • May cause cauliflower ear and saddle node deformities
  • Clinical diagnosis requires 3 of the following - (a) recurrent chondritis of both auricles; (b) nonerosive inflammatory arthritis; (c) chondritis of nasal cartilage; (d) ocular inflammation including conjunctivitis, keratitis, scleritis, episcleritis or uveitis; (e) chondritis of upper respiratory tract including larynx or tracheal cartilage; (f) cochlear or vestibular damage with sensorineural hearing loss, tinnitus or vertigo
Laboratory
  • Nonspecific elevated sedimentation rate, mild leukocytosis, normochromic normocytic anemia; variable elevated ANCA
  • Prognosis varies from prolonged course to aggressive and fulminant disease leading to death from respiratory tract or cardiovascular involvement (aortic insufficiency)
Treatment
  • Responds to steroids or dapsone (this also confirms diagnosis)
  • Advanced cases require immunosuppressive agents
Microscopic (histologic) description
  • Mixed inflammatory infiltrate (lymphocytes, plasma cells, neutrophils, occasional eosinophils) extending into cartilage with blurring of interface between cartilage and adjacent soft tissue
  • Cartilage shows loss of normal basophilia, loss of chondrocytes and destruction of lacunar architecture at advancing edge of inflammation with cartilage replaced by fibrous tissue
Positive stains