Ear

Inflammatory / infectious / autoimmune / systemic disorders

Relapsing polychondritis



Topic Completed: 1 October 2013

Minor changes: 15 December 2020

Copyright: 2002-2021, PathologyOutlines.com, Inc.

PubMed Search: Relapsing polychondritis [title] ear pathology


Nat Pernick, M.D.
Page views in 2020: 1,092
Page views in 2021 to date: 1,115
Cite this page: Pernick N. Relapsing polychondritis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/earrelapsingpolychondritis.html. Accessed December 8th, 2021.
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
Back to top
Image 01 Image 02