Ear
Inflammatory / infectious / autoimmune / systemic disorders
Relapsing polychondritis
Author: Nat Pernick, M.D.
Last author update: 1 October 2013
Last staff update: 15 December 2020
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Relapsing polychondritis [title] ear pathology
Table of Contents
Definition / general | Laboratory | Treatment | Microscopic (histologic) description | Positive stains | Additional referencesCite this page: Pernick N. Relapsing polychondritis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/earrelapsingpolychondritis.html. Accessed June 3rd, 2023.
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
- Granular deposition of IgG and C3 in perichondrial fibrous tissue (Hum Pathol 1980;11:19)
Additional references
