Connective Tissue Diseases Codexery

Atrophic polychondritis

Recurrent cartilage inflammation that can affect multiple body systems.

The disease affects men and women equally, with an annual incidence of about 3.5 cases per million.[1] Diagnosis is often challenging because symptoms vary and can be delayed for months or years.[2] In relapsing polychondritis, cartilage tissues are attacked and destroyed by the body's own immune system.[3]

Quick Facts

Annual incidence
about 3.5 cases per million
Peak age range
40 to 50 years
Sex distribution
occurs as often in men as in women
Ear involvement at presentation
about 20%
Cauliflower ear deformity occurrence
about 10%
Nasal involvement at presentation
15%

Facts from the source article.

Key facts and history

Cartilage inflammation that relapses is very characteristic of the disease.[4] Involvement of the laryngotracheobronchial cartilages causes one-third of all deaths among persons with RP.[5] The initial identification of the condition was made by Rudolf Jaksch von Wartenhorst in 1923, who gave it the name Polychondropathia.[6] A 32-year-old male brewer was the patient described, exhibiting fever, asymmetric polyarthritis, along with painful, swollen, and deformed ears and nose.[1]

Jaksch von Wartenhorst believed the disorder was degenerative and linked its origin to the patient's high alcohol consumption.[1] Pearson and his team introduced the term Relapsing Polychondritis in 1960, which is the designation used today.[7] At the time of initial presentation, ear cartilage inflammation is seen in roughly 20% of individuals, and eventually occurs in 90%.[8] The period of life with the greatest frequency of onset is between 40 and 50 years of age.[1]

What the research sources say

Clinical criteria for diagnosing RP were first put forward by McAdam et al. in 1976.[9] Damiani et al. broadened those criteria in 1979, and later Michet et al. made revisions in 1986.[9] Dapsone, at dosages ranging from 25 mg/day up to 200 mg/day, has been reported as effective in multiple case reports.[10]

References

  1. Wikipedia, "Relapsing polychondritis" (CC BY-SA 4.0), which cites: "Relapsing polychondritis", Rheumatic Disease Clinics of North America, May 2013, p. 263–76 (original not read by us) en.wikipedia.org
  2. Wikipedia, "Relapsing polychondritis" (CC BY-SA 4.0), which cites: Puéchal, "Relapsing polychondritis", Joint Bone Spine, March 2014, p. 118–24 (original not read by us) en.wikipedia.org
  3. Wikipedia, "Relapsing polychondritis" (CC BY-SA 4.0), which cites: "Relapsing Polychondritis: Autoimmune Disorders of Connective Tissue", Merck Manual Home Health Handbook (original not read by us) en.wikipedia.org
  4. Wikipedia, "Relapsing polychondritis" (CC BY-SA 4.0), which cites: Puéchal, "Relapsing polychondritis", Joint Bone Spine, March 2014, p. 118–24; Puéchal, "Relapsing polychondritis", Joint Bone Spine, March 2014, p. 118–24 (original not read by us) en.wikipedia.org
  5. Wikipedia, "Relapsing polychondritis" (CC BY-SA 4.0), which cites: Drosos, "Prof.", Orphanet Encyclopedia, April 2018; Puéchal, "Relapsing polychondritis", Joint Bone Spine, March 2014, p. 118–24; Puéchal, "Relapsing polychondritis", Joint Bone Spine, March 2014, p. 118–24; Cantarini, "Diagnosis and classification of relapsing polychondritis.", Journal of Autoimmunity, 2014, p. 53–9; Puéchal, "Relapsing polychondritis", Joint Bone Spine, March 2014, p. 118–24; Puéchal, "Relapsing polychondritis", Joint Bone Spine, March 2014, p. 118–24 (original not read by us) en.wikipedia.org
  6. Wikipedia, "Relapsing polychondritis" (CC BY-SA 4.0), which cites: Jaksch Von Wartenhorst, "Polychondropathia", Wiener Archiv für Innere Medizin, 1923, p. 93–100; "Relapsing polychondritis", Rheumatic Disease Clinics of North America, May 2013, p. 263–76 (original not read by us) en.wikipedia.org
  7. Wikipedia, "Relapsing polychondritis" (CC BY-SA 4.0), which cites: "Relapsing polychondritis", Rheumatic Disease Clinics of North America, May 2013, p. 263–76; "Atrophic polychondritis with the report of a case", Annals of the Rheumatic Diseases, June 1961, p. 189–93 (original not read by us) en.wikipedia.org
  8. Wikipedia, "Relapsing polychondritis" (CC BY-SA 4.0), which cites: Puéchal, "Relapsing polychondritis", Joint Bone Spine, March 2014, p. 118–24; Puéchal, "Relapsing polychondritis", Joint Bone Spine, March 2014, p. 118–24; Puéchal, "Relapsing polychondritis", Joint Bone Spine, March 2014, p. 118–24; Puéchal, "Relapsing polychondritis", Joint Bone Spine, March 2014, p. 118–24 (original not read by us) en.wikipedia.org
  9. Wikipedia, "Relapsing polychondritis" (CC BY-SA 4.0), which cites: Cantarini, "Diagnosis and classification of relapsing polychondritis.", Journal of Autoimmunity, 2014, p. 53–9; McAdam, "Relapsing polychondritis: prospective study of 23 patients and a review of the literature.", Medicine, May 1976, p. 193–215; Cantarini, "Diagnosis and classification of relapsing polychondritis.", Journal of Autoimmunity, 2014, p. 53–9; Damiani, "Relapsing polychondritis--report of ten cases.", The Laryngoscope, June 1979, p. 929–46; Michet CJ, "Relapsing polychondritis. Survival and predictive role of early en.wikipedia.org
  10. Wikipedia, "Relapsing polychondritis" (CC BY-SA 4.0), which cites: "Relapsing polychondritis", Clin. Dermatol., 2006, p. 482–5 (original not read by us) en.wikipedia.org

Each numbered sentence above was checked against the source it cites before publication. Sentences citing Wikipedia adapt text from the English Wikipedia article Relapsing polychondritis (contributors), used under CC BY-SA 4.0; this entry is available under the same licence. Other sources were used for facts only; no text or images are copied from them.

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