Relapsing Polychondritis

Aka: Relapsing Polychondritis

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II. Epidemiology

  1. Incidence: Rare
  2. Age: Peaks age 40 to 50 years

III. Pathophysiology

  1. Not infectious
  2. Rare, multisystem, autoimmune, Collagen vascular disease
  3. Affects at least 2 cartilaginous sites

IV. Findings: Symptoms and signs

  1. Characteristics: Ear
    1. Cartilaginous Auricle is primary site of ear involvement
      1. Pain and swelling
      2. Inflammation and local destruction
    2. Ear lobule is spared
      1. Ear lobule is the non-cartilaginous, lower External Ear
  2. Characteristics: Other cartilaginous sites involved
    1. Nasal chondritis (often accompanies ear involvement)
    2. Migrating nonerosive Oligoarthritis
    3. Other tissues involved may include skin, eye, renal and neurologic
  3. Characteristics: Serious other cartilaginous involvement (associated with increased morbidity and mortality)
    1. Tracheobronchial tree
    2. Cardiac connective tissue
  4. Associated findings
    1. Sensorineural Hearing Loss may be associated
    2. Vertigo in 20% of cases
  5. Course
    1. Recurrent self-limited flares

V. Associated Conditions

  1. Other autoimmune disorders

VI. Differential Diagnosis

  1. Otitis Externa
  2. Ramsay Hunt Syndrome (Herpes Zoster Oticus)
  3. Auricular Perichondritis
  4. Squamous Cell Carcinoma of the Skin
  5. Granulomatosis with Polyangiitis
  6. Chondrodermatitis nodularis helicis
    1. Auricular helix with painful, swollen Nodule (which later ulcerates)
    2. Benign, skin and cartilage inflammation of the auricular helix
    3. Typically localizes to the patient's preferred sleeping side (ear down)

VII. Diagnosis

  1. Biopsy

VIII. Management

  1. Systemic Corticosteroids (and other Immunotherapy)
  2. Refer to Otolaryngology (ENT) or Rheumatology

IX. References

  1. Duran (2024) Am Fam Physician 110(5): 529-30 [PubMed]

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Ontology: Polychondritis, Relapsing (C0032453)

Definition (MSH) An acquired disease of unknown etiology, chronic course, and tendency to recur. It is characterized by inflammation and degeneration of cartilage and can result in deformities such as floppy ear and saddle nose. Loss of cartilage in the respiratory tract can lead to respiratory obstruction.
Concepts Disease or Syndrome (T047)
MSH D011081
ICD10 M94.1
SnomedCT 111256009, 85780006, 72275000
English Polychondritides, Relapsing, Relapsing Polychondritides, Relapsing Polychondritis, Polychondritis, Chronic Atrophic, Atrophic Polychondritides, Chronic, Atrophic Polychondritis, Chronic, Chronic Atrophic Polychondritides, Chronic Atrophic Polychondritis, Polychondritides, Chronic Atrophic, RELAPSING POLYCHONDRITIS, relapsing polychondritis (diagnosis), relapsing polychondritis, Polychondritis, Relapsing, Polychondritis, Relapsing [Disease/Finding], polychondritis relapsing, chronic polychondritis, Systemic chondromalacia (disorder), Relapsing polychondritis, Chronic polychondritis, Systemic chondromalacia, Meyenburg's disease, Relapsing polychondritis (disorder), polychondritis; recrudescent [Brill-Zinsser], recrudescent [Brill-Zinsser]; polychondritis, Systemic chondromalacia -RETIRED-, Chondromalacia, systemic, Chronic atrophic polychondritis
Spanish policondritis recidivante, policondritis recidivante (trastorno), Policondritis atrófica crónica, Policondritis recidivante, policondritis crónica, policondritis crónica (trastorno), condromalacia sistémica - RETIRADO -, condromalacia sistémica - RETIRADO - (concepto no activo), condromalacia sistémica, Policondritis Atrófica Crónica, Policondritis Recurrente
Dutch recidiverende polychondritis, chronische atrofische polychondritis, polychondritis; recidiverend, recidiverend; polychondritis, Polychondritis, chronische atrofische, Polychondritis, recidiverende, Recidiverende polychondritis
German chronische atrophische Polychondritis, rezivierende Polychondritis, Panchondritis [Rezidivierende Polychondritis], Polychondritis chronica atrophicans, Polychondritis, rezidivierende
Italian Policondrite atrofica cronica, Policondrite cronica atrofica, Policondrite recidivante
Portuguese Policondrite recorrente, Policondrite atrófica crónica, Policondrite Atrófica Crônica, Policondrite Recidivante
Swedish Återfallspolykondrit
Japanese ﾏﾝｾｲｲｼｭｸｾｲﾀﾊﾂｾｲﾅﾝｺﾂｴﾝ, ｻｲﾊﾂｾｲﾀﾊﾂｾｲﾅﾝｺﾂｴﾝ, 軟骨炎-再発性多発性, 再発性多発性軟骨炎, 慢性萎縮性多発性軟骨炎, 多発性軟骨炎-再発性, 軟骨炎-慢性萎縮性多発性
Finnish Uusiutuva monirustotulehdus
Russian POLIKHONDRIT RETSIDIVIRUIUSHCHII, ПОЛИХОНДРИТ РЕЦИДИВИРУЮЩИЙ
Czech Relabující polychondritida, Chronická atrofická polychondritida, relapsující polychondritida, recidivující polychondritida, relabující polychondritida, polychondritida recidivující
Korean 재발성 다발연골염
Polish Zapalenie chrząstek nawracające, Zapalenie wielochrząstne nawracające, Zapalenie chrząstek zanikowe
Hungarian Krónikus atrophiás polychondritis, Recurrens polychondritis
Norwegian Polychondritis chronica atrophicans, Residiverende polykondritt
French Polychondrite chronique atrophiante, Polychondrite récidivante, Polychondrite à rechute
Derived from the NIH UMLS (Unified Medical Language System)

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