Relapsing polychondritis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ,Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]

Overview

Relapsing polychondritis is a condition where cartilage deteriorates.

It is also known as Chronic atrophic polychondritis, Meyenburg-Altherr-Uehlinger syndrome, von Meyenburg's disease, Generalized chondromalacia, or Systemic chondromalacia.

Historical Perspective

Classification

Polychondritis is one of many subclasses of disease in the area of Rheumatology.

Pathophysiology

  • The pathogenesis of relapsing polychondritis is characterized by [feature1], [feature2], and [feature3].
  • The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
  • On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Causes

Reasons for disease onset are not known.

It is considered to possibly be an auto-immune disease[1] in which the human's body's immune system begins to attack and destroy the cartilage tissues in the body.

Differentiating Relapsing Polychondritis from other Diseases

  • Cauliflower ear
  • Chondritis
  • Chondromdermatitis
  • Hansen's disease
  • Nodularis helicis
  • Perichondritis
  • Rhinophyma
  • Skin cancer
  • Syphilis

Epidemiology and Demographics

  • The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
  • In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Relapsing polychondritis is more commonly observed among patients of 40 and 60 years old but it can occur in childhood.[2][3]

Gender

  • Relapsing polychondritis affects men and women equally.

Race

  • There is no racial predilection for [disease name].
  • [Disease name] usually affects individuals of the [race 1] race.
  • [Race 2] individuals are less likely to develop [disease name].

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

Symptoms

  • Decreased joint mobility
  • Joint erythema
  • Joint inflammation
  • Joint swelling
  • Pain on movement of joint

Physical Examination

All cartilage areas can be affected, though in many cases the disease will affect several areas where cartilage is found in the body, and leave others entirely alone. Parts of the body with cartilage, and therefore potentially affected by polychondritis, include the ears, nose, throat, heart valves and of course all areas where musculo-skeletal tissues are connected by cartilage. Specific resultant conditions may include Type 3 Tracheomalacia and Vasculitis.

Laboratory Findings

  • There are no specific laboratory findings associated with relapsing polychondritis.
  • Abnormal laboratory findings consistent with the diagnosis of relapsing polychondritis include:
    • CBC with differentials
      • Low hemoglobin
      • Leukocytosis
      • Eosinophilia
    • ESR and CRP are usually raised
    • Antibody testing
      • Anti-type II collagen antibodies is usually raised in early phase of disease[4][5]
      • Antineutrophil cytoplasmic antibodies is found in patients who has vasculitis[6]
    • Urinary glycosaminoglycans levels may be elevated[7]
    • Urinary collagen type II neoepitope levels is elevated in active inflammation.[7]
      • Levels of urinary collagen type II neoepitope is used to assess response to treatment.[8]
    • Serum levels of cartilage oligomeric matrix protein is elevated and used as a marker of disease activity.[9]

Imaging Findings

Other Diagnostic Studies

Presentation

Symptoms

Treatment

Medical Therapy

Treatment plans typically involve suppression of the immune system with medicines, which often result in a side effect of increasing the risk of other infections.

Surgery

Prevention

References

  1. "Relapsing Polychondritis: Autoimmune Disorders of Connective Tissue: Merck Manual Home Edition".
  2. Knipp S, Bier H, Horneff G, Specker C, Schuster A, Schroten H, Lenard HG, Niehues T (2000). "Relapsing polychondritis in childhood--case report and short review". Rheumatol. Int. 19 (6): 231–4. PMID 11063294.
  3. Belot A, Duquesne A, Job-Deslandre C, Costedoat-Chalumeau N, Boudjemaa S, Wechsler B, Cochat P, Piette JC, Cimaz R (March 2010). "Pediatric-onset relapsing polychondritis: case series and systematic review". J. Pediatr. 156 (3): 484–9. doi:10.1016/j.jpeds.2009.09.045. PMID 19880136.
  4. Foidart JM, Abe S, Martin GR, Zizic TM, Barnett EV, Lawley TJ, Katz SI (November 1978). "Antibodies to type II collagen in relapsing polychondritis". N. Engl. J. Med. 299 (22): 1203–7. doi:10.1056/NEJM197811302992202. PMID 714080.
  5. Ebringer R, Rook G, Swana GT, Bottazzo GF, Doniach D (October 1981). "Autoantibodies to cartilage and type II collagen in relapsing polychondritis and other rheumatic diseases". Ann. Rheum. Dis. 40 (5): 473–9. PMC 1000784. PMID 7030234.
  6. Papo T, Piette JC, Le Thi Huong D, Godeau P, Meyer O, Kahn MF, Bourgeois P (May 1993). "Antineutrophil cytoplasmic antibodies in polychondritis". Ann. Rheum. Dis. 52 (5): 384–5. PMC 1005055. PMID 8323388. Vancouver style error: initials (help)
  7. 7.0 7.1 Passos CO, Onofre GR, Martins RC, Graff DL, Pagani EA, Sodré CT, Silva LC (July 2002). "Composition of urinary glycosaminoglycans in a patient with relapsing polychondritis". Clin. Biochem. 35 (5): 377–81. PMID 12270767.
  8. Kraus VB, Stabler T, Le ET, Saltarelli M, Allen NB (October 2003). "Urinary type II collagen neoepitope as an outcome measure for relapsing polychondritis". Arthritis Rheum. 48 (10): 2942–8. doi:10.1002/art.11281. PMID 14558101.
  9. Kempta Lekpa F, Piette JC, Bastuji-Garin S, Kraus VB, Stabler TV, Poole AR, Marini-Portugal A, Chevalier X (2010). "Serum cartilage oligomeric matrix protein (COMP) level is a marker of disease activity in relapsing polychondritis". Clin. Exp. Rheumatol. 28 (4): 553–5. PMID 20810035.

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