Relapsing polychondritis
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
- In 1923, Jaksch-Wartenhorst had used the term polychondropathia to describe his first case about relapsing chondritis.
- In 1960, the term relapsing polychondritis was first coined by Pearson.[1]
Classification
- There is no classification of relapsing polychondritis.
Pathophysiology
- The pathogenesis of relapsing polychondritis is unknown.
- Several studies have suggested the role of both cellular immunity and abnormal autoantibody in the pathogenesis of relapsing polychondritis.[2]
- The HLA class II has been associated with the development of relapsing polychondritis, there is increase susceptibility with HLA-DR4.[3]
- On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
- On microscopic histopathological analysis of relapsing polychondritis, following features are seen:
- Lymphocytes, plasma cells, neutrophils, and eosinophils are seen in the cartilage.
- Cartilage lost the chondrocytes and is replaced by fibrous tissue.
Causes
Reasons for disease onset are not known.
It is considered to possibly be an auto-immune disease[4] 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.[5][6]
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
| Diagnostic criteria for Relapsing ploychondritis | ||
|---|---|---|
| Author | Criteria | Requirement |
| Recurrent chondritis of both auricles | ||
| Non-erosive inflammatory polyarthritis | ||
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
- Urinary glycosaminoglycans levels may be elevated[10]
- Urinary collagen type II neoepitope levels is elevated in active inflammation.[10]
- Levels of urinary collagen type II neoepitope is used to assess response to treatment.[11]
- Serum levels of cartilage oligomeric matrix protein is elevated and used as a marker of disease activity.[12]
- CBC with differentials
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
- ↑ PEARSON CM, KLINE HM, NEWCOMER VD (July 1960). "Relapsing polychondritis". N. Engl. J. Med. 263: 51–8. doi:10.1056/NEJM196007142630201. PMID 14431246.
- ↑ Buckner JH, Van Landeghen M, Kwok WW, Tsarknaridis L (January 2002). "Identification of type II collagen peptide 261-273-specific T cell clones in a patient with relapsing polychondritis". Arthritis Rheum. 46 (1): 238–44. doi:10.1002/1529-0131(200201)46:1<238::AID-ART10030>3.0.CO;2-M. PMID 11817597.
- ↑ Lang B, Rothenfusser A, Lanchbury JS, Rauh G, Breedveld FC, Urlacher A, Albert ED, Peter HH, Melchers I (May 1993). "Susceptibility to relapsing polychondritis is associated with HLA-DR4". Arthritis Rheum. 36 (5): 660–4. PMID 8489544.
- ↑ "Relapsing Polychondritis: Autoimmune Disorders of Connective Tissue: Merck Manual Home Edition".
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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)
- ↑ 10.0 10.1 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.
- ↑ 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.
- ↑ 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.
External links
- Polychondritis Educational Society, Ltd. (PES)
- Dr. D. E. Trentham research paper
- MedicineNet.com
- The Polychondritis Group - Support Group
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