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 used the term polychondropathia to describe his first case about relapsing chondritis.
- In 1960, the term relapsing polychondritis was first coined by Pearson.
Classification
- There is no established 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.
- The HLA class II has been associated with the development of relapsing polychondritis, there is increase susceptibility with HLA-DR4.[1]
- On gross pathology, thickening of cartilage of ear, nose, epiglottis, and cricoid and tracheal rings are characteristic findings of relapsing polychondritis.
- On microscopic histopathological analysis of relapsing polychondritis, following features are seen:
- Lymphocytes, plasma cells, neutrophils, and eosinophils are seen in the cartilage.
- Cartilage has lost the chondrocytes and normal architecture at the later stages of inflammation.
- Cartilage is replaced by fibrous tissue in the end.
Differentiating Relapsing Polychondritis from other Diseases
- Chondritis
- Chondromdermatitis
- Hansen's disease
- Nodularis helicis
- Perichondritis
- Rhinophyma
- Skin cancer
- Syphilis
Epidemiology and Demographics
- The incidence of relapsing polychondritis is approximately 4 per 100,000 individuals in Minnesota.
Age
- Relapsing polychondritis is more commonly observed among patients aged 40 to 60 years but it can also occur in childhood.[2][3]
Gender
- Relapsing polychondritis affects men and women equally.
Race
- Relapsing polychondritis usually affects Caucasians.
Risk Factors
- Common risk factors in the development of relapsing polychondritis include:
- Genetic susceptibility:
- Increased risk with HLA DR4
- Other connective tissue disorders
- Genetic susceptibility:
Natural History, Complications and Prognosis
- The majority of patients with relapsing polychondritis remain asymptomatic for [duration/years].
- Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
- If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
- Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
- Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].
Diagnosis
Diagnostic Criteria
| Mc Adam diagnostic criteria for relapsing polychondritis[4] | |
|---|---|
| Criteria | Requirement |
| Recurrent chondritis of both auricles | 3 out of 6 features
are required for definitive diagnosis of relapsing polychondritis |
| Non-erosive inflammatory polyarthritis | |
| Nasal chondritis | |
| Inflammation of auricular structures | |
| Chondritis of respiratory tract | |
| Cochlear and/or vestibular damage | |
Symptoms
- Ear pain
- Eye pain, redness, and watery eyes
- Decreased visual acuity
- Proptosis
- Rhinorrhea
- Epistaxis
- Pain on the movement of joint
- Joint swelling
- Cough
- Shortness of breath
- Dysphagia
- Chest pain
- Headache
- Confusion
Physical Examination
- Patients with relapsing polychondritis usually appear fatigued.
- Physical examination may be remarkable for:
- Ear examination:
- On inspection, there is inflammation and swelling
- On palpation, there is tenderness
- Eye examination:
- On inspection, lid retraction, redness of the eye, ptosis, and proptosis
- Visual acuity is decreased
- Lung examination:
- On auscultation, wheezing and expiratory stridor is found
- Heart examination:
- S1, S2 is normal, the murmur can be heard depending on the involvement of valve
- Joint examination:
- On inspection, there is redness and swelling
- On palpation, there is tenderness
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
- Urinary collagen type II neoepitope levels is elevated in active inflammation[12]
- Levels of urinary collagen type II neoepitope is used to assess response to treatment[13]
- Serum levels of cartilage oligomeric matrix protein is elevated and used as a marker of disease activity[14]
- CBC with differentials
Imaging Findings
- X-ray of the chest shows tracheal stenosis.
- CT scan of the chest shows following findings:[15]
- Subglottic stenosis
- Thickening of anterior and lateral tracheal wall with sparing of the posterior membranous wall
Source: Case courtesy of Dr Charlie Chia-Tsong Hsu[16][17]
- MRI is better than CT scanning to see the difference between edema, fibrosis, and inflammation
Other Diagnostic Studies
Treatment
Medical Therapy
- The mainstay of therapy for relapsing polychondritis is medical therapy.
- NSAIDs are used as an initial treatment of relapsing polychondritis.
- Preferred regimen: Naproxen 500 mg PO q12h or ibuprofen 800 mg PO q6h x 7 to 10 days.
- If NSAIDs are contraindicated or resistant then following regimens are used:
- Preferred regimen: Dapsone 50 to 100 mg PO q24h x 4 months depending on the severity of the disease.
- Preferred regimen: Prednisone 30 to 60 mg PO q24h in divided dose.
- Treatment of relapse:
- Preferred regimen: Prednisone 5 to 7.5 mg PO q24h
- Treatment of life-threatening organ damage:
- Preferred regimen: Initial therapy is the combination of prednisone 1 mg/kg PO q24h and cyclophosphamide 2 mg/kg PO q24h.
- Maintenance therapy depends on the renal function of a patient.
- Patient without nephritis, Preferred regimen: Methotrexate 15mg PO once weekly.
- Patient with nephritis, Preferred regimen: Azathioprine
Surgery
- Medical therapy is the mainstay of therapy for relapsing polychondritis, however various surgical options include:
- Tracheostomy
- Cardiac valve replacement
- Aortic aneurysm repair
- Saddle-nose deformity repair
Prevention
- There are no primary preventive measures available for [disease name].
- Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
- Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
References
- ↑ 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.
- ↑ 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.
- ↑ McAdam LP, O'Hanlan MA, Bluestone R, Pearson CM (May 1976). "Relapsing polychondritis: prospective study of 23 patients and a review of the literature". Medicine (Baltimore). 55 (3): 193–215. PMID 775252.
- ↑ Coppola M, Yealy DM (January 1992). "Relapsing polychondritis: an unusual cause of painful auricular swelling". Ann Emerg Med. 21 (1): 81–5. PMID 1539895.
- ↑ Kent PD, Michet CJ, Luthra HS (January 2004). "Relapsing polychondritis". Curr Opin Rheumatol. 16 (1): 56–61. PMID 14673390.
- ↑ O'Hanlan M, McAdam LP, Bluestone R, Pearson CM (1976). "The arthropathy of relapsing polychrondritis". Arthritis Rheum. 19 (2): 191–4. PMID 1259793.
- ↑ Balsa A, Expinosa A, Cuesta M, MacLeod TI, Gijón-Baños J, Maddison PJ (1995). "Joint symptoms in relapsing polychondritis". Clin. Exp. Rheumatol. 13 (4): 425–30. PMID 7586772.
- ↑ 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)
- ↑ 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.
- ↑ Lee KS, Ernst A, Trentham DE, Lunn W, Feller-Kopman DJ, Boiselle PM (August 2006). "Relapsing polychondritis: prevalence of expiratory CT airway abnormalities". Radiology. 240 (2): 565–73. doi:10.1148/radiol.2401050562. PMID 16801364.
- ↑ href="https://radiopaedia.org/">Radiopaedia.org
- ↑ href="https://radiopaedia.org/cases/31793">rID: 31793
External links
- Polychondritis Educational Society, Ltd. (PES)
- Dr. D. E. Trentham research paper
- MedicineNet.com
- The Polychondritis Group - Support Group
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