Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Relapsing polychondritis
- Radiologic Findings
- Fig 1. Chest PA shows normal bilateral lung fields. There is subtle diffuse narrowing of the trachea.
Fig 2-4. Contrast-enhanced chest CT images with mediastinal window setting show diffuse wall thickening of the trachea and proximal bronchi, relatively sparing the posterior membranous portion of the trachea.
Fig 5. F18-FDG PET-CT scan shows increased FDG uptake along the thickened cartilaginous portion of the trachea.
Fig 6 (For answer and discussion only). Patient’s nasal cartilages and right auricular cartilage also showed increased FDG uptake, suggesting multisystemic cartilaginous inflammation.
- Brief Review
- Relapsing polychondritis is a rare multisystem inflammatory disease characterized by repeated episodes of inflammation and progressive destruction of cartilages.
Respiratory involvement is seen at presentation of disease in about 10 % and during the course of the disease in about 50% of the patients. Laryngeal and tracheobronchial cartilages can be involved, causing hoarseness or dyspnea. Recurrent inflammation may result in permanent airway stenosis, which can cause respiratory failure. Typical image finding is smooth airway wall thickening with increased attenuation, sparing the posterior membranous portion of the large airways. Other findings include wall calcification, luminal narrowing, and dynamic collapse of the airways as well as lobar air trapping.
Other common manifestations of relapsing polychondritis include auricular chondritis, nasal chondritis, polyarthritis, and ocular inflammation, such as episcleritis and scleritis (60%).
There is no specific test for diagnosis of relapsing polychondritis. Instead, there several clinical diagnostic criteria as below:
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McAdam et al.
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Bilateral auricular chondritis
Nasal chondritis
Respiratory tract chondritis
Non-erosive sero-negative polyarthritis
Ocular inflammation
Audiovestibular damage
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3 out of 6 criteria
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Damiani et al.
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Bilateral auricular chondritis (A)
Nasal cartilage inflammation (A)
Respiratory tract condritis (A)
Non-erosive sero-negative polyarthritis (A)
Ocular inflammation (A)
Audiovestibular involvement (A)
Histological confirmation (B)
Positive response to corticosteroid or dapsone (C)
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3(A) criteria or 1(A) and 1(B) criteria or 2(A) and 1(C) criteria
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Michet et al.
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Auricular cartilage inflammation (A)
Nasal cartilage inflammation (A)
Laryngotracheal cartilage inflammation (A)
Ocular inflammation (B)
Hearing loss (B)
Vestibular dysfunction (B)
Sero-negative arthritis (B)
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2(A) criteria or 1(A) and 2(B) criteria
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For treatment, oral NSAIDs or corticosteroids may be used. In severe cases, immunosuppressants, such as azathioprine or methotrexate, can be used to minimize steroid doses.
- Please refer to
Case 432, Case 520, Case 766, -
- References
- 1. Behar JV, Choi YW, Hartman TA, Allen NB, McAdams HP. Relapsing polychondritis affecting the lower respiratory tract. AJR Am J Roentgenol. 2002;178(1):173-177.
2. Cantarini L, Vitale A, Brizi, MG et al. Diagnosis and classification of relapsing polychondritis. J Autoimmun. 2014;48–49:53–59.
3. Sharma A, Gananapandithan K, Sharma K, Sharma S. Relapsing polychondritis: a review. Clin Rheumatol. 2013;32(11)1575-1583.
4. McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore) 1976;55(3):193-215.
5. Damiani JM, Levine HL Relapsing polychondritis-report of ten cases. Laryngoscope 1979;89:929-946.
6. Michet CJ Jr, McKenna CH, Luthra HS, O’Fallon WM. Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med. 1986;104(1):74-78
- Keywords
- Airway, others,