대한흉부영상의학회 Weekly Chest Cases

Case No : 1106 Date 2019-01-07

Courtesy of Eui Jin Hwang, MD., Hyungjin Kim, MD., Soon Ho Yoon, MD / Seoul National University Hospital
Age/Sex 30 / M
Chief ComplaintFever (up to 38 ℃), Cough, Hoarseness

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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
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,

No. of Applicants : 89

▶ Correct Answer : 35/89, 39.3%

- The University of Kyoto Hospital , Japan TOMOAKI OTANI
- Showa university Northern Yokohama Hospital , Japan KOTA WATANABE
- Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
- Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
- Higashi-Ohmi General Medical Center , Japan AKITOSHI INOUE
- King Abdulaziz University Hospital , Saudi Arabia Amr M. Ajlan
- MacKay Memorial Hospital , Taiwan YEN TING CHEN
- NIMS, HYDERABAD , India BHASKAR K
- Chonbuk National University Hospital , Korea (South) EUN HA JUNG
- Other , Korea (South) SEONGSU KANG
- Freelance resident in Reunion Island , Reunion Fabien HO
- Oita University, Faculty of Medicine , Japan Fumito Okada
- The Jikei university , Japan TAKU GOMI
- Onomichi municipal hospital , Japan Hirofumi Mifune
- Fujieda Municipal General Hospital , Japan HAYATO NOZAWA
- McGill University / University College London , Canada Ilan Azuelos
- HHS , Canada S LEE
- Gifu University Hospital , Japan Yo Kaneko
- Dong-A University, College of Medicine , Korea (South) KI-NAM LEE
- The University of Tokyo Hospital , Japan RYO KUROKAWA
- Chonbuk National University Hospital , Korea (South) YOUNGKWANG LEE
- National Taiwan University Hospital Hsin-Chu Branch , Taiwan LI-TA KENG
- , United States MAANSI P
- , Japan NAOMI YUASA
- Azienda Ospedaliera di Cremona , Italy Pietro Sergio
- Multimagem Diagn泥sticos , Brazil PEDRO PAULO TEIXEIRA E SILVA TORRES
- Teikyo University Hospital , Japan KAORU SUMIDA
- Kyoto Prefectural University of Medicine , Japan TADASHI TANAKA
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- Private sector , Greece VASILIOS TZILAS
- , Japan YUMI MAEHARA
- Hamamatsu University Hospital , Japan YUKI HAYASHI
- Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
- Japanese Red Cross Wakayama Medical Center , Japan AKIHIKO SAKATA
- Jiangsu province hospital , China WANGJIAN ZHA

▶ Correct Answer as Differential Diagnosis : 11/89, 12.4%

- ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
- , Japan MASAFUMI KAIUME
- The University of Tokyo Hospital , Japan Akifumi Hagiwara
- Lille , France BENOIST CAPON
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
- Mallinckrodt Institute of Radiology , United States Naganathan BS Mani
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
- Ajou University Hospital , Korea (South) HYERIN KIM
- Kizawa Memorial Hospital , Japan Shoji Okuda
- , Korea (South) JONGSUN LEE

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1106 Date 2019-01-07

Discussion

No. of Applicants : 89