대한흉부영상의학회 Weekly Chest Cases

Case No : 766 Date 2012-07-02

Courtesy of Young-Hoon Cho, Eun-Young Kim, MI Young Kin / Asan mediacl center
Age/Sex 54 / M
Chief ComplaintChronic soreness of the throat, hoarseness, and recent dyspnea.

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Figure 1

Diagnosis With Brief Discussion

Diagnosis: Relapsing polychondritis
Radiologic Findings: The patient’s axial CT scans show diffuse and smooth thickening of the tracheal wall and bilateral main stem bronchi with multiple calcification. However, there is sparing of the posterior membranous portion of the trachea, where cartilage is not present. Additionally, cricoids cartilage show similar findings with multifocal calcification. On coronal scan, marked calcification involving the bilateral costal cartilages is noted.
The diagnosis of relapsing polychondritis was made based on the patient’s clinical symptoms and laboratory findings. Methotrexate and corticosteroid therapy were performed.
Brief Review: Relapsing polychondritis is a rare, autoimmune syndrome characterized by recurrent episodes of cartilaginous inflammation with subsequent degeneration, loss of structure, and fibrosis. It commonly involves cartilaginous structures of the external ear, nose, peripheral joints, larynx, and tracheobronchial tree. Peak prevalence occurs between 40 and 60 years of age, and there is no reported gender preference. Auricular chondritis is the most common initial presentation. Although respiratory manifestation is reported to be about 20% initially, up to 50% eventually present with air-way symptoms with disease progression. Airway involvement is associated with poor prognosis and is leading cause of death in affected patients.

At radiography, diffuse or localized airway involvement may be seen. The larynx and upper trachea are affected most frequently, but the disease may involve airways to the subsegmental level. Progressive cartilage calcification and destruction of integrity is commonly reported imaging feature of relapsing polychondritis. Airway wall also show increased thickness, seen in about 80% of patients. Wall thickening are typically smooth and diffuse. Fixed airway narrowing and/or obstruction can be seen in some cases. One most important imaging finding to remember is that above findings spare to posterior membranous portion of the airway, because there is normally no cartilaginous tissue present. When costal cartilages are involved, similar finding of cartilage destruction with progressive calcification can be noted on simple radiograph or CT images.

No single medical or surgical treatment is uniformly effective in curing the disease, relieving the symptoms, or preventing progression of airway manifestations. The mainstray of treatment remains medical management with corticosteroids.
References: 1. Ernst A, Sung A, Michaud G, et al. Relapsing polychondritis and airway involvement. Chest 2009;135:1024-1030.
2. Prince J, Duhamel D, Levin D, et al. Nonneoplastic lesions of the tracheobronchial wall: radiographic findings with bronchoscopic correlation. Radiographics 2002;22:215-230.
3. Behar J, Choi YW, Hartman T, et al. Relapsing polychondritis affecting the lower respiratory tract. AJR 2002;178:173-177.
Keywords: trachea, non-infectious inflammation, inflammation,

No. of Applicants : 100

▶ Correct Answer : 62/100, 62.0%

- Kosin University , Korea (South) Woonha Lee
- Kyung Hee Universitiy Hospital at Gangdong , Korea (South) Seong Jong Yun
- HUEC , Brazil Diogo Pinheiro
- Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
- CHRU Lille , France Paul Lebert
- CHU Poitiers , France CHAN paul
- GM InHospital , Korea (South) Ju Won Lee
- University of British Columbia , Canada Amr Ajlan
- Oita University, Faculty of Medicine , Japan Fumito Okada
- Ishikawa Matto Central Hospital , Japan Manabu Akimoto
- Kurashiki Seijin-byo Center , Japan Akihiro Tada
- Okayama University , Japan Takashi Tanaka
- Universidad del Desarrollo- Clinica Alemana , Chile Patricio Maximiliano Latorre Brajovic
- Onomichi municipal hospital , Japan Hirofumi Mifune
- aarthi scans , India salome rajesh
- Kyunghee Medical Center , Korea (South) Jungeun Lee
- CHRU lille , France manuel toledano
- Kyunghee university medical center at Kangdong , Korea (South) Soyoun Shin
- Korea Uivercity Medical Center , Korea (South) Euddeum Shim
- Dong-A University Hospital , Korea (South) Ji-yeon Han
- McGill University Health Center , Canada Alexandre Semionov
- Dongnam Institute of Radiological and Medical Sciences , Korea (South) Dae-Wook Yeh
- clinique de SAVOIE , France, Metropolitan gay-depassier philippe
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
- Kohka Public Hospital , Japan Akitoshi Inoue
- CNUH , Korea (South) Kim Soohyun
- , Korea (South) Kim mo
- Chonnam university hospital , Korea (South) Park young
- XiangYa hospital , China Xia Yu
- Seoul National University Hospital , Korea (South) YongSub Song
- Kyungpook National University Hospital , Korea (South) Jaekwang Lim
- SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OOKUBO
- China Medical University , Chia-Yi Christian Hospital ,Taiwan, R.O.C. , Taiwan Jun Jun Yeh
- Korea University Guro Hospital , Korea (South) Kyungwon Doo
- Montreal , Canada Drago Le King
- CAC Rennes , France nicolas gautier
- SMC , Korea (South) Yi Kyung Kim
- Seosan Medical Center , Korea (South) Sang Min Lee
- SNUH , Korea (South) Euijin Hwang
- Sunchun university hospital, Bucheon , Korea (South) So Young Bae
- Korea university , Korea (South) Kun Woo Kang
- Hanyang University Hospital , Korea (South) Yo Won Choi
- Kizawa Memorial Hospital , Japan Yo Kaneko
- Ondokuz Mayis University , Turkey Cetin Celenk
- CMC , Korea (South) Soh Jeong
- chungbuk national university hospital , Korea (South) soojung lee
- KHMC , Korea (South) Park So hyun
- mmcri , India sangeetha srinivasan
- Yongdong Severance Hospital , Korea (South) Chul Hwan Park
- Centre Hospitalier Lyon Sud , France matthieu ROUSSET
- IRSA La Rochelle France , France Denis Chabassiere
- Fortis hospital , Mohali , India Shaleen Rana
- Samsung Medical Center , Korea (South) Jihyun LEE
- Dr.Rajendra Prasad Government Medical College - Tanda, (Himachal Pradesh) , India Narvir Chauhan
- Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
- Private sector , Greece Vasilios Tzilas
- Radnet Teleradiology Istanbul , Turkey Armagan Sarac
- NDMVP Nashik , India Imran Jindani
- All India Institute of medical sciences , India Justin Moses
- Onomichi Municipal Hospital , Japan Yoshihisa Masaoka
- Chonnam national university hospital , Korea (South) Jang-hyeon Song
- Bupyong Serim Hospital , Korea (South) Hee Seok Choi

▶ Correct Answer as Differential Diagnosis : 16/100, 16.0%

- James Paget Hospital , U.K , United Kingdom nabil mahmood
- VGHKS , Taiwan Fu-Zong Wu
- Vitalife Clinics , Pune , India. , India Rahul Deshmukh
- Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
- IRSA , France, Metropolitan BIGOT
- UWO , Canada S Lee
- , Korea (South) Chan Park
- Chonnam National University Hospital , Korea (South) So yeon Ki
- Chonnam National University Hospital , Korea (South) Jung hyun Kim
- Goa Medical College , India Paresh Desai
- All India Institute of Medical Sciences , India Jitesh Ahuja
- Himeji St.Mary Hospital , Japan Yuichiro Kanie
- IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
- Mallinckrodt Institute of Radiology , United States Naganathan Mani
- UPHS , United States NK Gupta
- jaslok hospital & research centre mumbai , India JAINENDRA JAIN

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 766 Date 2012-07-02

Discussion

No. of Applicants : 100