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Weekly Chest CasesArchive of Old Cases

Case No : 432 Date 2006-02-06

  • Courtesy of Ki Nam Kim, MD, Ki Nam Lee, MD. / Dong-A University, Busan, Korea
  • Age/Sex 32 / M
  • Chief ComplaintCough for three months and multiple joint pain He was treated with steroid two years ago for autoimmune uveitis.
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Diagnosis With Brief Discussion

Diagnosis
Relapsing Polychondritis
Radiologic Findings
Chest CT images show diffuse, smooth wall thickening and subtle increased attenuation of the trachea, both main, and lobar bronchi, predominantly involving anterolateral cartilaginous portions. Luminal diameter of airway is relatively preserved. He had had a saddle nose with deficient septal cartilage, and auricular chondritis for 5years. The diagnosis is based on characteristic combinations of clinical features since the pathology was not obtainable. He had no septal cartilage due to destruction.
Brief Review

     Relapsing polychondritis (RP) is a rare multisystem autoimmune disease of unknown origin characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues. Elastic cartilage of the ears and nose, hyaline cartilage of peripheral joints, vertebral fibrocartilage and tracheobronchial cartilage, as well as proteoglycan-rich structures of the eye, heart, blood vessels or inner ear may all be affected. In most patients RP manifests in a fluctuating but progressive course which eventually results in a significant shortening of life expectancy.

     About 20% of patients have respiratory symptoms at presentation, and eventually a third to a half of the patients will develop respiratory tract involvement manifested by laryngeal tenderness, hoarseness, dyspnea, and stridor or wheeze.

     Airway involvement causes narrowing, primarily of the larynx or trachea, but the major bronchi can also be involved. Stenoses are much less common and usually single and localized, but they can be multiple. The most common CT manifestations were increased attenuation and smooth airway wall thickening that notably spared the posterior membranous portion of the airway. Lobar air trapping and airway collapse at end expiration were seen in half of patients, likely due to loss of cartilaginous support of airway walls.

 

Diagnostic Criteria

Clinical Features

The presence of three or more of the given clinical features

 


                 by McAdam et al (1976)

  • Chondritis or larynx, trachea, or bronchi
  • Chondritis of nasal cartilages
  • Chondritis of both auricles
  • Audiovestibular damage
  • Ocular inflammation
  • Nonerosive seronegative inflammatory polyarthritis

The presence of any one of the given combinations

 


                by Damiani and Levine (1979)

  • Three or more McAdams signs (histologic confirmation not necessary)
  • One or more of McAdams signs with positive histologic confirmation at biopsy of the cartilage
  • Involvement of two or more separate anatomic locations with response to steroids and/or dapsone
References
1. Hansell DM, Armstrong P, Lynch DA, McAdams HP. Imaging of diseases of the chest. 4th. Elsevier Mosby,

2005:589-590

2. Behar JV, Choi YW, Hartman TA, Allen NB, McAdams HP. Relapsing polychondritis affecting the lower

respiratory tract. AJR 2002;178:173-177

3. Sarodia BD, Dasgupta A, Mehta AC. Management of airway manifestations of relapsing polychondritis: case

reports and review of literature. Chest 1999;116:1669-1675
Keywords
Airway, Multiple organ, Non-infectious inflammation, Relapsing Polychondritis ,

No. of Applicants : 49

▶ Correct Answer : 25/49,  51.0%
  • - Inje University Ilsan Paik Hospital Bae Geun Oh
  • - Jinju Korea Hospital, Korea Ji Hoon Park
  • - Jikei University School of Medicine, Tokyo, Japan Masuo Ujita
  • - Max Hospital, New delhi, India Anurag Jain
  • - China Medical University Hospital Sea-Harn Pan
  • - Dongguk University International Hospital, Korea Hee Seok Choi
  • - Samsung Medical Center, Seoul, Korea Ha Young Kim
  • - Hospital of the Unversity of Pennsylvania, USA Lisa Jones
  • - China Medical University Hospital, Taiwan Hsin-Yi Lai
  • - Daegu Fatima Hospital, Korea Sang-Hee Cho
  • - Shinchon Severance Hospital, Seoul, Korea Jae Seung Seo
  • - Hopital Calmette, CHRU, Lille, France Toledano Manuel
  • - Chikuhou Rousai Hospital, Japan Kouei Uchida
  • - Maimonides Medical Center; Brooklyn, New York, USA Naomi Twersky
  • - Hangang SacredHeart Hospital, Korea Eil Seong Lee
  • - Cabinet de Radiologie Guiton, La Rochelle, France Jean-Luc Bigot
  • - National Taiwan University Hospital, Taiwan Kao-Lang Liu
  • - Kangdong SacredHeart Hospital, Korea Kyoung Ja Lim
  • - ASL Bologna, Maggiore Hospital, Bologna, Italy Marcellino Burzi
  • - Homs National Hospital, Homs, Syria Rami Abou Zalaf
  • - Hospital HIS - Site Ixelles, Brussels, Belgium Emmanuel Agneessens
  • - Radiologie Guiton, La Rochelle, France Denis Chabassiere
  • - China Medical University Hospital,Taiwan Jun-Jun Yeh
  • - Severance Hospital, Korea Yong Eun Chung
  • - Incheon Sarang Hospital, Korea Jung Hee KIm
▶ Correct Answer as Differential Diagnosis : 6/49,  12.2%
  • - Konkuk University Hospital, Seoul, Korea Jeong Geun Yi
  • - Annemasse, Polyclinique de Savoie, France Gay-Depassier Philippe
  • - Ondokuz Mayis University, Samsun, Turkey Cetin Celenk
  • - Ultracare, Coimbatore, India Debabrata Das
  • - Shinchon Severance Hospital, Seoul, Korea Hye-Jeong Lee
  • - Social Security Hospital, Ankara, Turkey Meric Tuzun
▶ Semi-Correct Answer : 1/49,  2.0%
  • - Doma Hospital, Damascus, Syria Mostafa Dakak
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Current Editor : Sung Shine Shim, MD, PhD. Email : sinisim@ewha.ac.kr

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