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Weekly Chest CasesArchive of Old Cases

Case No : 1246 Date 2021-09-07

  • Courtesy of Jong Hyuk Lee, Wonju Hong, Jongsoo Park, Eui Jin Hwang / Seoul National University Hospital
  • Age/Sex 70 / M
  • Chief ComplaintDyspnea, 3YA
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Tracheal amyloidosis
Radiologic Findings
Fig. 1. Chest radiograph showing diffuse narrowing of the trachea.
Figs. 2–4. Computed tomography scans showing diffuse and nodular thickening of the trachea with associated narrowing of the tracheal lumen. Bronchoscopy demonstrated diffuse tracheal and bronchial mucosal thickening. Bronchoscopic biopsy of the tracheal wall revealed chronic inflammation with apple-green birefringence on Congo red staining, suggesting tracheobronchial amyloidosis.
Bronchoscopy revealed diffuse mucosal thickening of the trachea with luminal narrowing.
Brief Review
Amyloidosis is a rare disorder of protein folding, and the amyloid deposits consist mainly of protein fibrils. The most important proteins are amyloid L and amyloid A. Amyloidosis affects the respiratory tract in approximately 50% of the cases. Amyloidosis can involve the lung parenchyma, trachea, bronchi, mediastinum, pleura, and heart. Histologic examination reveals amyloid deposits in subepithelial interstitial tissues, which show a characteristic apple-green birefringence when stained with Congo red and examined under polarizing microscopy.
Tracheobronchial amyloidosis typically manifests after the fifth decade of life, with dyspnea, cough, and occasionally hemoptysis or asthma-like symptoms.
Most of the patients show normal chest radiographic findings. However, focal or diffuse thickening of the airway with luminal narrowing can be seen. The findings of tracheobronchial amyloidosis on computed tomography include thickening of the airway wall with associated narrowing of the lumen and, in some cases, calcification foci. Wall thickening may be focal or diffuse and nodular, plaque-like, or circumferential. Tracheobronchial amyloidosis is generally confined to the trachea but can extend to the main, lobar, and segmental bronchi, and bronchial involvement is frequently associated with distal atelectasis, bronchiectasis, or air trapping. However, these imaging findings are nonspecific, and the differential diagnoses include a variety of diseases, such as relapsing polychondritis and tracheobronchopathia osteochondroplastica. Unlike these two disease entities, tracheobronchial amyloidosis can involve the noncartilaginous posterior wall.
References
1. Christopher W, Janathan C. Muller’s Imaging of the Chest 2nd edition: 538-542.
2. Czeyda-Pommersheim F, Hwang M, Chen SS, Strollo D, Fuhrman C, Bhalla S (2015) Amyloidosis: modern cross-sectional imaging. Radiographics 35:1381-1392.
Keywords

No. of Applicants : 82

▶ Correct Answer : 6/82,  7.3%
  • - Other , Korea (South) SEONGSU KANG
  • - , Korea (South) JIN YOUNG LEE
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - College of Medicine, CHA University , Korea (South) PARK JONG WON
  • - , Korea (South) WONJU HONG
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 44/82,  53.7%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan HIROAKI ARAKAWA
  • - , China XUFGENG ZHENG
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Showa General Hospital , Japan MASAFUMI KAIUME
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - Other , Korea (South) HYEJIN YANG
  • - , United Kingdom SAMSON KADE
  • - Other , Korea (South) MINGI SHIN
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - IRSA LA ROCHELLE , France JEAN LUC BIGOT
  • - , Italy PAOLO BALDASSARI
  • - Tiger Gate Hospital , Japan SHIN-ICHI CHO
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Osaka University , Japan AKINORI HATA
  • - Shimada General Medical Center , Japan HAYATO NOZAWA
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - Yeungnam University Medical Center , Korea (South) JONGSOO PARK
  • - Gifu University Hospital , Japan Yo Kaneko
  • - , Japan KANAE TAKAHASHI
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Other , Korea (South) SEUNGHYUN LIM
  • - Mallinckrodt Institute of Radiology , United States NAGANATHAN MANI
  • - Osaka City General Hospital , Japan SHU MATSUSHITA
  • - Chungbuk National University Hospital , Korea (South) MIHYEON PARK
  • - Other , Korea (South) MINSU KIM
  • - Seoul Veterans Hospital , Korea (South) SEONG WON JANG
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - Holy Family Hopsital , India RAJESH GOTHI
  • - Other , Korea (South) KYU-CHONG LEE
  • - Kyoto University Hospital , Japan SATOSHI IKEDA
  • - Paras hospital, Panchkula , India SHALEEN RANA
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Chonbuk National University Hospital , Korea (South) DONG HAN SHIN
  • - , Japan KAZUMA TERAUCHI
  • - National Center for Global Health and Medicine , Japan TOMOKI IMOKAWA
  • - Private sector , Greece VASILIOS TZILAS
  • - Chonnam National University Hospital , Korea (South) JINWANG PARK
  • - , Japan YUKI HAYASHI
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
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Current Editor : Eui Jin Hwang, M.D., Ph.D Email : weeklychestcases@gmail.com

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