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Weekly Chest CasesArchive of Old Cases

Case No : 900 Date 2015-01-26

  • Courtesy of Margaret Mbabazi MD , Chang Hyun Lee, MD, PhD / Seoul National University Hospital, Department of Radiology
  • Age/Sex 77 / F
  • Chief ComplaintDry mouth for several years
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Pulmonary Amyloidosis, nodular type
Radiologic Findings
Chest radiograph shows multiple bilateral calcified and non calcified lung nodules. In addition, there is extensive reticulation in both lungs.
Axial non-contrast chest CT scans show multiple nodules in both lungs and some of them demonstrated cavitary changes. Dense calcifications or ossification in some nodules are seen. A combination of multifocal bronchiectasis with wall thickening and cysts is also seen in the lungs.
Brief Review
Amyloidosis is a heterogeneous group of diseases characterized by extracellular deposition of insoluble fibrillar proteins. The two most common forms are light-chain (AL) and reactive (AA) amyloidosis. The spectrum of pulmonary amyloidosis includes laryngeal, tracheobronchial, parenchymal and mediastinal lymph node disease. Pulmonary amyloidosis secondary to Sjogren’s syndrome is rare but presents with pulmonary nodules. The nodules usually are concentrated in the lower lobes and are typically subpleural or peripheral (1). They vary in size and shape with calcification or ossification is seen in up to 50% of cases.


In rare instances, nodular amyloid deposits may be associated with cysts, but their relationship is unclear. Proposed mechanisms include air trapping and destruction of alveolar walls by amyloid deposition and subsequent ischemia (2).
Sjogren syndrome, amyloid deposition, and lymphocytic interstitial pneumonia can occur together, and the cysts may then be a manifestation of lymphocytic interstitial pneumonia.
Amyloidosis is diagnosed on the basis of apple-green birefringence under a polarized light microscope after staining with Congo red, and the presence of rigid, non-branching fibrils, 7.5–10 nm in diameter, on electron microscopy.
Please refer to
Case 277, Case 379, Case 608, Case 786,
References
1. Gillmore JD, Hawkins PN. Amyloidosis and the respiratory tract. Thorax 1999;54:444–51.
2. Ohdama S, Akagawa S, et al. Primary diffuse alveolar septal amyloidosis with multiple cysts and calcification. Eur Respir J 1996;9:1569–71.
Keywords
Lung, Metabolic and storage lung disesae,

No. of Applicants : 78

▶ Correct Answer : 50/78,  64.1%
  • - VHS medical center , Korea (South) Seong hee Jeon
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Otsu Red Cross Hospital , Japan Hirotsugu Nakai
  • - Kyoto University , Japan Akihiko Sakata
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Ewha Womans University Hospital , Korea (South) YOOKYUNG KIM
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - University of British Columbia , Canada Amr Ajlan
  • - Asan medical center , Korea (South) Sang Young Oh
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Seoul university hospital , Korea (South) Youngjin Ryu
  • - DAYA General Hospital,Thrissur,Kerala , India Raveendran TK
  • - James Paget Hospital , U.K , United Kingdom nabil mahmood
  • - The University of Tokyo Hospital , Japan Takeyuki Watadani
  • - Shiga University of Medical Science , Japan Akitoshi Inoue
  • - Avrasya Hospital , Turkey Murat Ulusoy
  • - Seoul National University Hospital , Korea (South) Sunkyung Jeon
  • - Ishikawa Matto Central Hospital , Japan Manabu Akimoto
  • - GHICL , France manuel toledano
  • - National Center hospital of Neurology and Psychiatry , Japan Kaoru Sumida
  • - Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki
  • - SNUH , Korea (South) Eui Jin Hwang
  • - Northern Yokohama Showa university , Japan Kota Watanabe
  • - Nagasaki University Hospital, Department of Pathology , Japan TOMONORI TANAKA
  • - The University of Tokyo Hospital , Japan Toshihiro Furuta
  • - CHU Poitiers , France CHAN paul
  • - Bahcesehir University School of Medicine, Radiology Department , Turkey Mustafa Kemal Demir
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - CHU POITIERS , France SZTARK Guillaume
  • - Niigata City General Hospital , Japan Takao Kiguchi
  • - Tong young Red cross hospital , Korea (South)
  • - Ibaraki-gazou-shindan , Japan Shoichi Katoh
  • - Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
  • - Hanyang universitiy, radiology department , Korea (South) HyunGyu Lee
  • - Niigata University , Japan Atsushi Uehara
  • - University of Tsukuba Hospital , Japan Sodai Hoshiai
  • - Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
  • - Kizawa Memorial Hospital , Japan Yo Kaneko
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Gifu Central Hospital , Japan Haruo Watanabe
  • - CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - IRSA La Rochelle , France Denis Chabassiere
  • - CHRU Lille , France Julien Pagniez
  • - CLCC Rennes , France nicolas gautier
  • - Asan Medical Center , Korea (South) Jooae Choe
  • - Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani
  • - Onomichi Municipal Hospital , Japan Yoshihisa Masaoka
  • - All India Institute of medical sciences , India Justin Moses
  • - Kobe City Medical Center General Hospital , Japan Yasuhisa Kurata
▶ Correct Answer as Differential Diagnosis : 7/78,  9.0%
  • - Pamukkale University, School of Medicine, Dept. of Radiology , Turkey Nevzat Karabulut
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - Royal Perth Hospital , Australia Yuranga Weerakkody
  • - CNUH , Korea (South) Noh Hoon
  • - Okayama University Hospital , Japan Toshiyuki Komaki
  • - IRSA LA ROCHELLE , France, Metropolitan BIGOT
  • - VHS medical center , Korea (South) Keuntak Roh
▶ Semi-Correct Answer : 12/78,  15.4%
  • - UZA , Belgium Eline De Smet
  • - �stanbul , Turkey Ayhan Yilmaz
  • - Pamukkale University, Faculty of Medicine, Dept. of Radiology , Turkey Furkan Ufuk
  • - Freelance resident in Reunion Island , Reunion Fabien HO
  • - DELTA CARE HOSPITAL, THANJAVUR, INDIA , India SIVARAJA SUBRAMANIAM
  • - Haeundae Paik Hospital , Korea (South)
  • - Chonnam National University Hospital , Korea (South) So yeon Ki
  • - Hopital Bichat , France, Metropolitan Pauline Pradere
  • - Isfahan university of medical science , Iran Elham Ghorbani
  • - Chonnam national university hospital , Korea (South) INSOO JANG
  • - Wuhan Union Hospital , China Qiguang Cheng
  • - Medicheck health care , Korea (South) Chae Lim
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Current Editor : Sung Shine Shim, MD, PhD. Email : sinisim@ewha.ac.kr

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