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Weekly Chest CasesArchive of Old Cases

Case No : 949 Date 2016-01-05

  • Courtesy of Dong Jin Im, Hye-Jeong Lee / Severance Hospital
  • Age/Sex 45 / M
  • Chief ComplaintCough for 6 months
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Pleuroparenchymal fibroelastosis
Radiologic Findings
Chest PA shows bilateral apical pleural thickening and subpleural opacities.
Chest CT images show bilateral subpleural irregular opacities, pleural thickening and traction bronchiolectasis in both upper lobes. In the lower lobes there was no pleural irregularity, but there was a subtle traction bronchiectasis.
VATS biopsy of the right upper lobe demonstrated fibroelstosis involving the pleura and subpleural lung parenchyma consistent with pleuroparenchymal fibroelastosis.
Brief Review
Pleuroparenchymal fibroelastosis (PPFE) include the category of rare idiopathic interstitial pneumonia with idiopathic lymphoid interstitial pneumonia. PPFE is a rare condition that consists of fibrosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobes. HRCT shows dense subpleural consolidation with traction bronchiectasis, architectural distortion, and upper lobe volume loss. Pleural fibrosis is associated with subjacent intra-alveolar lung parenchymal fibrosis and alveolar septal elastosis. Most common presentation is in adults with a median age of 57 years with no sex predilection. Approximately half of patients have history of recurrent infections. Pneumothorax may complicate the course of the disease and even recur. A minority may have familial interstitial lung disease and nonspecific autoantibodies. Histological biopsies sometime show mild changes of PPFE with UIP and NSIP. Disease process is progressive in 60% of patients and may be fatal 40%.

Special Note from Archana Laroia, Associate Professor of Iowa University Hospital, English copyeditor of weekly chest cases- I think the authors may want to consider including that bone marrow or stem cell transplantation and lung transplantation are now known to cause PPFE, as a lung manifestation of chronic graft versus host disease (GVHD) and chronic rejection of transplanted lungs.
References
Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013;188:733-748
Keywords
Lung, Idiopathic interstitial pneumonia, IIP,

No. of Applicants : 92

▶ Correct Answer : 51/92,  55.4%
  • - Daejin Medical Center Bundang Jesaeng General Hospital , Korea (South) Donghwan Kim
  • - ROKAF 10th Fighter Wing , Korea (South) Kun Woo Kang
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Juntendo University Hospital , Japan Akifumi Hagiwara
  • - Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki
  • - stanley medical college , India yazhini v
  • - Kyoto University , Japan Akihiko Sakata
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Soonchunhyang University Seoul Hospital , Korea (South) Boda Nam
  • - Asan medical center , Korea (South) Sang Young Oh
  • - Fortis hospital , Mohali , India Shaleen Rana
  • - CHRU Lille , France Paul Lebert
  • - Union hospital,Wuhan , China Nan Diao
  • - Armed Forces Seoul Hospital , Korea (South) Eui Jin Hwang
  • - University of Tsukuba Hospital , Japan Sodai Hoshiai
  • - Saitama-Sekishinkai Hosptal , Japan Mihoko Yamazaki
  • - All India Institute of Medical Sciences , India Ashish Gupta
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OOKUBO
  • - Ewha Womans University Hospital , Korea (South) YOOKYUNG KIM
  • - IRSA La Rochelle , France Denis Chabassiere
  • - The University of Tokyo Hospital , Japan Toshihiro Furuta
  • - Seoul National University Hospital , Korea (South) Jong Hyuk Lee
  • - Osaka Rosai Hospital , Japan Hiromitsu Sumikawa
  • - Ewha Womans University , Korea (South) Sung Shine Shim
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - Niigata City General Hospital , Japan Takao Kiguchi
  • - Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
  • - the first affiliatited hospital of nanjing medical univercity , China Hai Xu
  • - Asan medical center , Korea (South) Cherry Kim
  • - National Center hospital of Neurology and Psychiatry , Japan Kaoru Sumida
  • - Asan medical center , Korea (South) Jaewook Lee
  • - Korea University Ansan Hospital , Korea (South) Ki Lee
  • - CNUH , Korea (South) Noh Hoon
  • - University of Tsukuba, Dept of Radiologt , Japan Manabu Minami
  • - Asan Medical Center , Korea (South) Jooae Choe
  • - Kizawa Memorial Hospital , Japan Yo Kaneko
  • - Gangnam Severance Hospital , Korea (South) Chul Hwan Park
  • - Nihon University Itabashi Hospital , Japan Yusuke Watanabe
  • - Niigata University , Japan Atsushi Uehara
  • - Ishikawa Matto Central Hospital , Japan Manabu Akimoto
  • - Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani
  • - Northern Yokohama Showa university , Japan Kota Watanabe
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Shinmatsudo Central General Hospital , Japan Taku Tajima
  • - Asan Medical Center , Korea (South) , Korea (South) Han Na Lee
  • - Centre Imagerie de Fribourg , Switzerland Benoit RIZK
  • - Ibaraki-gazou-shindan , Japan Shoichi Katoh
  • - The University of Tokyo Hospital , Japan Takeyuki Watadani
  • - Private sector , Greece Vasilios Tzilas
  • - University of Tsukuba , Japan Toshitaka Ishiguro
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
▶ Correct Answer as Differential Diagnosis : 8/92,  8.7%
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - University of British Columbia , Canada Amr Ajlan
  • - Otsu Red Cross Hospital , Japan Hirotsugu Nakai
  • - NIMS, HYDERABAD , India bhaskar kakarla
  • - XMU affiliated No.1 Hospital , China Qing Qiang Guo
  • - Asan , Korea (South) Sania ALBlushi
  • - Gifu Central Hospital , Japan Haruo Watanabe
  • - Okayama University Hospital , Japan Toshiyuki Komaki
▶ Semi-Correct Answer : 2/92,  2.2%
  • - Kinki University Faculty of Medicine, , Japan Mitsuru Matsuki
  • - Shiga University of Medical Science , Japan Akitoshi Inoue
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Current Editor : Sung Shine Shim, MD, PhD. Email : sinisim@ewha.ac.kr

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