대한흉부영상의학회 Weekly Chest Cases

Case No : 1005 Date 2017-01-30

Courtesy of Hae Jin Kim, MD. Tae Jung Kim, MD. / Samsung Medical center
Age/Sex 53 / F
Chief Complaintcough and dyspnea for 5 years

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Diagnosis With Brief Discussion

Diagnosis: Pleuroparenchymal fibroelastosis
Radiologic Findings: Figs 1-2. Chest PA and lateral view show increased reticular opacities in both lungs and marked apical pleural thickening.
Fig 3-6. Lung window setting of the chest CT scan shows pleural and subpleural thickening with severe fibrotic changes in the marginal parenchyma with apicocaudal distribution.
Figs 7-9. Coronal reformatted images show marked apical pleural thickening, volume loss of the upper lungs, and peripheral consolidation. Note the evolution between 2014 and 2016 (at the time of diagnosis).
Brief Review: She underwent VATS biopsy, and pathologic findings revealed subpleural dense fibrosis with elastosis and patchy lymphocytic infiltration, suggesting pleuroparenchymal fibroelastosis (PPFE). Her sister was also diagnosed with the same disease.
PPFE is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS) statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs). PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes. Pathologically, interstitial fibrosis takes the form of a dense consolidation with some preservation of alveolar septal outlines and demonstrates a distinctly abrupt interface with residual normal lung. Unrecognized cases of PPFE may be incorrectly diagnosed as sarcoidosis, atypical idiopathic pulmonary fibrosis, or other unclassifiable interstitial pneumonias.The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation, chemotherapy, and recurrent respiratory infections. The patients usually report progressive dyspnea and dry cough; pBetter awareness of the disease with its clinical and radiologic features can help with optimal management by the multidisciplinary team. Clinical outcome in PPFE is variable with a significant number of patients demonstrating progressive decline and death. Survival characteristics depend on the stage of the disease at presentation. Aside from lung transplantation, there is no demonstrated effective treatment.
Radiological differential diagnoses may include chronic hypersensitivity pneumonitis, atypical usual interstitial pneumonitis, collagen vascular diseases, irradiation injury, asbestos exposure, prominent apical cap fibrosis, and sarcoidosis.
References: 1. Reddy TL, Tominaga M, Hansell DM, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur. Respir. J. 2012;40:377–385.
2. Frankel SK, Cool CD, Lynch DA, et al. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 2004;126:2007–2013.
3. Travis WD, Costabel U, Hansell DM 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 (6): 733-48.
Keywords: Lung, Pleura,

No. of Applicants : 127

▶ Correct Answer : 74/127, 58.3%

- ospedale bellaria , Italy giorgia dalpiaz
- Showa university Northern Yokohama Hospital , Japan KOTA WATANABE
- staten island univ hosp , United States JOSEPH LOWRY
- Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
- Yokohama-asahi-chuo-general hospital , Japan KYOKO NAGAI
- Union hospital,Wuhan , China Nan Diao
- Ulsan University Hospital , Korea (South) SOYEOUN LIM
- Shiga University of Medical Science , Japan AKITOSHI INOUE
- The University of Tokyo Hospital , Japan Akifumi Hagiwara
- Dongguk University ILsan Hospital , Korea (South) JUNG EUN LEE
- NIMS, Hyderabad , India AMIT DADHANIA
- King Abdulaziz University Hospital , Saudi Arabia Amr M. Ajlan
- , India SAMSON K
- CH de la Cote Basque , France PAUL ARDILOUZE
- , Korea (South) HANKYUL KIM
- Ajou university hospital , Korea (South) Pae Sun Suh
- Korea University Guro Hospital , Korea (South) PARK SOYEON
- The University of Tokyo Hospital , Japan Yusuke Watanabe
- Yeungnam University Medical Center , Korea (South) SUHONG KIM
- Korea University Guro Hospital , Korea (South) GAYOUNG CHOI
- Korea University Guro Hospital , Korea (South) SOO YOUNG CHAE
- Chungbuk National University Hospital , Korea (South) SOOJUNG LEE
- Centro Hospitalar S. Joao , Portugal Marcio Cunha Rodrigues
- , Korea (South) SU YEON AHN
- Myongji Hospital , Korea (South) KYUNGHO KIM
- Asan Medical Center , Korea (South) HYUN JUNG KOO
- , Oman FAIZA ALKINDI
- Soonchunhyang University Hospital Seoul , Korea (South) SUNG HWAN BAE
- Amagasaki General Medical Center , Japan GENKI FUKUMOTO
- privato , Italy VINCENZO ENZO GENCHI
- , Korea (South) KIM GO EUN
- The Jikei university , Japan TAKU GOMI
- university of montreal , Canada Andrei Bogdan Gorgos I
- The first affiliated Hospital of Xiamen University , China Qing Qiang Guo
- Onomichi municipal hospital , Japan Hirofumi Mifune
- tongji medical university affiliated jingzhou central hospital , China HUANG YUAN YI
- The University of Tokyo Hospital , Japan Toshihiro Furuta
- Chonnam National University Hospital , Korea (South) JONG HYEON KIM
- Seoul National University Bundang Hospital , Korea (South) SUNG HYUN YOON
- Chonnam National University Hospital , Korea (South) YOUNSU AHN
- Yeungnam University Medical Center , Korea (South) JONGSOO PARK
- Gifu University Hospital , Japan Yo Kaneko
- Bahcesehir University School of Medicine, Radiology Department , Turkey Mustafa Kemal Demir
- Dong-A University, College of Medicine , Korea (South) KI-NAM LEE
- Samsung medical center , Korea (South) Ran Kim
- Samsung Medical Center , Korea (South) KYOWON GU
- Dong-a University Medical Center , Korea (South) WON JIN CHOI
- Medicheck health care , Korea (South) Chae hun Lim
- University of Tsukuba Hospital , Japan Hiroaki Takahashi
- Mallinckrodt Institute of Radiology , United States Naganathan BS Mani
- CLINIQUE STE CLOTILDE , Reunion patrick serge MASCAREL
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
- University of Toronto , Canada MICHEAL MCINNIS
- Kyeongpook National University Hospital , Korea (South) SUNGJUN MOON
- Samsung Medical Center , Korea (South) BODA NAM
- , Korea (South) HYERIN KIM
- Azienda Ospedaliera di Cremona , Italy Pietro Sergio
- Seoul National University Hospital , Korea (South) KUM JU CHAE
- Kanto Rosai Hospital , Japan Taku Tajima
- , Korea (South) DONGRYUL CHANG
- Aerospace medical group, Air Force Education and Training Command , Korea (South) Donghwan Kim
- , Korea (South) JINWOO HAN
- Ajou University Hospital , Korea (South) SEULGI YOU
- Fortis hospital , Mohali , India SHALEEN RANA
- The University of Tokyo Hospital , Japan KAORU SUMIDA
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- University of Tsukuba , Japan Toshitaka Ishiguro
- Private sector , Greece VASILIOS TZILAS
- Hallym University Medical Center , Korea (South) HWANG HYE JEON
- University of Utah , United States AKIHIKO SAKATA
- Korea University Guro Hospital , Korea (South) HWANSEOK YONG
- Ewha Womans University Hospital , Korea (South) YOOKYUNG KIM
- Korea University Guro Hospital , Korea (South) Yoonmi Choi
- , Korea (South) CHERRY KIM

▶ Correct Answer as Differential Diagnosis : 3/127, 2.4%

- Hyogo Prefectural Amagasaki General Medical Center , Japan TOMOAKI OTANI
- Seoul St. Mary , Korea (South) Myeong Im Ahn
- Oita University, Faculty of Medicine , Japan Fumito Okada

▶ Semi-Correct Answer : 1/127, 0.8%

- Kizawa Memorial Hospital , Japan Tomohiro Ando

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1005 Date 2017-01-30

Discussion

No. of Applicants : 127