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Weekly Chest CasesArchive of Old Cases

Case No : 1171 Date 2020-03-30

  • Courtesy of Jeongkyeom, Jun Ho Kim, Kyung Hee Lee / Inha University Hospital
  • Age/Sex 64 / M
  • Chief Complaintsputum
  • Figure 1
  • Figure 2
  • Figure 3
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  • Figure 5
  • Figure 6
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  • Figure 8
  • Figure 9
  • Figure 10

Diagnosis With Brief Discussion

Diagnosis
PPFE (pleuroparenchymal fibroelastosis)
Radiologic Findings
Non contrast enhanced chest CT scan shows pleural thickening in both upper thorax with subpleural fibrosis.
After about 4 years, non contrast enhanced chest CT scan shows progression of pleural thickening in both upper and extent to mid thorax with subpleural fibrosis and reticulation. And new findings are seen such as right fissural thickening, mild left fissural thickening, mild interlobular septal thickening in BLL.
Brief Review
Pleuroparenchymal fibroelastosis (PPFE) is an entity characterized by pleural and subpleural parenchymal fibrosis. It is extremely rare and was first described in 2004 by the Interstitial Lung Disease Program of the National Jewish Medical and Research Center of Denver.
Marked apical pleural thickening associated with superior hilar retraction is present at chest
X ray analysis, and High Resolution Computed Tomography (HRCT) shows pleural thickening, fibrosis, architectural distortion, traction bronchiectasis and honeycomb lung. The clinical course of this affection is progressive and prognosis is poor.

The etiology of PPFE is considered idiopathic in most cases, an association with a wide range of factors has been reported such as genetic predisposition

Chest radiographs in all cases showed marked apical pleural thickening, which was associated with superior hilar retraction.

HRCT images show dense pleural and subpleural consolidation with a reticular pattern, predominantly in the upper lobes. The upper lobes were always more severely involved, with involvement of the lower lobes being absent or less marked (apicocaudal distribution). Associated features included upper lobe volume loss, architectural distortion and traction bronchiectasis. One of the patients showed significant worsening relative to previous examinations. Two of the patients showed reticular opacities with some
areas of honeycombing and traction bronchiectasis. The other patient had co-existent cystic bronchiectasis. The patients underwent a TTB in the higher density
zones. All the patients were complicated with mild to moderate iatrogenic pneumothoraxes. Placement of a chest tube was needed in three patients. The pneumothorax resolved completely before discharge with a hospital stay ranging between 1 day and 17 days. One of the patients was also complicated with subcutaneous emphysema.

The therapeutic approach is highly variable in the published clinical cases with no clearly definitive therapeutic options other than lung transplantation. Disease progression occurs in 60 % of patients with death from disease in 40 %.

The differential diagnosis of PPFE includes asbestosis, connective tissue diseases, advanced fibrosing sarcoidosis and radiation or drug induced lung disease.
References
1. Sara Piciucchi, Sara Tomassetti, Gianluca Casoni, Nicola Sverzellati, Angelo Carloni, Alessandra Dubini, Giampaolo Gavelli, Alberto Cavazza, Marco Chilosi and Venerino Poletti, “High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis” Respir. Res. 2011;12 (1): 111
2. Cátia Esteves, Francisco R. Costa, Margarida T. Redondo, Conceição S. Moura, Susana Guimarães, António Morais, José M. Pereira “Pleuroparenchymal fibroelastosis: role of high-resolution computed tomography (HRCT) and CT-guided transthoracic core lung biopsy” Insights Imaging (2016) 7:155–162
3. Stephen K. Frankel, Carlyne D. Cool, David A. Lynch, and Kevin K. Brown. “Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity” Chest. 2004;126 (6): 2007-13
4. Taryn L. Reddy, Masaki Tominaga, David M. Hansell, Jan von der Thusen, Doris Rassl, Helen Parfrey, Suzy Guy, Orion Twentymane, Alexandra Rice, Toby M. Maher, Elisabetta A. Renzoni, Athol U.Wells and AndrewG.Nicholson. “Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes”. Eur Respir J 2012; 40: 377–385

Keywords
lung, idiopathic interstitial lung disease, pleuroparenchymal fibroelastosis,

No. of Applicants : 99

▶ Correct Answer : 81/99,  81.8%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Niigata University , Japan ATSUSHI UEHARA
  • - TWMU Medical Center East , Japan MASAFUMI KAIUME
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - Chonbuk National University Hospital , Korea (South) HYEJIN YANG
  • - , Korea (South) HYEYOUNG CHOI
  • - Chonnam National University Hwasun Hospital , Korea (South) INWOO CHOI
  • - CH de la Cote Basque , France PAUL ARDILOUZE
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Lille , France BENOIST CAPON
  • - Ondokuz Mayis University , Turkey CETIN CELENK
  • - Samsung Medical Center , Korea (South) CHOHEE KIM
  • - Chonnam National University Hospital , Korea (South) HONG SEOK CHOI
  • - Asan Medical Center , Korea (South) EUNJI CHOI
  • - Hospital de Braga , Portugal MARCIO RODRIGUES
  • - Asan Medical Center , Korea (South) WOOIL KIM
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - , Korea (South) DAJUNG KIM
  • - Scans world , India PHILSON JOSEPH MUKKADA
  • - DELTA CARE HOSPITAL, THANJAVUR, INDIA , India SIVARAJA SUBRAMANIAM
  • - Inje University Pusan Paik Hospital , Korea (South) DA SOM KIM
  • - Cabinet Capricorne, clinique sainte-clotilde , Reunion FABIEN HO
  • - Asan Medical Center , Korea (South) YURA AHN
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - The first affiliated Hospital of Xiamen University , China Qing Qiang Guo
  • - , Jordan HISHAM IBRAHIM KHATIB
  • - Hamamatsu University Hospital , Japan HAYATO NOZAWA
  • - Other , Korea (South) SANG HYUP HONG
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - Other , Korea (South) HYEON IL CHOI
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - , China MEI YUAN
  • - , Korea (South) PARK JONGMIN
  • - Yeungnam University Medical Center , Korea (South) JONGSOO PARK
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Chungbuk National University Hospital , Korea (South) JUNGHWAN KIM
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Chonbuk National University Hospital , Korea (South) YOUNGKWANG LEE
  • - Medicheck health care , Korea (South) Chae hun Lim
  • - Korea University Ansan Hospital , Korea (South) MOIN HA
  • - Soonchunhyang University Hospital Bucheon , Korea (South) SEONGHWAN BYUN
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Dae Jin Medical Center , Korea (South) JIYOUNG CHOI
  • - Hallym University Dongtan Medical Center , Korea (South) MINSU KIM
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - , Japan NAOMI YUASA
  • - Ajou University Hospital , Korea (South) HYERIN KIM
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - Asan Medical Center , Korea (South) JIHOON KIM
  • - Okayama Red Cross General Hospital , Japan SOICHIRO OKAMOTO
  • - Seoul Veterans Hospital , Korea (South) JANG SEONG WON
  • - , Taiwan NING CHIEN
  • - Columbia asia refferal hospital,Bengaluru , India PRAVIN KUMAR M
  • - Holy Family Hopsital , India RAJESH GOTHI
  • - Kyeongpook National University Hospital , Korea (South) PARK BYUNG GEON
  • - Escola Paulista de Medicina , Brazil RENATO MASSON DE ALMEIDA PRADO
  • - Kitano Hospital , Japan SATOSHI IKEDA
  • - Nishida Hospital , Japan SHOJI OKUDA
  • - University of Tsukuba Hospital , Japan SODAI HOSHIAI
  • - Other , Korea (South) JEONGHYUN LEE
  • - Chonbuk National University Hospital , Korea (South) MINJEE KIM
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Chonbuk National University Hospital , Korea (South) DONG HAN SHIN
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - National Center for Global Health and Medicine , Japan HIROSHI TAKUMIDA
  • - , Japan KAZUMA TERAUCHI
  • - Tokyo Metropolitan Bokutoh Hospital , Japan TOMOKI WADA
  • - Chonbuk National University Hospital , Korea (South) YOUNG JU SONG
  • - Private sector , Greece VASILIOS TZILAS
  • - , Japan YUMI MAEHARA
  • - College of Medicine, CHA University , Korea (South) PARK JONG WON
  • - Hamamatsu University Hospital , Japan YUKI HAYASHI
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - Chonnam National University Hwasun Hospital , Korea (South) WONGI JEONG
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - , Korea (South) HYUNGYU LEE
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 3/99,  3.0%
  • - St. Lukes International Hospital , Japan DAISUKE YAMADA
  • - Konkuk University Medical Center , Korea (South) JI HEE KANG
  • - Fortis hospital , Mohali , India SHALEEN RANA
▶ Semi-Correct Answer : 3/99,  3.0%
  • - IRSA LA ROCHELLE , France JEAN LUC BIGOT
  • - Seoul Veterans Hospital , Korea (South) JI HOON NOH
  • - , India SHYJUMON GEORGE
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Current Editor : Eui Jin Hwang, M.D., Ph.D Email : weeklychestcases@gmail.com

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