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Weekly Chest CasesArchive of Old Cases

Case No : 1033 Date 2017-08-14

  • Courtesy of Juyeon Lee and Woocheol Kwon / Wonju Severance Christian Hospital, Yonsei University Wonju College of Medicine
  • Age/Sex 48 / M
  • Chief ComplaintDyspnea on exertion for 1 month, aggravated 1 week ago, construction worker, current smoker (30PY)
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Pulmonary alveolar proteinosis
Radiologic Findings
Plain X-ray shows bilateral, symmetric ground glass opacities (GGO) with central distribution, sparing the apices and costophrenic angles. Associated interstitial thickening was also noted.
CT shows widespread bilateral geographic GGO with superimposed interlobular septal thickening, demonstrating a crazy paving pattern. No pleural effusion or enlarged mediastinal lymph nodes seen.
Whole lung bronchoalveolar lavage (BAL) was done bilaterally. The BAL fluid milky and opaque initially and becomes clear gradually.
Brief Review
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by abnormal intraalveolar accumulation of surfactant-like material that is positive at periodic acid-Schiff staining and rich in lipid. Inflammatory response in the adjacent interstitium is seen.
It is usually presents in young and middle-aged adults (20-50 years of age) and smoking is strongly associated with the condition. Three distinct subgroups of PAP are currently recognized: idiopathic, secondary, and congenital. Secondary PAP (5%-10% of cases) is recognized in patients with industrial inhalational exposure to materials such as silica particles, cement dust or etc.
The typical radiograph reveals bilateral central and symmetric lung opacities, with relative sparing of the apices and costophrenic angles (bat-wing pattern). Less commonly, radiographs show multifocal asymmetric opacities or extensive diffuse consolidation. There is a disparity between the moderate clinical symptoms and the more impressive radiographic abnormalities (clinicoradiologic discrepancy).
Crazy paving pattern, defined scattered or diffuse ground-glass attenuation with superimposed interlobular septal thickening and intralobular lines in a geographic distribution is a characteristic feature of this disease visible on high-resolution computed tomography (CT). But other etiologies such as pneumonia (especially pneumocystis pneumonia), alveolar hemorrhage, bronchoalveolar carcinoma, lymphangitic carcinomatosis, ARDS also shows crazy paving pattern.
Whole-lung lavage is the most widely accepted therapy. Post therapeutic BAL CT may reveal persistent septal lines despite interval resolution of ground-glass opacity.
References
1. FRAZIER, Aletta Ann, et al. Pulmonary alveolar proteinosis. Radiographics, 2008, 28.3: 883-899.
2. ROSSI, Santiago E., et al. Crazy-paving pattern at thin-section CT of the lungs: radiologic-pathologic overview. Radiographics, 2003, 23.6: 1509-1519.
Please refer to
Case 883 Case 880 Case 843 Case 818 Case 692 Case 670 Case 624 Case 525 Case 404 Case 275 Case 198
Keywords
Pulmonary Alveolar Proteinosis, Lung, PAP,

No. of Applicants : 116

▶ Correct Answer : 69/116,  59.5%
  • - Toranomon Hospital , Japan SHOHEI FUJITA
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Hyogo Prefectural Amagasaki General Medical Center , Japan TOMOAKI OTANI
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Niigata University , Japan ATSUSHI UEHARA
  • - Assistant Prof. , United States AMIT GUPTA
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - SQUH , Oman BADRIYA SALEH AL-QASSABI
  • - Gifu University Hospital , Japan TOMOHIRO ANDO
  • - McGill University Health Center , Canada Alexandre Semionov
  • - NIMS, HYDERABAD , India BHASKAR K
  • - IRSA LA ROCHELLE , France, Metropolitan BIGOT
  • - The University of Tokyo Hospital , Japan Yusuke Watanabe
  • - Ondokuz Mayis University , Turkey CETIN CELENK
  • - the First Affiliated Hospital of NanJing Medical University , China AIPING CHEN
  • - Complexo Hospitalar Santa Casa - Porto Alegre, Brazil , Brazil Carlos Eduardo Anselmi
  • - Korea University Guro Hospital , Korea (South) GAYOUNG CHOI
  • - Seoul National University Hospital , Korea (South) JAE WON CHOI
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONG SU KANG
  • - Seoul National University Hospital , Korea (South) Ju Gang Nam
  • - Asan Medical Center , Korea (South) HYUN JUNG KOO
  • - Soonchunhyang University Hospital Seoul , Korea (South) SUNG HWAN BAE
  • - Samsung Medical Center , Korea (South) JUHYUN KIM
  • - The first affiliated Hospital of Xiamen University , China Qing Qiang Guo
  • - Qatif Central Hospital , Saudi Arabia HANI MAKKY ALSALAM
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Yonsei University, Wonju Severance Christian Hospital , Korea (South) JUYEON LEE
  • - Chonnam National University Hospital , Korea (South) JONG HYEON KIM
  • - HHS , Canada S LEE
  • - Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
  • - Chonnam National University Hospital , Korea (South) YOUNSU AHN
  • - Asan Medical Center , Korea (South) JOOAE CHOE
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Samsung Medical Center , Korea (South) MIN YEONG KIM
  • - Dong-A University, College of Medicine , Korea (South) KI-NAM LEE
  • - , United Kingdom KRISHNA PRASAD PREMNATH BELLAM
  • - Samsung Medical Center , Korea (South) KYOWON GU
  • - National Taiwan University Hospital , Taiwan LI-TA KENG
  • - 怨„紐… , Korea (South) jin young kim
  • - University of Tsukuba Hospital , Japan HIROAKI TAKAHASHI
  • - MAGNUM DIAGNOSTICS - Goa INDIA , India PARESH K DESAI
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Other , Korea (South) HAYEON LEE
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - University of Illinois at Chicago , United States GYE YOUNG PARK
  • - , Viet Nam PHAM THAI PHI
  • - GHICL , France MANUEL TOLEDANO
  • - Wuhan Union Hospital , China QIGUANG CHENG
  • - The University of Tokyo Hospital , Japan TAKU TAJIMA
  • - Max Hospital New Delhi , India RAJESH GOTHI
  • - , India RAVI SHEKHAR
  • - Escola Paulista de Medicina , Brazil Renato Masson de Almeida Prado
  • - District TB centre, kasaragod,India , India rikhy krishnan
  • - MBAL BURGAS , Bulgaria VLADISLAV BOYANOV RUSINOV
  • - Vikram Hospital , India SANJEEV J MUDAKAVI
  • - Fortis hospital , Mohali , India SHALEEN RANA
  • - Ibaraki-gazou-shindan , Japan SHOICHI KATOH
  • - Kyoto Prefectural University of Medicine , Japan TADASHI TANAKA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
  • - Private sector , Greece VASILIOS TZILAS
  • - Orlando health , United States WAEL NASSER
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 25/116,  21.6%
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Higashi-Ohmi General Medical Center , Japan AKITOSHI INOUE
  • - Jilin university , Syria ABDO ABDO ABDO
  • - 罹…stanbul , Turkey AYHAN YILMAZ
  • - Amagasaki General Medical Center , Japan GENKI FUKUMOTO
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - Azienda Ospedaliera Universitaria Integrata di Verona , Italy GIAN ALBERTO SOARDI
  • - Korea University Guro Hospital , Korea (South) TAE SEOP CHOI
  • - Ajou University Hospital , Korea (South) Taeyang Ha
  • - Korea University Guro Hospital , Korea (South) YOUKYOUNG LEE
  • - Medicheck health care , Korea (South) Chae hun Lim
  • - Soonchunhyang University Hospital Seoul , Korea (South) BODA NAM
  • - , Korea (South) HYERIN KIM
  • - Azienda Ospedaliera di Cremona , Italy Pietro Sergio
  • - Multimagem Diagn泥˜sticos , Brazil PEDRO PAULO TEIXEIRA E SILVA TORRES
  • - Korea university medical center, guro hospital , Korea (South) Lee kyu chong
  • - Gifu University , Japan KEITA FUJIMOTO
  • - Ajou University Hospital , Korea (South) SEULGI YOU
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
  • - Korea University Guro Hospital , Korea (South) SUN HONG
  • - Teikyo University Hospital , Japan KAORU SUMIDA
  • - Seoul National University Hospital , Korea (South) YONGSUB SONG
  • - University of Utah , United States AKIHIKO SAKATA
  • - Korea University Guro Hospital , Korea (South) HWANSEOK YONG
▶ Semi-Correct Answer : 8/116,  6.9%
  • - King Abdulaziz University Hospital , Saudi Arabia Amr M. Ajlan
  • - , Korea (South) HANKYUL KIM
  • - speciality medical officer , India DIVYESHKUMAR RAMNIKLAL DADHANIA
  • - , United States JITESH AHUJA
  • - Laval university , Canada FARHAD ZARE
  • - Ajou University School of Medicine , Korea (South) YOOLIM BAEK
  • - Samsung medical center , Korea (South) Ran Kim
  • - Ewha Womans University Hospital , Korea (South) YOOKYUNG KIM
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