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Weekly Chest CasesArchive of Old Cases

Case No : 818 Date 2013-07-01

  • Courtesy of Sang-Woo Shim, Woon-Jung Kwon / Ulsan University Hostpital
  • Age/Sex 48 / M
  • Chief Complaintdyspnea for 3 months
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
  • Figure 7

Diagnosis With Brief Discussion

Diagnosis
Pulmonary Alveolar Proteinosis
Radiologic Findings
Chest PA radiograph shows ill-defined increased opacities in the right middle lung zone and the both lower lung zones.
Chest CT axial images(lung window setting) show ill-defined ground glass opacities with crazy-paving appearance and minimal traction bronchiectasis along the both lung central subpleural areas.
Fig.1. Photomicrograph of the right upper lobe lung tissue with H & E stain shows the accumulation of pinkish granular eosinophilic material(asterisk) within the alveoli. (H & E, 횞200)
Fig. 2. Immunohistochemistry; PAS(+), Congo-red (-)

Fig 1

Fig 2

Brief Review
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by abnormal intraalveolar accumulation of surfactant-like material. Three distinct subgroups of PAP are currently recognized: idiopathic, secondary, and congenital.
Patients with idiopathic or secondary PAP experience nonspecific, moderate respiratory symptoms including progressive dyspnea and dry or minimally productive cough. Less common signs and symptoms include fatigue, weight loss, low-grade fever, chest pain, and hemoptysis.
Classic radiographic finding is a helpful first step in diagnostic imaging but remains nonspecific for PAP. The typical radiograph reveals bilateral central and symmetric lung opacities, with relative sparing of the apices and costophrenic angles.
The finding of CT is an appearance of 'crazy-paving' that defined as a network of smoothly thickened reticular lines superimposed on areas of ground-glass opacity. Areas of crazy-paving in PAP are typically widespread and bilateral, often with sharply marginated areas of geographic or lobular sparing. There are widely variable patterns of regional or zonal predominance, including symmetric or asymmetric apical, basilar, central, peripheral, lobar, or diffuse lung involvement. Although the CT finding of crazy-paving is highly characteristic of PAP, it is also seen in several infectious, hemorrhagic, neoplastic, inhalational, and idiopathic conditions as well as in straightforward hydrostatic pulmonary edema. Therefore, the radiologic differential diagnosis of crazy-paving is broad and includes left heart failure, pneumonia (especially pneumocystis pneumonia), alveolar hemorrhage, bronchoalveolar carcinoma, lymphangitic carcinomatosis, diffuse alveolar damage (adult respiratory distress syndrome), radiation- or drug-induced pneumonitis, hypersensitivity pneumonitis, and pulmonary veno-occlusive disease.
References
1. Fraser and Pare's Diagnosis of Diseases of the Chest혻. 2000-2007
2. Aletta Ann Frazier, MD, Teri J. Franks, MD. From the Archives of the AFIP Pulmonary Alveolar Proteinosis. RadioGraphics 2008; 28:883-899
Please refer to
Case 692 Case 670 Case 624 Case 525 Case 505
Keywords
Lung, Metabolic and storage lung disesae,

No. of Applicants : 80

▶ Correct Answer : 46/80,  57.5%
  • - , Korea (South) Sungkwan Kim
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - Chonnam National University Hospital , Korea (South) Jung hyun Kim
  • - Kyung Hee University Hospital at Gangdong , Korea (South) Seong Jong Yun
  • - Kilpauk medical college , India abirami mahadevan
  • - Asan Medical Center , Korea (South) Sanghyun Choi
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Korea university anam hospital , Korea (South) Sung-Hye You
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - Tong young Red cross hospital , Korea (South) Lee Jin
  • - Konkuk University Medical Center , Korea (South) Inyoung Song
  • - sunder lal jain hospital , India sanjeev vachher
  • - SNUH , Korea (South) Eui Jin Hwang
  • - stanley medical college , India mahes waran
  • - , Korea (South) Chan Park
  • - Shiga University of Medical Science , Japan Akitoshi Inoue
  • - SKG radiology , Australia Yuranga Weerakkody
  • - Kobe City Medical Center General Hospital , Japan Yasuhisa Kurata
  • - UWO , Canada S Lee
  • - Onomichi Municipal Hospital , Japan Yoshihisa Masaoka
  • - Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
  • - Asan medical center , Korea (South) Hyun Joo Lee
  • - NDMVP Nashik , India Imran Jindani
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - kasturba medical college , India mugil varman
  • - Kyoto University , Japan Akihiko Sakata
  • - Dongtan Sacred Heart Hospital , Korea (South) Younghwan Ko
  • - CBNUH , Korea (South) jihyung lee
  • - chungbuk national university hospital , Korea (South) soojung lee
  • - Chungbuk University Hospital , Korea (South) Yunhee Jang
  • - kyungpook national university hospital , Korea (South) Hoseok Lee
  • - Kyungpook national universtiy hospital , Korea (South) Hong Ji hoon
  • - Kyungpook national university hospital , Korea (South) Choi Sunju
  • - Kizawa Memorial Hospital , Japan Yo Kaneko
  • - izmir hospital , Turkey bakhtiar bakhshi
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - GHICL , France manuel toledano
  • - Ishikawa Matto Central Hospital , Japan Manabu Akimoto
  • - NASA SCANS , India RAKESH BHATIA
  • - CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
  • - CAC Rennes , France nicolas gautier
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
  • - CHU Poitiers , France CHAN paul
  • - Eren T캇p Merkezi Istanbul , Turkey Armagan Sarac
  • - Kyungpook National University Hospital , Korea (South) Jaekwang Lim
  • - 異⑸ , Korea (South) sooncheul baek
▶ Correct Answer as Differential Diagnosis : 22/80,  27.5%
  • - Nagasaki University Hospital, Department of Pathology , Japan TOMONORI TANAKA
  • - radiologist, aditya imaging centre , India vivek patel
  • - MBAL Chernomorska , Bulgaria VLADISLAV RUSINOV
  • - ICD,MCH CALICUT , India rikhy krishnan
  • - James Paget Hospital , U.K , United Kingdom nabil mahmood
  • - University of British Columbia , Canada Amr Ajlan
  • - Seoul National University Hospital , Korea (South) YongSub Song
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OOKUBO
  • - sahyadri speciality hospital , India kothareddy dileepreddy
  • - Dongnam Institute of Radiological and Medical Sciences , Korea (South) Dae-Wook Yeh
  • - EWHA WOMANS MOKDONG UNIVERSITY HOSPITAL , Korea (South) Sang Min Lee
  • - McGill university , Canada Badriya Al-Qassabi
  • - Northern Yokohama Showa university , Japan Kota Watanabe
  • - Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
  • - Seoul medical center , Korea (South) SungTaek Kim
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - King fahed hospital in hofuf , Saudi Arabia jaafer Al-obaid
  • - Private sector , Greece Vasilios Tzilas
  • - All India Institute of medical sciences , India Justin Moses
  • - All India Institute of Medical Sciences , India Ashish Gupta
  • - NIMS, HYDERABAD , India bhaskar kakarla
  • - Medicheck health care , Korea (South) Chae Lim
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