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Weekly Chest CasesArchive of Old Cases

Case No : 670 Date 2010-08-30

  • Courtesy of Bo-Kyeong Kang, Hyun-Joo Lee, Eun Jin Chae / University of Ulsan College of Medicine, Asan Medical Center
  • Age/Sex 54 / F
  • Chief ComplaintMild subacute dyspnea
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Pulmonary alveolar proteinosis
Radiologic Findings
A 54-year-old female presented with abnormal findings on CT. The patient underwent annual physical examination and had no symptoms. On a chest radiograph, there were areas of diffuse ground-glass opacity in both lungs (Figure 1).
CT images show extensive, multifocal areas of ground-glass opacity with inter- and intralobular septal thickening and centrilobular nodules in both entire lungs (Figure 2-1 and 2).

Bronchoalveolar lavage was done at the anterior segment of the right upper lobe. There was turbid fluid material showing strong positive on PAS stain, consistent with pulmonary alveolar proteinosis.
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. PAP is attributed to the result of pathophysiologic mechanisms that impair pulmonary surfactant homeostasis and lung immune function. Clinical symptoms are variable, ranging from mild progressive dyspnea to respiratory failure.
The typical radiograph reveals bilateral, central and symmetric lung opacities, with relative sparing of the apices and costophrenic angles. The CT appearance of 밹razy-paving,� defined as a network of smoothly thickened septal 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. The radiologic differential diagnoses of crazy-paving include pulmonary edema, pneumonia, alveolar hemorrhage, diffuse alveolar damage, and lymphangitic carcinomatosis.
Definitive diagnosis is made with lung biopsy or bronchoalveolar lavage specimens that reveal intraalveolar deposits of proteinaceous material, dissolved cholesterol, and eosinophilic globules. Symptomatic treatment includes whole-lung lavage and multiple procedures may be required.
References
1. Frazier AA, Franks TF, Cooke EO, et al. Pulmonary Alveolar Proteinosis. RadioGraphics 2008; 28 :883�899.
Keywords
Lung, Interstitial lung disease, ILD,

No. of Applicants : 92

▶ Correct Answer : 33/92,  35.9%
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - radiologist, aditya imaging centre , India vivek patel
  • - NTUH , Taiwan Kuei-pin Chung
  • - PingTung Christian Hospital ,China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
  • - chp st martin , France Mariotte benoit
  • - Osaka University , Japan Osamu Honda
  • - Uzunkopru Devlet Hastanesi , Turkey Armagan Sarac
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - KUMC ansan , Korea (South) kihwan kim
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - Fukuyama daiichi Hospital , Japan Mototsugu Saeki
  • - Youngdong Hospital , Korea (South) Sang Min Lee
  • - NEPEAN HOSPITAL , Australia YOGESH THAKKAR
  • - McGill University , Canada Ben Smith
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - okayama university , Japan takeharu yamamoto
  • - SMG-SNU boramae medical center , Korea (South) Kwang Nam Jin
  • - Hangang sacred heart hospital , Korea (South) You Mie Han
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - Saga University , Japan Ryoko Egashira
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - bmcri , India sanjeev choudri
  • - jobless , France jean-baptiste Noel
  • - Chonnam National University Hwasoon Hospiral , Korea (South) You Ri Choi
  • - University of British Columbia , Canada Amr Ajlan
  • - Shinchon Severance Hospital , Korea (South) Ho-Joon Lee
  • - Seoul Nationial University Bundang Hospital , Korea (South) Hee Seok Choi
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
  • - Hotel-Dieu Montreal , Canada nicolas gautier
  • - Mackay Memorial Hospital , Taiwan Sheng-Yeh Shen
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - PRIVATE PRACTITIONER , India BALA SWAMY
▶ Correct Answer as Differential Diagnosis : 16/92,  17.4%
  • - KMC,Manipal , India satish maddukuri
  • - Chonnam university hospital , Korea (South) Minyoung Jung
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Doctors Hospital , Bahamas muneesh sharma
  • - Osaka University , Japan Hiromitsu Sumikawa
  • - Virgin Mary Hospital Burgas , Bulgaria VLADISLAV RUSINOV
  • - Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
  • - CHU brabois , France francois jausset
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - Trakya University School of Medicine , Turkey Umut Ugur
  • - CNUH , Korea (South) Hyedoo Jung
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OOKUBO
  • - Hospital M�e de Deus , Brazil Thiago Bento da Silva
  • - Shree Diagnostics , India amol jagdale
  • - kovai medical centre , India k bhaskar
  • - NMC hospital Dubai , United Arab Emirates vickrant malhotra
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