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Weekly Chest CasesArchive of Old Cases

Case No : 496 Date 2007-04-30

  • Courtesy of Yu Mi Jang, Eun Jin Chae, Kyung-Hyun Do, Joon Beom / Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
  • Age/Sex 48 / F
  • Chief ComplaintAbnormality on chest CT
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Pulomary Alveolar Proteinosis
Radiologic Findings
Chest radiograph (Fig. 1) shows ground glass opacity in bilateral CPA area.

HRCT scans (Fig. 2-5) show bilateral areas of ground glass opacity and thickening of the interlobular septa, showing a crazy paving appearance, which occur predominantly in the lower zones. There were no diagnostic abnormalities on BAL fluid. VATS biopsy was done. Histologically, alveoli area is filled with finely granular eosinophilic material that stains with periodic acid-Schiff.
Brief Review
In the reviews of the cytologic diagnosis of PAP, BAL fluid usually is grossly cloudy, with a predominance of amorphous granular extracellular material. This material is basophilic with Diff-Quik stain, usually eosinophilic with hematoxylin-eosin (H&E) stain, and strongly PAS-positive. It was previously recognized that BAL specimens dominated by granular and globular amorphous material with low numbers of macrophages and inflammatory cells are characteristic of PAP.

In most case, BAL alone is sufficient to diagnosis of PAP and actual lung tissue is not necessary except in problematic cases. In our case, BAL fluid analysis was not characteristic for PAP and VATS biopsy was done. Maygarden et al. report that two of the 8 PAP patients had atypical BAL cytologic findings and underwent open lung biopsy. One of these had BAL with numerous macrophages and scant proteinaceous extracellular globular material. The other patient had the characteristic granular and globular extracellular material in BAL fluid, with the addition of a moderate number of eosinophils.

Related cases: case 198, 404, symphosium 1998 6 and Imaging conference 2006 summer case 8.
Please refer to
Case 198, Case 404,
KSTR Symposium 1998  Case 6 ,
KSTR Imaging Conference 2006 Summer  Case 8,
References
1. Wang BM, Stern EJ, Schmidt RA, Pierson DJ. Diagnosing pulmonary alveolar proteinosis. A review and an update. Chest. 1997 Feb;111(2):460-6.

2. Maygarden SJ, Iacocca MV, Funkhouser WK, Novotny DB. Pulmonary alveolar proteinosis: a spectrum of cytologic, histochemical, and ultrastructural findings in bronchoalveolar lavage fluid.Diagn Cytopathol. 2001 Jun;24(6):389-95.
Keywords
Lung, Metabolic and storage lung disesae,

No. of Applicants : 56

▶ Correct Answer : 8/56,  14.3%
  • - Dept of radiology, Inje University Ilsan Paik Hospital, Korea Bae Geun Oh
  • - UHL NHS Trust, United kingdom, UK A. Donuru
  • - CHRU Lille hopital calmette, France Toledano Manuel
  • - Regional Imaging Riverina, Australia Rashid Hashmi
  • - CHU Besancon, France Sebastien AUBRY
  • - Kangnam sacred hospital, Korea Jae-won Kim
  • - ANNEMASSE, Polyclinique de SAVOIE, France Gay-Depassier Philippe
  • - Max Hospital, New Delhi, India Vickrant Malhotra
▶ Correct Answer as Differential Diagnosis : 12/56,  21.4%
  • - Dept. of Radiodiagnosis and Imaging, PGIMER, Chandigarh, India Ram Prakash Galwa
  • - Inha University Hospital, Korea Seung Woo Choi
  • - Korea Eun Ju Jeon
  • - Jikei University of Medicine, Japan Shigeki Misumi
  • - Hospital Sotiria, Athens, Greece Vasilios Tzilas
  • - Kyunghee University Medical Center, Korea Su Youn Sim
  • - IRSA La Rochelle, France Denis Chabassiere
  • - IRSA La Rochelle, France Jean-Luc BIGOT
  • - Doctors Hospital, Nassau, Bahamas N.B.S. Mani
  • - Yongsan Hospital, College of Medicine, Chung-Ang University, Korea Jae Seung Seo
  • - Aims, New Delhi, India Ashish Gupta
  • - China Medical University Hospital, Taiwan Jun-Jun Yeh
▶ Semi-Correct Answer : 22/56,  39.3%
  • - Hospital Sotiria Athens, Greece Delaveri keith
  • - Sctimst trivandrum, kerala, India Santhosh Kannath
  • - Ruby Hall Clinic, PUNE, India Nikhil Unune
  • - Balabhai Nanavati hospital, mumbai, India Susheel Kumar
  • - Yong Dong Severance Hospital, Yonsei University, Korea Yoo Jin Hong
  • - E-Da hospital, Taiwan Yu-Feng Wei
  • - Mackay Memorial Hospital, Taipei, Taiwan Chia-Fu Tsai
  • - KAUMS, Kashan, Iran Ebrahim Razi
  • - Changi General Hospital, Singapore Angeline Poh
  • - Yongdong Severance Hospital, Korea Park Chul Hwan
  • - Shin Chon Severance Hospital, Korea Ji Youn Kim
  • - Vital imaging centre, mumbai, India Ganesh Agrawal
  • - Ulsan University Hospital, Ulsan, Korea Jong-Chang Jang
  • - Osaka University, Japan Osamu Honda
  • - Aditya Imaging centre, Sayajigunj, Vadodara, Gujarat, India Vivek Patel
  • - Homs National Hospital, Homs - Syria Rami Abou Zalaf
  • - Hangang Sacred Heart Hospital, Korea Eil Seong Lee
  • - CHU NIMES, France BLONDEL Manuel
  • - EKH-Berlin, Germany Weber Michael
  • - CHU NIMES, France LARBI Amhed
  • - Nanavati Hospital, Mumbai, India Ajay Kumar
  • - Annecy hospital, France Gilles Genin
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