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Weekly Chest CasesArchive of Old Cases

Case No : 880 Date 2014-09-08

  • Courtesy of Kwang Nam Jin / Boramae Hospital
  • Age/Sex 66 / F
  • Chief Complaintnonproductive cough(3-4 months), radiologic abnormality(6 months)
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Pulmonary alveolar proteinosis
Radiologic Findings
Plain radiographs demonstrate progression of perihilar GGO and reticular opacities in both lungs for 6 months. Four years ago, there was no remarkable finding on plain radiograph.
CT images show symmetric ill-defined ground glass opacities and reticulation in the both lung central areas.

Percutaneous lung biopsy revealed pulmonary alveolar proteinosis.
Brief Review
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by abnormal intraalveolar accumulation of surfactant-like material. 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.
References
1.Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med 2003;349:2527-2539
2.Khan A, Agarwal R. Pulmonary alveolar proteinosis. Respir Care 2011;56:1016-1028
3.Rossi SE, Erasmus JJ, Volpacchio M, Franquet T, Castiglioni T, McAdams HP. "Crazy-paving" pattern at thin-section CT of the lungs: radiologic-pathologic overview. Radiographics 2003;23:1509-1519
Keywords
Lung, Metabolic and storage lung disesae,

No. of Applicants : 77

▶ Correct Answer : 40/77,  51.9%
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Shiga University of Medical Science , Japan Akitoshi Inoue
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Montreal , Canada Drago Le King
  • - Seoul National University Hospital , Korea (South) Sang Min Lee
  • - GHICL , France manuel toledano
  • - 異⑸ , Korea (South) Yunhee Jang
  • - Kyung Hee University Hospital at Gangdong , Korea (South) Seong Jong Yun
  • - Saket City Hospital , India Rajesh Gothi
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Ibaraki-gazou-shindan , Japan Shoichi Katoh
  • - The University of Tokyo Hospital , Japan Takeyuki Watadani
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Pamukkale University, School of Medicine, Dept. of Radiology , Turkey Nevzat Karabulut
  • - Kyoto University , Japan Akihiko Sakata
  • - Niigata City General Hospital , Japan Takao Kiguchi
  • - Asan medical center , Korea (South) Hyun Joo Lee
  • - Asan Medical Center , Korea (South) Hyung Jung Koo
  • - Kizawa Memorial Hospital , Japan Yo Kaneko
  • - National Hospital Organization Okinawa Hospital , Japan Yasuji Oshiro
  • - The University of Tokyo Hospital , Japan Toshihiro Furuta
  • - CHU Poitiers , France CHAN paul
  • - Scans world , India Philson Mukkada
  • - 異⑸ , China ZHANG YING
  • - Pulmonary Institute, Shaare Zedek Medical Center , Israel Abraham Bohadana
  • - Nagasaki University Hospital, Department of Pathology , Japan TOMONORI TANAKA
  • - Niigata University , Japan Atsushi Uehara
  • - Teikyo University Mizonokuchi Hospital , Japan Noriko Kobayashi
  • - CLCC Rennes , France nicolas gautier
  • - Onomichi Municipal Hospital , Japan Yoshihisa Masaoka
  • - Centre Imagerie de Fribourg , Switzerland Benoit Rizk
  • - Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki
  • - CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
  • - Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani
  • - Gifu Central Hospital , Japan Haruo Watanabe
  • - NASA SCANS , India RAKESH BHATIA
  • - Ishikawa Matto Central Hospital , Japan Manabu Akimoto
  • - Medicheck health care , Korea (South) Chae Lim
  • - Bupyong Serim Hospital , Korea (South) Hee Seok Choi
  • - Private sector , Greece Vasilios Tzilas
▶ Correct Answer as Differential Diagnosis : 17/77,  22.1%
  • - MBAL BURGAS , Bulgaria VLADISLAV RUSINOV
  • - NIMS, HYDERABAD , India bhaskar kakarla
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - Royal Perth Hospital , Australia Yuranga Weerakkody
  • - Ondokuz Mayis University , Turkey Cetin Celenk
  • - DAYA General Hospital,Thrissur,Kerala , India Raveendran TK
  • - Asan Medical Center , Korea (South) Jooae Choe
  • - Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
  • - chungbuk national university hospital , Korea (South) soojung lee
  • - Chungbuk univertisy hospital , Korea (South) Soohyun Lee
  • - National Center hospital of Neurology and Psychiatry , Japan Kaoru Sumida
  • - IRSA La Rochelle , France Denis Chabassiere
  • - Fortis hospital , Mohali , India Shaleen Rana
  • - IRSA LA ROCHELLE , France, Metropolitan BIGOT
  • - KUH , Korea (South) TaeHyung Kim
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - chp st martin , France Mariotte benoit
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