대한흉부영상의학회 Weekly Chest Cases

Case No : 843 Date 2013-12-23

Courtesy of Chul Hwan Park, Tae Hoon Kim / Gangnam Severance Hospital
Age/Sex 45 / M
Chief Complaintabnormal chest radiograph (Past history: S/P thyroidectomy for thyroid cancer

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initial chest PA

Diagnosis With Brief Discussion

Diagnosis: Pulmonary alveolar proteinosis
Radiologic Findings: Chest radiography showed ground glass opacity in subpleural area of right upper lung zone. Initial chest CT revealed well-defined multifocal patchy ground glass opacities with reticulation in subpleural areas of RUL, LUL, RML and RLL. Follow-up chest CT after 6 weeks revealed no significant interval change of multifocal patchy ground glass opacity lesions with reticulation.
Wedge biopsies were performed from RUL and RML. The specimens represented intra-alveolar collection of eosinophilic material with minimal lymphocytic infiltration. The pathologic diagnosis was pulmonary alveolar proteinosis.
Brief Review: Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by impaired surfactant metabolism and lipoproteinaceous material accumulation within alveoli (1, 2).
The prevalence of PAP has been reported as 3.7-6.2 cases per million (2). PAP is strongly related with smoking and usually occurs in young and middle-aged adults with male predominance (2, 3).
The characteristic finding of PAP is the filling of the alveolar spaces with fine granular eosinophilic and PAS-positive proteinaceous and lipid-rich surfactant-like material. Symptoms include dyspnea, non-productive cough, pleuritic chest pain, malaise and low grade fever (3). However, about one third of patients can be asymptomatic.
Chest radiograph usually shows bilateral, symmetric air-space consolidation or ground-glass opacity with a perihilar or basal distribution. High resolution CT typically reveals patchy ground-glass opacity with superimposed smooth inter-lobular / intra-lobular septal thickening (crazy paving) in polygonal shapes (1, 3). Although the distribution of PAP is usually predominant in perihilar / hilar area, PAP could affect the subpleural areas of the lung predominantly in some cases (4, 5).
The standard for the diagnosis of PAP is the lung biopsy. The bronchoalveolar larvage can be helpful in the diagnosis (2). The treatments of PAP are the whole lung lavage and correction of the underlying causes (secondary PAP) (2).
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
4.Inui N, Chida K, Suda T, Toyoshima M, Todate A, Ide K, et al. [A case of pulmonary alveolar proteinosis presenting with peripheral infiltrates]. Nihon Kokyuki Gakkai Zasshi 1999;37:333-336
5.Haga T, Kasamatsu N, Kobayashi T, Shibata M, Ogasawara T, Hashizume I. [A case of pulmonary alveolar proteinosis presenting with peripheral ground-glass opacitiy]. Nihon Kokyuki Gakkai Zasshi 2009;47:71-75
Please refer to: Case 496 Case 505 Case 525 Case 624 Case 670 Case 692 Case 818
Keywords: Lung, Metabolic and storage lung disesae,

No. of Applicants : 66

▶ Correct Answer : 30/66, 45.5%

- Tokyo Metropolitan Police Hospital , Japan Akifumi Hagiwara
- Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
- Niigata City General Hospital , Japan Takao Kiguchi
- Onomichi municipal hospital , Japan Hirofumi Mifune
- Kyoto University , Japan Akihiko Sakata
- Niigata University , Japan Atsushi Uehara
- district tuberculosis centre,poonamallee , India gayathri ganesh
- Saint Malo , France jean-baptiste Noel
- Ondokuz Mayis University , Turkey Cetin Celenk
- GHICL , France manuel toledano
- Asan medical center , Korea (South) Sang Young Oh
- Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
- Asan medical center , Korea (South) Hyun Joo Lee
- Kizawa Memorial Hospital , Japan Yo Kaneko
- CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
- Teikyo University Mizonokuchi Hospital , Japan Kaoru Sumida
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
- Northern Yokohama Showa university , Japan Kota Watanabe
- Kobe City Medical Center General Hospital , Japan Yasuhisa Kurata
- kanto Rosai Hp , Japan Takana Yamakawa
- IRSA La Rochelle , France Denis Chabassiere
- Shiga University of Medical Science , Japan Akitoshi Inoue
- Asan medical center , Korea (South) Kichang Han
- CAC Rennes , France nicolas gautier
- Ishikawa Matto Central Hospital , Japan Manabu Akimoto
- C.B.H. Dpt of Imaging-Clinica , Italy Carlo Florio
- Kyungpook National University Hospital , Korea (South) Jaekwang Lim
- Medicheck health care , Korea (South) Chae Lim
- Fukuyama City Hospital , Japan Mayu Uka
- Radiology Department, the First Affliated Hospital of Nanjing Medical University (Jiangsu province Hospital) , China Qiguang Cheng

▶ Correct Answer as Differential Diagnosis : 12/66, 18.2%

- Avrasya Hospital , Turkey Murat Ulusoy
- Oita University, Faculty of Medicine , Japan Fumito Okada
- chp st martin , France Mariotte benoit
- Kyung Hee University Hospital at Gangdong , Korea (South) Seong Jong Yun
- SKG radiology , Australia Yuranga Weerakkody
- Hopital Bichat , France, Metropolitan Pauline Pradere
- CHRU Lille , France Paul Lebert
- Nagasaki University Hospital, Department of Pathology , Japan TOMONORI TANAKA
- IRSA LA ROCHELLE , France, Metropolitan BIGOT
- Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
- jaslok hospital & research centre mumbai , India JAINENDRA JAIN
- All India Institute of medical sciences , India Justin Moses

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 843 Date 2013-12-23

Discussion

No. of Applicants : 66