대한흉부영상의학회 Weekly Chest Cases

Case No : 692 Date 2011-01-31

Courtesy of Jae Kwang Lim, Duk Sik Kang / Kyungpook National University Hospital
Age/Sex 46 / F
Chief ComplaintChest x-ray abnormality at health checkup

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Diagnosis With Brief Discussion

Diagnosis: Pulmonary alveolar proteinosis
Radiologic Findings: A 46-year-old female presented with abnormal findings on chest radiograph. The patient underwent annual physical examination and had no respiratory symptoms. Chest radiograph shows bilateral multifocal ground-glass opacities (Figure 1). CT images show multifocal patchy areas of ground-glass opacity, peribronchial and subpleural in distribution, with inter- and intralobular interstitial thickening in both lungs (Figure 2 and 3). Diagnostic impression was BOOP. But
histopathological diagnosis turned out to be pulmonary alveolar proteinosis by wedge resection of right middle lobe.
Brief Review: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by accumulation of protein- and lipid-rich material resembling surfactant within the parenchymal airspaces. More than 90% of cases occur as acquired disease of unknown etiology that appears to be related to the presence of an antibody to granulocyte-macrophage colony-stimulating factor. Clinical symptoms are variable, ranging from mild progressive dyspnea to respiratory failure.
The characteristic radiographic pattern of PAP consists of bilateral patchy areas of consolidation that have a vaguely nodular appearance. The CT appearance of crazy-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. Its subpleural and peribronchial distribution like this case appears to be unusual for PAP.
Definitive diagnosis is made with lung biopsy or bronchoalveolar lavage, with 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 : 108

▶ Correct Answer : 9/108, 8.3%

- Oita University, Faculty of Medicine , Japan Fumito Okada
- Fukuyama daiichi Hospital , Japan Mototsugu Saeki
- Ondokuz Mayis University , Turkey Cetin Celenk
- Kobe City Medical Center General Hospital , Japan Hitomi Nagano
- CHRU lille , France manuel toledano
- Shree Diagnostics , India amol jagdale
- clinique de SAVOIE , France, Metropolitan gay-depassier philippe
- Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
- NDMVP Nashik , India Imran Jindani

▶ Correct Answer as Differential Diagnosis : 13/108, 12.0%

- PingTung Christian Hospital ,China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
- radiologist, aditya imaging centre , India vivek patel
- Onomichi municipal hospital , Japan Hirofumi Mifune
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
- Virgin Mary Hospital Burgas , Bulgaria VLADISLAV RUSINOV
- Istanbul , Turkey Armagan Sarac
- Osaka University , Japan Hiromitsu Sumikawa
- attikon hospital , Greece konstantinos kagouridis
- IRSA La Rochelle France , France Denis Chabassiere
- IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
- Seoul Nationial University Bundang Hospital , Korea (South) Hee Seok Choi
- Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
- NTUH , Taiwan Kuei-pin Chung

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 692 Date 2011-01-31

Discussion

No. of Applicants : 108