대한흉부영상의학회 Weekly Chest Cases

Case No : 404 Date 2005-07-25

Courtesy of Hye-Jung Choo, MD, Yeon Joo Jeong, MD, Kun-Il Kim, MD. / Pusan National University Hospital, Busan, Korea
Age/Sex 28 / M
Chief ComplaintProductive cough with hemoptysis for two months

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Diagnosis With Brief Discussion

Diagnosis: Pulmonary Alveolar Proteinosis
Radiologic Findings: Posteroanterior chest radiograph (Fig. a) shows bilateral reticular areas of increased opacity, which occur predominantly in the lower zones. High-resolution CT scans (Fig. b-d) show bilateral areas of ground-glass attenuation with interlobular septal and intralobular interstitial thickenings. VATS biopsy was done. Histologically, alveoli are filled with finely granular eosinophilic material that stains with periodic acid-Schiff.
Brief Review: Pulmonary alveolar proteinosis can be understood as a syndrome of altered surfactant homeostasis, leading to a pathologic accumulation of surfactant (1). Pulmonary alveolar proteinosis is most common in adults between 20 and 50 years of age, although it has been reported in a wide range of ages. The clinical presentation of pulmonary alveolar proteinosis is nonspecific. Patients with pulmonary alveolar proteinosis have an insidious onset of symptoms that typically includes dyspnea, fatigue, and nonproductive cough, but patients with the condition can be asymptomatic (2). Classic radiographic finding is bilateral, symmetric alveolar consolidation or ground-glass opacity, particularly in a perihilar or hilar distribution resembling pulmonary edema. Occasionally, involvement is unilateral. Interstitial disease can also be seen, especially in longstanding cases. High-resolution CT typically shows diffuse ground-glass attenuation with superimposed intra- and interlobular septal thickening, often in polygonal shapes representing the secondary pulmonary lobule. Involvement could be central or peripheral. The diagnosis is established with bronchoalveolar lavage (3, 4).
References: 1. Claypool WD, Rogers RM, Matuschak GM. Update on the clinical diagnosis, management, and pathogenesis of pulmonary alveolar proteinosis (phospholipidosis). Chest 1984; 85:550 -558

2. Wang BM, Stern EJ, Schmidt RA, Pierson DJ. Diagnosing pulmonary alveolar proteinosis: a review and an update. Chest 1997; 111:460-466.

3. Holbert JM, Costello P, Li W, Hoffman RM, Rogers RM. CT features of pulmonary alveolar proteinosis. AJR Am J Roentgenol 2001; 176:1287-1294.

4. 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 : 26

▶ Correct Answer : 7/26, 26.9%

- SSGH, Vadodara Medical College, Gujarat, India Sushil Mansingani
- Chung Li Ten-Chen Hospital, Taiwan Zheng Gui-Lin
- IRCCS S.Luca Hospital, Milano, Italy Filippo Casolo
- Ping Tung Christian Hospital,Taiwan Jun-Jun Yeh
- Max Hospital, New Delhi, India Anurag Jain
- Monaldi Hospital, Naples, Italy Gaetano Rea
- Ewha Womans University Mokdong Hospital, Korea Yookyung Kim

▶ Correct Answer as Differential Diagnosis : 10/26, 38.5%

- Yonsei University Hospital, Korea Eun Hye Yoo
- ASL Bologna, Maggiore Hospital, Bologna, Italy Marcellino Burzi
- Samsung Medical Center, Seoul, Korea Ha Young Kim
- Radiologie Guiton, La Rochelle, France Denis Chabassiere
- Homs National Hospital, Homs, Syria Rami Abou Zalaf
- Hangang SacredHeart Hospital, Korea Eil Seong Lee
- Gwangmyoung Sungae Hospital, Korea Jiyong Rhee
- Cabinet de Radiologie Guiton, La Rochelle, France Jean-Luc Bigot
- Max Hospital, New Delhi, India Vickrant Malhotra
- Social Security Hospital, Ankara, Turkey Meric Tuzun

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 404 Date 2005-07-25

Discussion

No. of Applicants : 26