Discussion
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
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- Keywords
- Lung, Metabolic and storage lung disesae,