Focal Pulmonary Alveolar Proteinosis Presenting as a Solitary Pulmonary Nodule

肺蛋白質沉著症是一種磷脂白質堆積於肺泡的罕見疾病，此疾病的臨床症狀是多變化的，可從呼吸衰竭到自然康復。這種堆積於肺泡的磷脂白質造成胸腔放射影像呈現出典型之非特定肺泡型的浸潤，且通常是雙側及肺門周圍的實質化浸潤。肺蛋白質沉著症極罕見以單一肺結節為影像學的表現。我們報告一位先前健康良好的46歲女性，偶然在健康篩檢時發現右肺單一肺結節。經切除後，證實為局部性肺蛋白質沉著症；而在切除5年後以兩個結節的類似型態復發。

關鍵字

肺蛋白質沉著症；單一肺結節

並列摘要

Pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates within alveoli. The clinical course of the disease is variable, ranging from respiratory failure to spontaneous resolution. The classical radiographic patterns are non-specific airspace consolidation, and usually a bilateral and perihilar patchy consolidation in a ”bat-wing” distribution. The high-resolution computed tomography (HRCT) scan of the thorax shows widespread airspace consolidation, or the so-called ”crazy-paving” pattern. We report a 46-year-old woman with focal PAP presenting as a solitary pulmonary nodule. She had also had a recurrence of focal PAP with a similar nodular pattern 5 years after resection of the previous lesion. Since the radiographic presentation of focal PAP is not a characteristic of typical bilateral or asymmetric airspace consolidation, it should be considered in the differential diagnosis of solitary pulmonary nodules in middle-aged patients.

並列關鍵字

pulmonary alveolar proteinosis ； solitary pulmonary nodule

國際替代計量

Focal Pulmonary Alveolar Proteinosis Presenting as a Solitary Pulmonary Nodule

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