The Clinical Clues of Pulmonary Alveolar Proteinosis: A Report of 11 Cases and Literature Review

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease characterized by the abnormal alveolar accumulation of surfactant components. The diagnosis of PAP can be easily missed since it is rare and lacks specific clinical symptoms. It is of great importance to have a better understanding of the crucial clue to clinically diagnose PAP and take PAP into consideration in the differential diagnosis of interstitial pulmonary diseases or other diseases with similar manifestations. Here, we analyze the clinical characteristics of 11 cases of PAP patients in local hospital and review the relevant literature in order to provide more information in diagnosis and management of PAP. In our observation, cyfra21-1 and neuron-specific enolase (NSE) known as tumor markers probably can be useful serum markers for diagnosis of PAP. As for the method of pathologic diagnosis, open-lung biopsy was the gold standard but now it is less required because findings on examination of bronchoalveolar lavage fluid (BALF) can help to make the diagnosis. We also have deep experience about when and how to carry out lung lavage.

An exploratory study investigating biomarkers associated with autoimmune pulmonary alveolar proteinosis (aPAP)

“…Lung function findings from this study agree with published observations 4 , 14 , 22 , 25 – 27 . In patients with aPAP, DL CO is frequently reduced, whereas FVC and FEV 1 are generally within normal limits (although some patients with aPAP show decreased FVC) 26 , 28 .…”

Alveolar Proteinosis: Bronchoalveolar Lavage Experience and Current Literature Review

“…In a study by Mo et al in which they examined 11 PAP patients, a total of 8 patients had BAL procedure done. Their results showed that patients with a large alveolar lavage volume and/or bilateral lavage had a greater improvement in PaO2 than those who had less volume and/or unilateral lavage, subsequently leading to the conclusion that high-volume lavage is more effective in the treatment of PAP [10].…”

Pulmonary alveolar proteinosis with upper‐lobe predominance in a non‐smoking female

“…It is about two‐fold more common in male than in female, and is more common in smokers or those with a positive history of causative exposure. The onset is usually insidious, with such non‐specific symptoms as dyspnoea, cough, fatigue, and occasionally fever . Pulmonary function tests almost consistently reveal a reduction in the DLco indicating the presence of ventilation‐perfusion mismatch, with or without a restrictive‐type ventilatory deficit .…”