PULMONARY ALVEOLAR PROTEINOSIS: VARIABILITY IN CLINICAL FEATURES

Abstract

TYPE: Abstract Publication TOPIC: Diffuse Lung Disease PURPOSE: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of surfactant in alveoli and terminal airways resulting in hypoxemic respiratory failure. Its radiological presentation may have particular characteristics important to a suspected diagnosis. Gold standard for treatment is whole lung lavage (WLL), an invasive procedure without standard indications. METHODS: We reported and discussed about the clinical features of 3 patients clinically diagnosed as PAP. RESULTS: At the time of diagnosis, patients were 42-year-old (female, Case 1), 60-year-old (male, Case 2), and 50-year-old (male, Case 3). Two severe patients presented with progressive dyspnoea and hypoxemic respiratory failure, and one mild patient only with dry cough and hypoxemia. Two of them was ex-smokers (Cases 2 and 3). There were nodular bilateral diffuse infiltrates on posteroanterior chest radiography. Computed tomography demonstrated diffuse alveolar opacity with a “crazy paving" configuration (thickening of interlobular and intralobular septa and ground-glass opacities) extending throughout both lung-fields but sparing some areas, particularly in the lung periphery. In all the cases PAP was diagnosed based on transthoracic lung biopsy. The second patient presented spontaneous regression of the PAP without the need for WLL. In the other two cases, the number of WLL varied. CONCLUSIONS: We presented a rare clinical entity with typical clinical and radiological features in order to improve differential diagnostic approach to patients with bilateral lung opacities. CLINICAL IMPLICATIONS: In patients with pulmonary alveolar proteinosis timely diagnosis and adequate treatment can improve the prognosis. DISCLOSURE: No significant relationships. KEYWORDS: whole lung lavage, Pulmonary alveolar proteinosis