A clinical case of late diagnosis of PAP with good response to whole lung lavage

Abstract

Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by the accumulation of surfactant in alveoli and terminal airways resulting in respiratory failure. PAP comprises part of a spectrum of disorders of surfactant homeostasis (clearance and production). We describe the case of a 54 years-old woman hospitalized in March 2017 with diagnosis of acute hypoxemic respiratory failure secondary to pulmonary interstitial disease. In our case, the patient arrived at our observation after developing a severe respiratory insufficiency although a long term history of respiratory symptoms. This diagnostic delay suggests that alveolar proteinosis should always be considered as a possible diagnosis in patients with dyspnea, cough and crazy paving at HRTC. In spite of this the disease resolved with a cycle of total lung lavage and the symptomatology has improved with the resolution of the radiological picture without any other therapeutic approach.