Clinical Aspects and Evolution under Current Treatments on Pulmonary Alveolar Phospholipoproteinosis Patients

A 34-year-old female smoker (4 packs a year), with the following symptoms: dry cough, moderate dyspnea, fatigue, night sweats and no exposure to respiratory poisoning or contact with tuberculosis. Clinical findings at admission: normal temperature, Hippocratic fingers and cyanosis, diminished bilateral breath sounds, fine bilateral rattling respiratory sounds, rhythmic cardiac noises, oxygen saturation 95%, pulse 87, blood pressure 125/80 mm Hg. Chest computed tomography describes infiltration images of intra-alveolar and intra-bronchial with matte glass pattern, confluent and stretched across all lung segments. Pulmonary biopsy: preserved pulmonary architecture, thick alveolar septs and terminal bronchioles, alveoli and macrophages loaded with lipoprotein material. Bronchial aspirate: negative BAAR, no tumor cells. Bronchoalveolar lavage: opalescent, abundant PAS + appearance. total cell numbers - 4.3 million, macrophages - 34.1%, lymphocytes - 56.9%, neutrophils - 10%, eosinophils - 0.4%, epithelial cells - 33%.The treatment option chosen for this case was total bronchoalveolar lavage for therapeutic purposes. The patient had a rapid favourable evolution and at discharge was recommended a periodic imaging and functional control. Key Messages: Bronchoalveolar lavage is the optimal diagnostic method. Moderate/severe forms, total bronchoalveolar lavage is recommended. Systemic corticotherapy or immunosuppression is not indicated. GM-CSF administered subcutaneously or by nebulization. Rituximab/plasmapheresis are under evaluation. Pulmonary transplantation is indicated in patients who do not respond to therapeutic bronchoalveolar lavage repeated 6-12 months.