Belchior, I.Cerdeira, A.Santos, M.Braga, J.Aragâo, I.Martins, A.2011-11-202011-11-202011Rev Port Pneumol. 2011 May-Jun;17(3):139-420873-2159http://hdl.handle.net/10400.16/869Abstract Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by abnormal accumulation of a lipoproteinaceous material in the alveoli, which may lead to respiratory failure and has an associated high risk for infections. The mainstay treatment for PAP is whole lung lavage. A pregnant woman, previously diagnosed with primary PAP, the most common form of PAP, was admitted with dyspnea and worsening respiratory function. In one month period, a wholelung bronchopulmonary lavage was performed twice, with clinical and functional improvement. Pregnancy was carried to term and a healthy baby was delivered. The mechanisms of respiratory impairment are discussed as well as treatment options and response. Resumo A proteinose alveolar pulmonar (PAP) é uma doença rara caracterizada pela acumulação anormal de material lipoproteináceo nos alvéolos, que pode levar a insufi ciência respiratória, estando associada a um risco elevado de infecções. O tratamento gold-standard da PAP é a lavagem pulmonar total.engPregnancyPulmonary alveolar proteinosisPulmonary lavageRespiratory failureGravidezProteinose alveolar PulmonarLavagem pulmonarInsuficiência respiratóriajournal article