Pulmonary alveolar proteinosis and first successful whole lung lavage in Sri Lanka: a case report

Abstract

BackgroundPulmonary alveolar proteinosis is a rare disease characterized by accumulation of lipoproteinaceous material within alveoli. There are three clinically distinct forms: congenital, acquired and secondary. Whole lung lavage is currently the gold standard therapy for severe cases of pulmonary alveolar proteinosis. In Sri Lanka this is the first reported successful whole lung lavage for a patient with pulmonary alveolar proteinosis.Case presentationWe describe the case of a 15-year-old Sri Lankan girl who presented with symptoms of progressive shortness of breath and dry cough for 6 months’ duration. She had a history of exposure to silica in her household environment. High-resolution computed tomography revealed crazy paving appearance in both lungs suggestive of pulmonary alveolar proteinosis. An open lung biopsy revealed intra-alveolar granular amphophilic material which was strongly periodic acid–Schiff positive and diastase resistant, which is consistent with pulmonary alveolar proteinosis. She was followed up for 2 years with periodical segmental bronchoalveolar lavages which showed minimal improvement in her symptoms as well as in exercise desaturation. Due to severe dyspnea and hypoxemia on exertion, she underwent whole lung lavage. It resulted in a marked improvement in her symptoms, exercise desaturation, and chest X-ray results.ConclusionWhole lung lavage was successfully performed for the first time in Sri Lanka for a patient with pulmonary alveolar proteinosis.

Unilateral Presentation of Pulmonary Alveolar Proteinosis

“…Specific indications include a definitive histologic diagnosis and one of the following: resting PaO 2 <65 mm Hg (at sea level), alveolar-arterial O 2 gradient ≥40 mm Hg, measured shunt fraction >10-12%, or severe dyspnea and hypoxemia at rest or on exercise. 10 More recently, therapy with GM-CSF has been attempted. Although a positive effect of GM-CSF has been shown in PAP, its long-term safety has not been determined, and the optimal dose, the optimal duration of treatment, and the optimal route of GM-CSF remain unclear.…”

Anaesthetic Management of Whole Lung Lavage in a Resource-Limited Setting: Case Report

“…The process of WLL has complex cardiopulmonary interactions and is a challenge for an anaesthetist. In Sri Lanka, only a few cases have been reported 3,4 but none have described the experience of anaesthetising for WLL. We report a case of a 32year-old female with severe PAP who underwent WLL in a resource-limited setting.…”