[Alveolar lipoproteinosis, an incidental finding during an unrelated pre-surgical study with inadequate response to complete pulmonary washing].
The alveolar lipoproteinosis (ALP) is a rare pulmonary disease, characterized by an excess of phospholipids in the distal airway, and the most symptom of which is dyspnea. 35 years old patient in whom we observed incidentally pulmonary infiltrations in a chest X-ray during a unrelated pre-surgical study. We could not make a definitive diagnosis after further investigations carried out in the Division of Respiratory Medicine. We, therefore, sent this patient to the Division of Chest Surgery for a complementary video-thoracoscopy. Biopsy showed presence of ALP. The ALP is a rare disease that originates diagnosis difficulties, and that often needs lung biopsies to confirm its diagnosis. Once treated, its prognosis is excellent. The safest and most effective treatment is a complete pulmonary washing, that, in our patient, was required 7 months after diagnosis as this patient presented clinical deterioration and worsening in the lung function studies. This treatment did not achieve the expected goal. In the subsequent 6 months, we repeated the same treatment and failed again. We then started a treatment with granulocyte-macrophage-colony stimulating factor (GM-CSF) and obtain good response.
D000328:Adult; D018893:Bronchoalveolar Lavage / Q000379:methods*; D005260:Female; D006801:Humans; D033162:Incidental Findings; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D011300:Preoperative Care; D011649:Pulmonary Alveolar Proteinosis / Q000000981:diagnostic imaging* / Q000628:therapy*; D013902:Radiography, Thoracic; D020775:Thoracic Surgery, Video-Assisted / Q000379:methods; D017211:Treatment Failure