Lipoprotéinose alvéolaire pulmonaire de présentation inhabituelle

Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of granular lipoproteinaceous material within the alveoli; the interstitium is preserved. We report the case of a 27-year-old patient hospitalized for exploration of asymptomatic bilateral interstitial syndrome. Neither the radiographic signs nor the aspect of bronchoalveolar lavage was typical. The diagnosis was confirmed by pathology examination of surgical lung biopsy specimen which revealed lesions of interstitial fibrosis and cholesterol granulomas in association with pulmonary alveolar proteinosis. Since there was no functional deterioration, therapeutic abstention was decided.