Prakash U B, Barham S S, Carpenter H A, Dines D E, Marsh H M
Mayo Clin Proc. 1987 Jun;62(6):499-518. doi: 10.1016/s0025-6196(12)65477-9.
A retrospective review of Mayo Clinic records through 1983 revealed 84 patients (24 male and 10 female; mean age, 41 years) with the diagnosis of pulmonary alveolar phospholipoproteinosis. The major clinical features were dyspnea, cough, fever, and chest pain. Chest roentgenograms usually showed bilateral symmetric alveolar infiltrates, but asymmetric, unilateral, and chronic patchy patterns were also noted. Diagnosis was established by thoracotomy-lung biopsy in 26 patients. Histologic analysis revealed uniform filling of the alveoli by periodic acid-Schiff-positive material and maintenance of normal alveolar architecture. Electron microscopy showed enlarged alveolar macrophages with lamellar osmiophilic inclusions, dense granules, and myeloid bodies. Of the 21 patients who underwent therapeutic bronchoalveolar lavage, 13 had no recurrence of the disease during a mean follow-up of 8.8 years. In patients who underwent pulmonary function testing both before and after lavage, significant restrictive dysfunctions present before the procedure were alleviated afterward. Three deaths occurred among the 34 patients. Pulmonary alveolar phospholipoproteinosis may result from defective clearance of phospholipids by the alveolar macrophages, excessive production of phospholipids by type II pneumocytes, or both. It is likely a nonspecific response to a variety of injuries to the alveolar macrophage or type II pneumocyte or both, including exposure to certain dusts and chemicals and occurrence of hematologic diseases or infections. The uncommon occurrence of this disorder suggests individual susceptibility.
对梅奥诊所截至1983年的记录进行的回顾性研究发现，84例患者（24例男性，10例女性；平均年龄41岁）被诊断为肺泡蛋白沉积症。主要临床特征为呼吸困难、咳嗽、发热和胸痛。胸部X线片通常显示双侧对称的肺泡浸润，但也可见不对称、单侧和慢性斑片状影像。26例患者通过开胸肺活检确诊。组织学分析显示肺泡内均匀填充有过碘酸雪夫染色阳性物质，肺泡结构保持正常。电子显微镜检查显示肺泡巨噬细胞增大，含有板层状嗜锇包涵体、致密颗粒和髓样小体。在接受治疗性支气管肺泡灌洗的21例患者中，13例在平均8.8年的随访期间疾病未复发。在灌洗前后均进行肺功能测试的患者中，灌洗前存在的明显限制性功能障碍在灌洗后得到缓解。34例患者中有3例死亡。肺泡蛋白沉积症可能是由于肺泡巨噬细胞对磷脂的清除功能缺陷、II型肺泡上皮细胞磷脂生成过多，或两者兼而有之。它可能是对肺泡巨噬细胞或II型肺泡上皮细胞或两者的多种损伤的非特异性反应，包括接触某些粉尘和化学物质以及发生血液系统疾病或感染。这种疾病的罕见发生提示个体易感性。
