Papiris Spyros A, Tsirigotis Panagiotis, Kolilekas Likurgos, Papadaki Georgia, Papaioannou Andriana I, Triantafillidou Christina, Papaporfyriou Anastasia, Karakatsani Anna, Kagouridis Konstantinos, Griese Matthias, Manali Effrosyni D
2nd Pulmonary Department, 'Attikon' University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Expert Rev Respir Med. 2015 Jun;9(3):337-49. doi: 10.1586/17476348.2015.1035259. Epub 2015 Apr 12.
Pulmonary alveolar proteinosis (PAP) is categorized into hereditary, secondary and autoimmune PAP (aPAP) types. The common pathogenesis is the ability of the alveolar macrophages to catabolize phagocytized surfactant is affected. Hereditary PAP is caused by mutations involving the GM-CSF signaling, particularly in genes for the GM-CSF receptor and sometimes by GATA2 mutations. Secondary PAP occurs in hematologic malignancies, other hematologic disorders, miscellaneous malignancies, fume and dust inhalation, drugs, autoimmune disorders and immunodeficiencies. aPAP is related to the production of GM-CSF autoantibodies. PAP is characterized morphologically by the inappropriate and progressive 'occupation' of the alveolar spaces by an excessive amount of unprocessed surfactant, limiting gas exchange and gradually exhausting the respiratory reserve. Myeloid cells' immunity deteriorates, increasing the risk of infections. Treatment of PAP is based on its etiology. In aPAP, recent therapeutic advances might shift the treatment option from the whole lung lavage procedure under general anesthesia to the inhalation of GM-CSF 'as needed'.
肺泡蛋白沉积症（PAP）分为遗传性、继发性和自身免疫性PAP（aPAP）类型。常见的发病机制是肺泡巨噬细胞分解代谢吞噬的表面活性剂的能力受到影响。遗传性PAP由涉及GM-CSF信号传导的突变引起，特别是GM-CSF受体基因的突变，有时也由GATA2突变引起。继发性PAP发生于血液系统恶性肿瘤、其他血液系统疾病、各类恶性肿瘤、吸入烟雾和粉尘、药物、自身免疫性疾病和免疫缺陷。aPAP与GM-CSF自身抗体的产生有关。PAP的形态学特征是肺泡腔被过量未处理的表面活性剂不适当且进行性地“占据”，限制气体交换并逐渐耗尽呼吸储备。髓样细胞免疫功能恶化，增加感染风险。PAP的治疗基于其病因。在aPAP中，最近的治疗进展可能会将治疗选择从全身麻醉下的全肺灌洗手术转变为“按需”吸入GM-CSF。
