CAJ | 학술논문

肺泡蛋白沉积症(PAP)是一种肺部少见病,以肺泡腔和细支气管腔沉积PAS染色阳性的不可溶性富磷脂蛋白质物质为特征.可分为因表面活性蛋白或GM-CSF受体基因突变导致的先天性PAP、因GM-CSF抗体阻断了肺泡巨嗟细胞的激活导致的自身免疫性PAP和继发于血液系统疾病或吸入有毒气体导致的继发性PAP.临床表现主要为进行性气促、低氧血症,胸部高分辨率CT主要表现为毛玻璃样高密度影和“疯狂堆砌”改变,支气管肺泡灌洗液PAS染色阳性可确诊.肺泡灌洗治疗有效,GM-CSF替代疗法和针对GM-CSF抗体的治疗是正在研究的治疗方法.
폐포단백침적증(PAP)시일충폐부소견병,이폐포강화세지기관강침적PAS염색양성적불가용성부린지단백질물질위특정.가분위인표면활성단백혹GM-CSF수체기인돌변도치적선천성PAP、인GM-CSF항체조단료폐포거차세포적격활도치적자신면역성PAP화계발우혈액계통질병혹흡입유독기체도치적계발성PAP.림상표현주요위진행성기촉、저양혈증,흉부고분변솔CT주요표현위모파리양고밀도영화“풍광퇴체”개변,지기관폐포관세액PAS염색양성가학진.폐포관세치료유효,GM-CSF체대요법화침대GM-CSF항체적치료시정재연구적치료방법.
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of PAS positive surfactant.PAP can be divided into the genetic PAP which results from mutations in surfactant proteins or GM-CSF receptor genes,the PAP secondary to toxic inhalation or haematological disorders,and the auto-immune PAP with anti-GM-CSF antibodies blocking activation of alveolar macrophages,Dyspnoea and hypoxemia are the most common presenting symptoms.Highresolution computed tomography shows ground-glass opacities and a characteristic "crazy paving" pattern.The PAS positive bronchoalveolar lavage findings establish the diagnosis.Whole lung lavage is effective,The recombinant GM-CSF therapy and therapies targeting anti-GM-CSF antibodies (rituximab and plasmapheresis) are being investigated.