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目的探讨自身免疫性肺泡蛋白沉积症(PAP)患者的临床特征及影像学与血清粒-巨细胞集落刺激因子自身抗体(GMAb)和血清细胞角蛋白碎片(CYFRA21-1)等生物学标志的关系。方法连续选取南京大学医学院附属鼓楼医院2006年1月至2012年12月血清GMAb阳性的PAP患者,收集患者临床影像学及CYFRA21-1等生物学标志资料,进行分析比较。结果共纳入26例肺泡蛋白沉积症患者,血清GMAb水平为28.64(19.2~75.4)μg/mL,均超过诊断界值2.39μg/mL,诊断为自身免疫性PAP。30例正常对照组的血清GMAb水平为0.10(0.05~0.15)μg/mL,均低于诊断界值。26例自身免疫性PAP中34.6%有职业吸入暴露史,血清GMAb水平与患者的疾病严重评分(DSS)、肺功能指标、高分辨率CT(HRCT)评分及血气分析等参数无显著相关性(P>0.05),患者DSS与PaO2、FVC%pred、TLCO%pred及HRCT评分中的病灶范围评分(OE)和病灶严重度评分(OS)具有显著相关性(P<0.05),患者的血清细胞角蛋白碎片(CYFRA21-1)水平为9.9(4.3~19.5)ng/mL,较正常对照组明显升高(P<0.05),但升高水平与DSS、肺功能、影像学各指标无显著相关性(P>0.05)。患者胸部HRCT表现为铺路石征比例为92.3%,表现为病灶地图样分布比例为100%。结论血清GMAb及血清CYFRA21-1可作为自身免疫性PAP患者的重要血清学诊断性标记,职业吸入暴露在自身免疫性PAP中较为常见。
Objective To investigate the clinical features and the relationship between the imaging and the biological markers of serum GMAb and CYFRA21-1 in patients with autoimmune pulmonary alveolar proteinosis (PAP) . Methods Serum GMAb-positive PAP patients were randomly selected from Drum Tower Hospital of Nanjing University Medical College from January 2006 to December 2012. The clinical imaging and CYFRA21-1 biomarkers were collected and analyzed. Results A total of 26 patients with pulmonary alveolar proteinosis were enrolled in this study. Serum GMAb level was 28.64 (19.2-75.4) μg / mL, both exceeding diagnostic limit of 2.39 μg / mL and diagnosed as autoimmune PAP. Serum levels of GMAb in 30 normal controls were 0.10 (0.05-0.15) μg / mL, both lower than the diagnostic cutoff. There were 34.6% of 26 autoimmune PAPs with occupational exposure history, serum GMAb level had no significant correlation with patient’s disease severity score (DSS), pulmonary function index, high resolution CT (HRCT) score and blood gas analysis P> 0.05). There was a significant correlation between DSS and PaO2, FVC% pred, TLCO% pred and HRCT scores (OE) and severity of severity score (OS) The level of CYFRA21-1 was 9.9 (4.3-19.5) ng / mL, which was significantly higher than that of the normal control group (P <0.05), but there was no significant correlation between the elevated level and the indexes of DSS, pulmonary function and imaging (P> 0.05). Patient chest HRCT showed paving stone syndrome rate was 92.3%, showing the lesion pattern distribution ratio of 100%. Conclusions Serum GMAb and serum CYFRA21-1 can be used as important serological diagnostic markers in patients with autoimmune PAP. Occupational inhalation exposure is more common in autoimmune PAP.