胸部高分辨CT在肺泡蛋白沉积症分级诊断中的应用价值

目的从胸部影像学角度探讨肺泡蛋白沉积症(PAP)的诊断分级,以指导治疗。方法对上海市肺科医院2000年1月至2010年12月收治的31例确诊PAP患者胸部高分辨CT(HRCT)进行分级,选择4个代表层面(主动脉弓、隆突、左或右下肺静脉汇合层面和膈上层面),病灶在这些层面的所占范围进行5级评分,结合临床症状、肺功能指标,建立一套PAP胸部HRCT诊断分级标准,根据诊断分级提出相应的治疗决策。结果 (1)按胸部HRCT病灶范围将PAP患者分为4级:1级(≤8分)3例;2级(>8～16分)12例;3级(>16～24分)6例;4级(>24分)10例。(2)PAP患者胸部HRCT评分与呼吸困难评分、症状总评分呈正相关(r=0.748、0.578,P均<0.01)。(3)PAP患者胸部HRCT评分与用力肺活量(FVC)占预计值%、第一秒用力呼气容积(FEV1)占预计值%、一氧化碳弥散量占预计值%及动脉血氧分压(PaO2)均呈负相关(r=-0.486、-0.376、-0.596、-0.444,P<0.01或0.05)。(4)结合胸部HRCT分级和PaO2将PAP患者分为4期:1期:HRCT分级1级伴PaO2≥8.0 kPa;2期:HRCT分级2级伴PaO2≥8.0 kPa;3期:HRCT分级3级伴PaO2≥8.0 kPa;4期:HRCT分级4级,或HRCT分级2～3级伴PaO2<8.0 kPa。(5)不同PAP患者胸部HRCT诊断分级建议:1、2期建议对症治疗及长期随访胸部HRCT;3期患者建议序贯肺泡灌洗或GM-CSF治疗;4期患者建议全肺灌洗。结论胸部HRCT诊断分级可以作为评估PAP患者病情严重程度的基础,并在此基础上选择合适的治疗方法,对临床上诊断和治疗PAP具有一定指导意义。

The role of high resolution computed tomography in grading diagnosis of pulmonary alveolar proteinosis:a application study

Objective To explore the grading diagnosis of pulmonary alveolar proteinosis（PAP） with chest imaging for guidance of treatment. Methods The high resolution computed tomography（HRCT） was graded in 31 patients conformed with PAP and selected 4 representative lays（aortic arch,tracheal carina,left/right inferior lung vein converging and above of diaphragm） for 5 grade scoring of nidus in the scope of those lays.A suit of HRCT diagnostic scoring gradation standard for PAP was established in combination of clinical symptoms and lung functional indicators.Moreover,the corresponding therapy was raised according to the diagnostic gradation. Results（1） Based on the HRCT nidus scope,PAP patiens were divided into four levels including level 1（score≤8）,level 2（824）.（2） The HRCT scoring was positively correlated with breath difficulty scoring and total scoring of symptoms in PAP patients（ r=0.748 vs 0.578,both P<0.01）.（3） The HRCT scoring was negatively correlated with forced vital capacity/predicated value（FVC% Pred）,forced expiratory volume in one second/predicted value（FEV₁% Pred）,diffusing capacity of the lung for carbon monoxide /predicted value（D_LCO% Pred）and arterial partial pressure of oxygen（PaO₂）in PAP patients（ r=-0.486,-0.376,-0.596,-0.444, P<0.01 or 0.05）.（4） According to HRCT grading and PaO₂,PAP patients were divided into 4 phases:phase 1:HRCT 1 and PaO₂≥8.0 kPa,phase 2:HRCT 2 and PaO₂≥8.0 kPa,phase 3:HRCT 3 and PaO₂≥8.0 kPa,phase 4:HRCT 4 or 2³ and PaO₂<8.0 kPa.（5） Diagnostic grading suggestions for different PAP patients:symptomatic treatment and long-term follow-up HRCT were recommended for patients in phase 2;repeatedly alveolar washing or GM-CSF treatment was for patients in phase 3;and whole lung lavage was for patients in phase 4. Conclusion HRCT diagnostic grading could be considered as the basis in evaluation of the severity of PAP hence the suitable therapies,which may have a certain of guidance in clinical diagnosis and PAP treatment.