原发性干燥综合征合并间质性肺病的临床分析

目的探讨原发性干燥综合征合并间质性肺病(pSS-ILD)的发生情况、特征及早期诊断方法.方法对96例原发性干燥综合征患者(pSS),采用速率反射比浊法检测类风湿因子(RF)、C-反应蛋白(CRP)、血蛋白电泳、C3、C4;间接免疫荧光法和免疫印迹法检测抗核抗体(ANA)及其亚类.血气分析测定动脉血pH值、动脉血氧分压(PO2)、动脉血二氧化碳分压(PCO2).肺功能仪测定潮气容积(VT)、最大肺活量(VCmax)、用力肺活量(FVC)、第一秒用力呼气容积(FEV1)、最大呼气中段流量(MMFF)、最大通气量(MVV)和一氧化碳弥散功能(DLCO).放射学检查包括胸部正侧位X线片和肺高分辨CT(HRCT)扫描.结果本组pSS患者ILD的发生率为21.9%,易出现于抗SSA抗体阳性、RF高滴度、高γ球蛋白血症及临床活动期的患者;肺功能检测异常主要为弥散功能降低和限制性通气障碍;肺HRCT在发现pSS-ILD时优于普通X线胸片.结论pSS-ILD的发生与原发病的活动明显相关,肺弥散功能、HRCT对诊断pSS-ILD及判断预后具有重要价值;早期诊断与治疗的患者预后良好.

A clinical analysis of patients with interstitial lung disease in primary sj(o)gren syndrome

Objective To investigate the prevalence and features of interstitial lung disease in primary sjogren syndrome (pSS-ILD) and its early diagnosis. Methods Ninety-six cases with pSS were analyzed. Serum rheumatoid factor (RF), C reactive protein (CRP), serum protein electrophoresis, and complement C3, C4 were measured by velocity reflex turbidimetry. Antinuclear antibodies (ANAs) and anti-ENA antibodies were measured by indirect immunofluorescence technique and Western blot. Arterial gas was analyzed including PaO2, PaCO2. The following pulmonary function tests were performed: tidal volume (VT), maximal vital-capacity (VCmax), forced vital capacity (FVC), one second forced expiratory volume (FEV1), maximal midexpiratory flow (MMFF), maximal voluntary ventilation (MVV), and diffusing capacity CO (DLCO). Radiology examinations included chest X radiographs, and chest HRCT. Results The incidence of pSS-ILD was 21.9%. pSS-ILD frequently occurred in the patients with anti-SSA antibody and high tite of RF and patients with active disease. The impairment of lung function was the decreased DLCO and restricted ventilatory function abnormalities. HRCT was more sensitive than chest X radiography in the diagnosis of pSS-ILD. Conclusions The incidence of pSS -ILD is associated with the activity of pSS. Pulmonary diffusion ventilation function and HRCT have high valve in the diagnosis and prognosis of pSS-ILD. The prognosis is good if diagnosed and treated early.