原发性干燥综合征患者肺部病变的临床分析

目的 探讨原发性干燥综合征(pSS)患者肺部病变的临床特点.方法 回顾性分析1985至2005年在北京协和医院确诊的pSS患者522例,按肺部病变[肺部影像学异常、肺功能下降或超声心动图肺动脉收缩压隐隐约约≥40 mm Hg(1 mm Hg=0.133 kPa)]将522例患者分为肺部病变组和无肺部病变组,对两组的临床特点进行分析.采用SPSS 13.0统计学软件包对正态分布的计量资料行t检验,偏态分布的计量资料行非参数检验,计数资料行卡方检验.结果 (1)522例患者中有肺部病变者221例(42.3%),多见于发病后48个月.肺部病变组发病年龄[(43±13)岁]晚于无肺部病变组[(37±14)岁](t=-5.445,P=0.000);肺部病变组口干、眼干及猖獗齿较无肺部病变组更常见(分别为89.6%,/35 81.1%,χ2=7.145,P=0.008;78.7%vs 66.4%,χ2=9.472,P=0.002;55.2%vs42.2%,χ2=8.647,P=0.003).(2)522例患者中最常见的pSS肺部病变是问质性肺病(116例,23.2%),其次是肺动脉高压(44例,12.5%).(3)11例行肺组织病理检测患者中5例为非特异性间质性肺炎,3例为淋巴细胞间质性肺炎.(4)肺部病变组更常出现雷诺现象、低热、关节肿、抗U1RNP抗体及高γ球蛋白血症(分别为26.7% vs 13.0%,χ2=15.77,P=0.000;20.4%vs 13.0%,χ2=5.175,P=0.023;29.4%vs21.6%,χ2=4.164,P=0.041),而肾小管酸中毒少见(5.4%vs12.6%,χ2=7.616,P=0.006).(5)pSS肺部病变可增加死亡风险达5.5倍,感染是最常见的死亡原因(9例).结论 肺部病变是pSS常见的系统损害,严重影响疾病预后,因此,确诊pSS的患者均应进一步明确肺部病,变情况.

Lung involvement of primary Sjogren's syndrome

Objective To evaluate the incidence, clinieal manifestations and immunological features of lung involvement in patients of primary Sjogren's syndrome (pSS). Methods Five hundred twenty-two patients with pSS in Peking Union Medical College Hospital between 1985 and 2005 were screened retrospectively for lung involvement by either the "abnormalities of chest imaging, lung function or the pulmonary artery systolic pressure estimated by ultrasonic echocardiogram ≥40 mm Hg ( 1 mm Hg = 0. 133 kPa), excluding infections, chronic obstructive pulmonary disease,asthma, congenital heart disease, rheumatic heart disease and other diseases. The difference was compared between patients with and without lung involvement. Alpatients fulfilled the 2002 international classification (criteria) for pSS. Results(1) The incidence of lung involvement in pSS was 42.3% (221/522) and occurred from 0 to 384 months (median, 48 months) after onset, while 25.2% occurred before the diagnosis of pSS. Only 47. 1% of the patients showed respiratory symptoms. The average age of onset was older in patients with lung involvement than in those without lung involvement [(43 ± 13)yr vs (37 ± 14)yr, t = -5.445, P = 0.000]. Incidences of dry mouth (89. 6% vs 81.1% ,χ2 =7. 145, P = 0. 008), dry eyes (78.7% vs 66. 4% ,χ2 = 9. 472, P = 0. 002 ) and rampant caries (55.2% vs 42. 2% , χ2 = 8. 647, P = 0. 003 ) were higher in patients with lung involvement than those without. There was no significant difference in sex ratio between the two groups.(2) Interstitial lung disease was the most common lung involvement and occurred in 23.2% of the patients. Pulmonary artery hypertension in 12. 5%, multiple pulmonary bullae in 9. 2%, pleural effusion in 6. 0% and multiple pulmonary nodules in 5.6%.(3) The major histopathologieal patterns were nonspecific interstitial pneumonia ( 5/11 eases),lymphocytic interstitial pneumonia ( 3/11 cases).(4) Incidences of Ranaud ' s phenomenon ( 26.7% vs 13.0%, χ2 = 15.77, P = 0. 000 ) , low-grade fever (20.4% vs 13.0% ,χ2=5. 175, P =0. 023), arthrosis (29.4% vs 21.6% ,χ2 =4.164, P=0. 041), anti- U1RNP (18.2% vs 11.2%,χ2 =5.069, P=0.024) and hypergammaglobulinemia (51.6% vs 39.5%, χ2 =6. 597,P =0.01 )were higher in patients with lung involvement than in those without. The incidence of renal tubule acidosis was lower in patients with lung involvement than in those without (5.4% vs 12. 6%, χ2 =7. 616, P =0.006). (5) The death incidence in pSS with pulmonary involvement was 5. 5 times higher than in those without. The most frequent cause of death was infection ( 64.3% ), especially pulmonary infection. Conclusion Lung involvement in pSS is common. As it is an important factor related to the prognosis of this disease, chest X-ray, HRCT, lung function and ultrasonic echocardiogram after the diagnosis are suggested.