中华内科杂志
中華內科雜誌
중화내과잡지
CHINESE JOURNAL OF INTERNAL MEDICINE
2008年
4期
296-299
,共4页
颜淑敏%曾小峰%赵岩%董怡
顏淑敏%曾小峰%趙巖%董怡
안숙민%증소봉%조암%동이
干燥综合征,原发性%抗着丝点抗体
榦燥綜閤徵,原髮性%抗著絲點抗體
간조종합정,원발성%항착사점항체
Sj(o)gren's syndrome,primary%Anticentromere antibodies
目的 通过分析抗着丝点抗体(ACA)阳性原发性干燥综合征(pSS)患者的临床特点,提高对本病的诊治水平.方法 回顾性分析北京协和医院风湿免疫科收治的60例ACA阳性pSS患者(ACA阳性组)的临床资料,并与同期收治的139例ACA阴性pSS者(ACA阴性组)相比较.结果 (1)ACA阳性组发病年龄晚于ACA阴性组[(48±11)岁比(41±12)岁,P=0.000],而2组在性别构成、口干、眼干、唇腺活检阳性率差异元统计学意义.(2)肝脏受累是ACA阳性组患者最常见的腺外脏器损害,其发生率远高于ACA阴性组(68.3%比37.0%,P=0.000),而肾脏受累(13.3%比30.9%,P=0.009)、神经系统受累(1.7%比11.5%,P=0.025)、高γ球蛋白血症的发生率(20.8%比45.7%,P=0.002)均低于ACA阴性组.(3)2组抗核抗体阳性率无差异,但ACA阳性组以散点型免疫荧光表型最为常见(占61.7%).与ACA阴性组相比,ACA阳性组中抗SSA抗体、抗SSB抗体、抗U1 RNP抗体的阳性率减低(P＜0.05),而抗线粒体抗体及其M2亚型的阳性率增高(P＜0.05).(4)ACA阳性组死亡5例,其中3例死于胃食管静脉曲张破裂出血.结论 对ACA阳性pSS者应警惕肝脏受累.由于ACA阳性pSS在发病年龄、腺外器官受累、免疫指标、自身抗体谱及预后方面均不同于经典pSS,故可能是pSS的一种独特亚型.
目的 通過分析抗著絲點抗體(ACA)暘性原髮性榦燥綜閤徵(pSS)患者的臨床特點,提高對本病的診治水平.方法 迴顧性分析北京協和醫院風濕免疫科收治的60例ACA暘性pSS患者(ACA暘性組)的臨床資料,併與同期收治的139例ACA陰性pSS者(ACA陰性組)相比較.結果 (1)ACA暘性組髮病年齡晚于ACA陰性組[(48±11)歲比(41±12)歲,P=0.000],而2組在性彆構成、口榦、眼榦、脣腺活檢暘性率差異元統計學意義.(2)肝髒受纍是ACA暘性組患者最常見的腺外髒器損害,其髮生率遠高于ACA陰性組(68.3%比37.0%,P=0.000),而腎髒受纍(13.3%比30.9%,P=0.009)、神經繫統受纍(1.7%比11.5%,P=0.025)、高γ毬蛋白血癥的髮生率(20.8%比45.7%,P=0.002)均低于ACA陰性組.(3)2組抗覈抗體暘性率無差異,但ACA暘性組以散點型免疫熒光錶型最為常見(佔61.7%).與ACA陰性組相比,ACA暘性組中抗SSA抗體、抗SSB抗體、抗U1 RNP抗體的暘性率減低(P＜0.05),而抗線粒體抗體及其M2亞型的暘性率增高(P＜0.05).(4)ACA暘性組死亡5例,其中3例死于胃食管靜脈麯張破裂齣血.結論 對ACA暘性pSS者應警惕肝髒受纍.由于ACA暘性pSS在髮病年齡、腺外器官受纍、免疫指標、自身抗體譜及預後方麵均不同于經典pSS,故可能是pSS的一種獨特亞型.
목적 통과분석항착사점항체(ACA)양성원발성간조종합정(pSS)환자적림상특점,제고대본병적진치수평.방법 회고성분석북경협화의원풍습면역과수치적60례ACA양성pSS환자(ACA양성조)적림상자료,병여동기수치적139례ACA음성pSS자(ACA음성조)상비교.결과 (1)ACA양성조발병년령만우ACA음성조[(48±11)세비(41±12)세,P=0.000],이2조재성별구성、구간、안간、진선활검양성솔차이원통계학의의.(2)간장수루시ACA양성조환자최상견적선외장기손해,기발생솔원고우ACA음성조(68.3%비37.0%,P=0.000),이신장수루(13.3%비30.9%,P=0.009)、신경계통수루(1.7%비11.5%,P=0.025)、고γ구단백혈증적발생솔(20.8%비45.7%,P=0.002)균저우ACA음성조.(3)2조항핵항체양성솔무차이,단ACA양성조이산점형면역형광표형최위상견(점61.7%).여ACA음성조상비,ACA양성조중항SSA항체、항SSB항체、항U1 RNP항체적양성솔감저(P＜0.05),이항선립체항체급기M2아형적양성솔증고(P＜0.05).(4)ACA양성조사망5례,기중3례사우위식관정맥곡장파렬출혈.결론 대ACA양성pSS자응경척간장수루.유우ACA양성pSS재발병년령、선외기관수루、면역지표、자신항체보급예후방면균불동우경전pSS,고가능시pSS적일충독특아형.
Objective To investigate the clinical manifestations, immunological features and prognosis of primary Sj(o)gren's syndrome(pSS) with anticentromere antibodies(ACA). Methods Sixty pSS patients with ACA in our hospital between 1985 and 2006 were screened retrospectively and compared with those without ACA. Results The mean age at the onset of pSS with ACA was higher than that of those without ACA[(48±11)yr vs(41±12)yr,P=0.000].There was no difference in sex ratio,dry mouth.dry eyes and positive salivary gland biopsy between the two groups(P＞0.05).Compared with those without ACA,patients with ACA presented a higher prevalence of liver involvement(68.3%vs 37.0%,P=0.000),while a lower prevalence of renal involvement(13.3%vs 30.9%,P=0.009), neuropathy(1.7% vs 11.5%,P=0.025)and hypergammaglobulinemia(20.8%vs 45.7%,P=0.002). The difference was not significant between the two groups in Raynaud's phenomenon,articular involvement,myositis,hematologic involvement,lung involvement,and thyroiditis. While both groups showed the same prevalence of antinuclear antibody(ANA),the patterns of ANA-IF were different and the discrete speckled pattern was the most freqent in patients with ACA and occurred in 61.7%. Different from those without ACA,patients with ACA presented a lower prevalence of anti-SSA,anti-SSB, rheumatoid factor, and antiU1 RNP,while showed a higher prevalence of antimitochondrial antibodies(AMA) and AMA-M2. The most frequent cause of death was the complications associated with cirrhosis,notably bleeding varices(3/5 cases). Conclusion Patients with ACA present a high risk of liver involvement. Because of the remarkable difference in the mean age of disease onset and also differences in systemic damage, immunological and antibody features, pSS with ACA may be a special subtype of pSS.
