原发性干燥综合征继发肺间质病变的临床特点

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1、，·326·生华风湿病学盔_2010年5月第l4卷第5期ChinJRheum

2、a101，Mav2010．V01．14，N0．5·临床研究·原发性干燥综合征继发肺问质病变的临床特点雷玲赵铖米存东钟小宁【摘要】目的探讨原发性干燥综合征(pSS)继发肺问质病变(ILD)的临床、实验室及病理特点。方法收集我院2006-2008年住院的15例pSS—ILD的临床资料，与同期住院的18例psS无1LD进行比较：其中8例pSS—ILD有随访，观察治疗后胸部高分辨CT(HRCT)的变化情况。结果①pSS—ILD的发病年龄[(57~11)岁]比Dss无IL

3、D[(43~11)岁]晚(R0．o1)。6例pSS—ILD患者首发症状为咳嗽、咯痰、胸闷和(或)活动后气促，出现呼吸道临床表现、心脏受累及肺动脉高压(PAH)较oSS无ILD明显增多(P<0．01)。②pSS—ILD血清免疫球蛋白G(IgG)水平[(23+8)g／L]比pSS无ILD[(17~5)玑]明显增高(P<0．01)。③pSS—ILD胸部HRCT常见网格状、条索状、结节状影，8例胸膜受累，蜂窝状改变常见(5例)，3例出现PAH。8例患者治疗6个月，4例胸部HRCT吸收好转，2例无明显变化，2例病变进展。④病理常见肺泡壁及肺泡组织局

4、灶性纤维化，肺泡腔内有炎细胞浸润，间质多灶性淋巴细胞浸润，1例患者符合非特异性间质性肺炎(NSIP)改变。结论pSS—ILD的发病年龄较大，易出现呼吸道临床表现，心脏受累及继发PAH，血清IgG水平高。1／3患者HRCT可见蜂窝状改变，多数患者治疗后病变吸收好转。病理可见NSIP改变。【关键词】干燥综合征；肺疾病，问质性；临床特点ClinicalfeaturesofinterstitiallungdiseaseinpatientswithprimarySj6gren。ssyndromeLEILingZHA0Chert，MjCun-dong

5、．ZHONGXicm—ning．Departmen!ofRheumatology，theFirstAfiliatedHospital,GucmgxiMedicalUniversity,Ncmning530021，ChinaCorrespondingauthor：ZHOⅣGXlap～ning．EmZ：XnzhonflOl@sina．conl【Abstract10bjectivcToexploretheclinical，laboratory，andpathologicmanifestationsoftheinterstitiallungdis

6、ease(ILD)associatedwithprimarySjSgrenssyndrome(pSS．pSS一!LD)．MethodsClin-icaldataof15patientswithpSS—ILDadmittedtoour·hospitalfr0m2oo6to20o8werecollectedandthedifferentfeatureswerecomparedwith18patientswithpSSwithoutILD．EightpatientswithpSS—ILDwerefollowedupandobservedthec

7、hangesofhigh—resolutioncomputedtomography(HRCT)．ResultsTheageatonsetwaslaterinpSS—ILDl(57+l1)yearsJthaninpSSwithoutILDl(43~11)yearsJ(P<0．01)．Theinitia1symptomsinsixofpatientswithpSS—ILDwerecough，expectoration，chestdistressordyspneauponexe~ion．Therespiratoryelinicalmanifes

8、tations．circulationsysteminvolvementandpulmonaryarteryhypertension(PAH)weremoreeommoninpSS—ILDthaninpSSwithoutILD(P<0．01)．ComparedwithpatientswithpSSwithoutILD．patientswithpSS-I1DhadsignificantlyhigherserumIgGlevel(P<0．01)．ThecommonfindingsofHRCTincludedreticularchanges，i

9、regularlinearhyper—attenuatingareas，andnodules．Pleuralinvolvementwasfoundin8patients．honeyeombch