目的 调查PM和DM等特发性炎性肌病(ⅡM)患者的生育情况,了解ⅡM疾病活动性和治疗对胎儿的影响,并探讨妊娠与ⅡM发病的相关性.方法 制定调查项目,对北京协和医院2007-2013年间住院的ⅡM育龄女性(22～53岁)患者75例进行问卷调查,包括患者的生育情况、妊娠过程中和分娩后疾病的活动性、用药情况和胎儿的结局,采用x2检验比较不同ⅡM疾病活动性对妊娠患者和胎儿的影响,以及妊娠对ⅡM的影响.结果 75例患者(19例PM,56例DM)中62例共计妊娠144次,其中有18次妊娠在ⅡM发病后出现,还有2例ⅡM患者在妊娠过程中发病,2例在分娩或流产后1个月内发病.ⅡM发病后妊娠和ⅡM发病前妊娠的异常妊娠比例分别为38.9％(7/18)、10.7％(13/122),二者差异有统计学意义(x2=10.21,P=0.001).其中ⅡM发病后妊娠的18次,7次妊娠病情有活动,异常妊娠发生率71.4％(5/7),包括2例自然流产、1例胎停育、2例早产;而病情相对稳定的11次妊娠,仅发生1例早产和1例胎儿停育,异常妊娠发生率为18.2％(2/11),二者差异有统计学意义(x2=5.103,P=0.024).2例ⅡM发病后妊娠的患者妊娠前病情稳定,妊娠过程中出现病情活动,其中1例患者妊娠2次都出现皮疹,1次胎儿停育,1次早产(孕33+4周),另1例患者在妊娠5个月时出现肌无力症状,胎儿发生早产(孕36+3周).1例于分娩后18d发病,婴儿正常,1例于人工流产后1个月内发病.ⅡM合并妊娠患者中,病情稳定者无治疗或口服小剂量激素维持,病情活动者则多口服大中剂量泼尼松(30～60 mg/d)治疗,或联用羟氯喹(0.2 g,每日2次),有2例联用静脉用免疫球蛋白(IVIG),患者病情均得到缓解.结论 ⅡM患者合并妊娠时容易导致自然流产、胎儿宫内停育、早产等,和ⅡM疾病活动性呈正相关.ⅡM活动期合并妊娠时,对患者和胎儿最有效和安全的治疗可能是IVIG,同时需联合中等或大剂量糖皮质激素.
目的 調查PM和DM等特髮性炎性肌病(ⅡM)患者的生育情況,瞭解ⅡM疾病活動性和治療對胎兒的影響,併探討妊娠與ⅡM髮病的相關性.方法 製定調查項目,對北京協和醫院2007-2013年間住院的ⅡM育齡女性(22～53歲)患者75例進行問捲調查,包括患者的生育情況、妊娠過程中和分娩後疾病的活動性、用藥情況和胎兒的結跼,採用x2檢驗比較不同ⅡM疾病活動性對妊娠患者和胎兒的影響,以及妊娠對ⅡM的影響.結果 75例患者(19例PM,56例DM)中62例共計妊娠144次,其中有18次妊娠在ⅡM髮病後齣現,還有2例ⅡM患者在妊娠過程中髮病,2例在分娩或流產後1箇月內髮病.ⅡM髮病後妊娠和ⅡM髮病前妊娠的異常妊娠比例分彆為38.9％(7/18)、10.7％(13/122),二者差異有統計學意義(x2=10.21,P=0.001).其中ⅡM髮病後妊娠的18次,7次妊娠病情有活動,異常妊娠髮生率71.4％(5/7),包括2例自然流產、1例胎停育、2例早產;而病情相對穩定的11次妊娠,僅髮生1例早產和1例胎兒停育,異常妊娠髮生率為18.2％(2/11),二者差異有統計學意義(x2=5.103,P=0.024).2例ⅡM髮病後妊娠的患者妊娠前病情穩定,妊娠過程中齣現病情活動,其中1例患者妊娠2次都齣現皮疹,1次胎兒停育,1次早產(孕33+4週),另1例患者在妊娠5箇月時齣現肌無力癥狀,胎兒髮生早產(孕36+3週).1例于分娩後18d髮病,嬰兒正常,1例于人工流產後1箇月內髮病.ⅡM閤併妊娠患者中,病情穩定者無治療或口服小劑量激素維持,病情活動者則多口服大中劑量潑尼鬆(30～60 mg/d)治療,或聯用羥氯喹(0.2 g,每日2次),有2例聯用靜脈用免疫毬蛋白(IVIG),患者病情均得到緩解.結論 ⅡM患者閤併妊娠時容易導緻自然流產、胎兒宮內停育、早產等,和ⅡM疾病活動性呈正相關.ⅡM活動期閤併妊娠時,對患者和胎兒最有效和安全的治療可能是IVIG,同時需聯閤中等或大劑量糖皮質激素.
목적 조사PM화DM등특발성염성기병(ⅡM)환자적생육정황,료해ⅡM질병활동성화치료대태인적영향,병탐토임신여ⅡM발병적상관성.방법 제정조사항목,대북경협화의원2007-2013년간주원적ⅡM육령녀성(22～53세)환자75례진행문권조사,포괄환자적생육정황、임신과정중화분면후질병적활동성、용약정황화태인적결국,채용x2검험비교불동ⅡM질병활동성대임신환자화태인적영향,이급임신대ⅡM적영향.결과 75례환자(19례PM,56례DM)중62례공계임신144차,기중유18차임신재ⅡM발병후출현,환유2례ⅡM환자재임신과정중발병,2례재분면혹유산후1개월내발병.ⅡM발병후임신화ⅡM발병전임신적이상임신비례분별위38.9％(7/18)、10.7％(13/122),이자차이유통계학의의(x2=10.21,P=0.001).기중ⅡM발병후임신적18차,7차임신병정유활동,이상임신발생솔71.4％(5/7),포괄2례자연유산、1례태정육、2례조산;이병정상대은정적11차임신,부발생1례조산화1례태인정육,이상임신발생솔위18.2％(2/11),이자차이유통계학의의(x2=5.103,P=0.024).2례ⅡM발병후임신적환자임신전병정은정,임신과정중출현병정활동,기중1례환자임신2차도출현피진,1차태인정육,1차조산(잉33+4주),령1례환자재임신5개월시출현기무력증상,태인발생조산(잉36+3주).1례우분면후18d발병,영인정상,1례우인공유산후1개월내발병.ⅡM합병임신환자중,병정은정자무치료혹구복소제량격소유지,병정활동자칙다구복대중제량발니송(30～60 mg/d)치료,혹련용간록규(0.2 g,매일2차),유2례련용정맥용면역구단백(IVIG),환자병정균득도완해.결론 ⅡM환자합병임신시용역도치자연유산、태인궁내정육、조산등,화ⅡM질병활동성정정상관.ⅡM활동기합병임신시,대환자화태인최유효화안전적치료가능시IVIG,동시수연합중등혹대제량당피질격소.
Objective We investigated female patients with idiopathic inflammatory myopathy (ⅡM),including polymyositis (PM) and dermatomyositis (DM) to understand if the activity and treatment of ⅡM affected the fetus outcomes as well as the correlation between pregnancy and the pathogenesis of ⅡM.Methods Questionnaires were designed,including patients' fertility,the activity and treatment of ⅡM during pregnancy and after childbirth,and the fetal outcomes.The questionnaires were conducted on 75 female patients (22-53 years old),who once hospitalized at Peking Union Medical College Hospital from 2007 to 2013.The mutual effect between the ⅡM disease activity,pregnancy and the fetus were compared by Chi square.Results Seventy-five patients (19 PM,56 DM) had 144 pregnancies,but only 18 of them became pregnant after the onset of the disease.There were two patients had onset of DM during pregnancy,and two patients suffered from DM after delivery or abortion within one month.The rates of abnormal pregnancy in patients with pregnancy before the onset of ⅡM was significantly lower than those with pregnancy after the onset of ⅡM (x2=10.21,P=0.001).Of the 18 pregnancies after the onset of ⅡM,7 had active ⅡM during pregnancy,3 of them flareddue to pregnancy.The 5 abnormal pregnancies (5/7) were seen in seven pregnancies totally,including two spontaneous abortion,one embryo diapause and two premature births.While of the other 11 pregnanciesin stable condition had only 2 abnormal pregnancies (2/11),including one fetus ended prematurely and one embryo diapause.The fetal outcomes were significantly related to the status of illness (x2=5.103,P=0.024).Two patients became pregant after the onset of the disease,both of them had stable disease before pregnancy.However,the disease became active during the pregnancies.One of them flared with rash during two pregnancies.The fetal end in embryo diapause and premature birth (33+4 weeks),respectively.Another flared with muscle weakness at five months of gestation.The fetal ended in premature birth (36+3 weeks).Two patients had onset of DM within one month following the delivery and voluntary abortion.Patients with active disease had taken medium to large doses of prednisone (30-60 mg) orally,in combination with hydroxychloroquine (0.2 bid) or without.In addition,two patients administrated with intravenous gamma globulin (IVIG) at low dose,whose illness condition had been well controlled.Conclusion Pregnancies after the onset of ⅡM tend to end with spontaneous abortion,embryo diapause,premature birth.The fetal outcomes are significantly related to disease activity.When ⅡM flared during pregnancy,IVIG might be the most effective and safe treatment,combined with corticosteroids.
