特发性炎性肌病育龄女性患者的生育情况调查

Pregnancy investigation in idiopathic inflammatory myopathy in China

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.

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＆#39; 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 （x^2=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 （x^2=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.