目的 总结慢性移植物抗宿主病(c-GV HD)相关性多发性肌炎(PM)的临床特点,提高临床对该病的认识.方法 以"chronic graft versus host disease"和"polymyositis"为关键词,检索 PubMed和Medline数据库;以"慢性移植物抗宿主病"和"多发性肌炎"为关键词,检索中国期刊全文(CNKI)、万方数据、维普数据库.检索时间设为1990-01—2017-08.筛选有关c-GV HD相关性PM 研究的文献,汇总分析患者的临床特点.结果 共检索到符合纳入标准的英文文献19篇,中文文献3篇,共44例患者.其中男28例,女16例,移植年龄4~67岁,平均(32.48 ± 14.57)岁.发病距离移植时间为4~68个月,中位数19个月,四分位数间距为22个月;出现肌无力44例(100%),肌痛29例(66.67%),肌肉萎缩5例(11.36%),呼吸肌无力3例(6.82%),腱反射减弱2例(4.55%).肌酶升高占90.48%(38/42);乳酸脱氢酶(LDH)升高占92.59%(25/27例);抗核抗体阳性35.71%(10/28);肌电图检查呈肌源性损害占82.86%(28/35);肌肉活检异常96.97%(32/33).44例患者均给予糖皮质激素、环孢素等免疫抑制剂治疗,反应时间为1~7个月,中位数3个月,四分位数间距为4个月,其中6例患者死亡.结论 c-GV HD相关性PM临床表现有其自身特点,相对于散发性PM 来说,其较少出现肌痛,可出现腱反射减弱,抗JO-1抗体阳性检出率低,病理活检可能并无炎性细胞浸或血管炎表现,HLADR52型出现率低等;而相对于c-GV HD,其皮肤、肺部、心脏、关节受累的概率更高.早期诊断、治疗可预防疾病进展所致的严重后果.%Objective To summarize the clinical features of chronic graft-versus-host disease(c-GVHD)-related polymyositis(PM). Methods With"chronic graft versus host disease"and"polymyositis"as key words,we searched the literatures from January 1990 to August 2017 in the PubMed,Medline,CNKI, WanFang,VIP journals database,the clinical data of patients with c-GVHD -related PM were collected and analyzed. Results A total of 19 English and 3 Chinese literatures were retrieved,including 44 patients. There were 28 males and 16 females,transplant age were 4-67 years old,mean age was(32.48 ± 14.57)years old;the onset time from transplantation was 4-68 months,with median of 19(22)months. 44(100%)cases had severe muscle weakness,29(66.67%)cases had myalgia,5(11.36%)cases had muscle atrophy,3 (6.82%)cases had respiratory muscle weakness,and 2(4.55%)cases had decreased tendon reflex. 38/42 (90.48%)patients had elevated muscle enzymes;lactate dehydrogenase(LDH)increased in 25/27(92.59%) cases;anti nuclear antibody was positive in 10/28(35.71%)cases;EMG showed myogenic changes in 28/35 (82.86%)cases;32/33(96.97%)cases had abnormal muscle pathological examination through biopsy. 44 patients were given corticosteroid,cyclosporine or other immunosuppressive agents,the duration of treatment was 1-7 months, with median of 3(4)months,6 patients died. Conclusions The clinical manifestation of c-GVHD-related PM has its own characteristics,and early diagnosis and treatment can prevent the progression of the disease.
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