78例抗合成酶综合征临床分析

Clinical analysis of seventy eight patients with antisynthetase syndrome

目的 :总结不同亚型抗合成酶综合征(antisynthetase syndrome,ASS)及ASS合并快速进展性间质性肺疾病(rapidly progressive interstitial lung diseases,RP-ILD)的特点。方法:回顾性分析78例ASS患者临床资料,比较不同亚型ASS临床表现及实验室检查,并着重分析ASS合并RP-ILD的临床特点、治疗及转归。结果:78例ASS患者分为5组:抗Jo-1抗体阳性组33例(42.3%),抗PL-7抗体阳性组15例(19.2%),抗PL-12抗体阳性组16例(20.5%),抗EJ抗体阳性组11例(14.1%),抗OJ抗体阳性组3例(3.8%)。ASS合并间质性肺疾病(interstitial lung diseases,ILD)发生率高达84.6%。ILD中有7例(9%)为RP-ILD,其中,2例抗PL-12抗体阳性(12.5%),4例抗Jo-1抗体阳性(12.1%),1例抗PL-7抗体阳性(6.6%)。7例RP-ILD患者中,6例为非特异型间质性肺炎(non-specific interstitial pneumonia,NSIP),1例为寻常型间质性肺炎(usual interstitial pneumonia,UIP)。6例RP-ILD类型为NSIP的患者使用甲泼尼龙联合环磷酰胺,5例经治疗后病情缓解,1例失访。1例RP-ILD类型为寻常型间质性肺炎的患者死亡。结论:ASS临床表现中,ASS合并ILD发生率最高。抗PL-12抗体阳性RP-ILD发生率较高。对以NSIP为主的RP-ILD,可予糖皮质激素联合免疫抑制剂治疗。

Objective:To summarize the characteristics of different subtypes of antisynthetase syndrome(ASS) and ASS complicated with rapidly progressive interstitial lung diseases(RP-ILD). Methods:Clinical data of 78 patients with ASS were retrospectively analyzed,clinical manifestations and laboratory test results of different subtypes of ASS were compared,and clinical characteristics,treatment and outcome of ASS combined with RP-ILD were emphatically analyzed. Results:A total of 78 ASS patients were divided into five groups:the anti-Jo-1 antibody positive group was 33 patients(42.3%),the anti-PL-7 positive group was 15 patients(19.2%),the anti-PL-12 positive group was 16 patients(20.5%),the anti-EJ positive group was 11 patients(14.1%) and anti-OJ positive group was 3 patients(3.8%). The incidence of ASS complicated with interstitial lung diseases(ILD) was 84.6%. Among ILD,RP-ILD was found in seven patients(9%),of which two were positive for anti-PL-12 antibody(12.5%),four were positive for anti-JO-1 antibody(12.1%),and one was positive for anti-PL-7 antibody(6.6%). Six of the seven RP-ILD patients had non-specific interstitial pneumonia(NSIP) and one had usual interstitial pneumonia(UIP). Six patients with NSIP in RP-ILD type were treated by methylprednisolone combined with cyclophosphamide,five of whom were relieved after treatment and one was lost to follow-up. One patient with UIP of RP-ILD type was passed away. Conclusion:Among the clinical manifestations of ASS,the incidence of ASS with ILD is the highest. The incidence of RP-ILD is the highest in the anti-PL-12 positive group. NSIP main in the RP-ILD can be treated with glucocorticoid combined with immunosuppressive therapy.