抗合成酶抗体综合征18例临床分析

Antisynthetase syndrome:Clinical analysis in 18 cases

目的:探讨不同抗氨酰基tRNA合成酶抗体介导的抗合成酶抗体综合征(ASS)的临床及实验室资料异同。方法:收集弋矶山医院住院的18例ASS患者的临床及实验室资料,分析其不同亚型的临床特点。结果:18例患者中抗Jo-1抗体阳性8例,抗PL-7抗体阳性7例,抗PL-12抗体阳性1例,抗EJ抗体阳性2例。抗PL-7抗体阳性组临床发热及铁蛋白升高较抗Jo-1抗体阳性组显著,差异有统计学意义(P<0.05)。18例ASS并发快速进展型间质肺炎(RPILD)5例(27%),其中抗EJ抗体阳性2例,抗PL-7抗体阳性3例,5例均检出高滴度抗Ro-52抗体。RPILD组较non-RPILD组更易出现发热,差异有统计学意义(P=0.009),RPILD组AST、LDH、TnI、ESR及血清铁蛋白(ferritin)均较non-RPILD组高,差异有统计学意义(P<0.05)。抗Jo-1抗体阳性不易合并RPILD,差异有统计学意义(P=0.044)。结论:不同ASS亚型临床表现及严重程度不一,各种抗体指标有一定差异,认识不同亚型的特点有助于指导治疗及判断预后。

Objective:To observe the clinical and laboratory similarities and differences of different subtypes of antisynthetase syndrome(ASS)mediated by diverse aminoacyl-tRNA synthase antibody.Methods:The clinical data and laboratory findings were obtained from 18 ASS patients admitted to and treated in our department to analyze the clinical characteristics of different subtypes of ASS.Results:In the 18 patients,8 were positive for anti-Jo-1 antibody,7 were positive for anti-PL-7 antibody,1was positive for anti-PL-12 antibody,and 2 were positive for anti-EJ antibody.Patients in the anti-PL-7 antibody-positive group had significantly higher incidence of clinical fever and ferritin level than those in the anti-Jo-1 antibody-positive group.The difference was significant(P<0.05).Five of the 18 patients were complicated with rapidly progressive interstitial pneumonia(RPILD),in whom 2 were positive for EJ antibody,and 3 were positive PL-7 antibody.Higher anti-Ro-52 antibody titer was seen in the 5 patients with RPILD.Patients in the RPILD group were prone to fever than those in the non-RPILD group(P=0.009),and the level of serum AST,LDH,TnI,ESR and ferritin was higher in the RPILD group than in the non-RPILD group(P<0.05).Patients with positive anti-Jo-1 antibody were less susceptible to RPILD(P=0.044).Conclusion:The clinical pictures and severity are diverse in patients of subtypes of ASS with distinct laboratory findings.Therefore,knowledge of the characteristics of subtype ASS can be conductive to the treatment plan and estimation of the prognosis of this condition.