抗MDA5抗体和抗SSA/Ro52抗体双阳性皮肌炎的临床分析(附5例报告及文献复习)

Analysis of Clinical Characteristics in Dermatomyositis Patients with Anti-MDA5 and Anti-SSA/Ro52 Antibodies(Reports of Five Cases and Literature Review)

目的探讨抗MDA5抗体和抗SSA/Ro52抗体双阳性皮肌炎的临床特征及治疗。方法回顾性分析5例抗MDA5抗体和抗SSA/Ro52抗体双阳性皮肌炎患者的临床资料,结合文献复习进行讨论。结果5例患者中男性1例、女性4例。主要临床表现为典型皮疹及肢体无力,可伴有呼吸困难、关节症状及口干眼干等症状,均诊断为抗MDA5抗体和抗SSA/Ro52抗体双阳性皮肌炎,且合并间质性肺炎;患者平均发病年龄(62.2±6.18)岁,平均病程(4.2±1.64)个月;1例患者血清肌酸激酶升高(472 U·L^-1);诊断明确后5例患者均接受了糖皮质激素与免疫抑制剂联合治疗。结论抗MDA5抗体与抗SSA/Ro52抗体双阳性的皮肌炎临床主要表现为典型皮疹,肢体无力、呼吸困难、关节症状及口干眼干等症状常见,血清肌酸激酶升高不明显,易合并间质性肺病;糖皮质激素与免疫抑制剂联合治疗为主要方案。预后不良。

Aim To explore the clinical characteristics and treatment of dermatomyositis patients with anti-MDA5 and anti-SSA/Ro52 antibodies.Methods The clinical symptoms,biochemiscal and electrophysiological examination,imaging examination,and pathological features on muscle biopsy of 5 cases were retrospectively analyzed.Related literature was reviewed.Results A total of 5 patients were recruited in this study,including 1 male and 4 females.Clinical symptoms included skin rashes and weakness.Some patients suffered from dyspnea,arthritis,dry mouth/dry eyes as well.Five patients had interstitial lung disease.The mean age of symptom onset was(62.2±6.18)years old.The mean course was(4.2±1.64)months.Serum creatine kinase increased in one patient(472 U·L^-1).Five patients were treated with glucocorticoid combined with immunosuppressant.Conclusion The main clinical manifestations of dermatomyositis patients with anti-MDA5 and anti-SSA/Ro52 antibodies were typical skin rashes,weakness,dyspnea,arthritis,dry mouth/dry eyes,etc.The elevation of serum creatine kinase was not obvious,and it was easy to combine with interstitial lung disease.Glucocorticoid combined with immunosuppressant was the main treatment,with poor prognosis.