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目的分析以肺部表现为首发症状的抗Jo-1抗体综合征的临床特征、治疗方法及预后,以提高对其认识。方法回顾性分析44例抗Jo-1抗体阳性的多发性肌炎/皮肌炎(PM/DM)患者的临床资料,重点分析9例以肺部表现为首发症状的抗Jo-1抗体综合征的肺部及肺外临床特征、治疗方案及预后。结果患者多以咳嗽、发热、气短等症状起病,肺部症状可出现于肌肉和皮肤表现之前,导致漏诊或误诊,胸部CT能够较及时发现肺间质病变;与非肺部表现起病的其他病例比较,除典型DM皮疹外,其余肺外临床特征如炎性肌病、多关节炎/关节痛、发热、技工手、雷诺现象等均无统计学差异;以激素联合免疫抑制剂治疗有效。结论以肺部表现为首发症状的抗Jo-1综合征患者常以非特异性肺部症状起病,应注意间质性肺病的诊断与鉴别诊断。激素联合免疫抑制剂治疗有效;治疗后磷酸肌酸激酶(CK)值的变化可能与疾病预后相关。
Objective To analyze the clinical features, treatment and prognosis of anti-Jo-1 antibody syndrome with lung manifestations as the first symptom to improve its understanding. Methods The clinical data of 44 patients with polymyositis / dermatomyositis (PM / DM) positive for anti-Jo-1 antibody were retrospectively analyzed. Nine cases of anti-Jo-1 antibody syndrome with pulmonary manifestations as the first symptom were analyzed. Of lung and extrahepatic clinical features, treatment options and prognosis. Results Most patients with cough, fever, shortness of breath and other symptoms, pulmonary symptoms may appear in the performance of the muscles and skin before, resulting in missed diagnosis or misdiagnosis, chest CT can be more timely detection of interstitial lung disease; and non-pulmonary manifestations of onset Other cases, in addition to the typical DM rash, the rest of the extra-pulmonary clinical features such as inflammatory myopathy, arthritis / joint pain, fever, mechanic hand, Renault phenomenon were no significant difference; hormone combination immunosuppressive therapy effective . Conclusion Patients with anti-Jo-1 syndrome, whose first manifestation is pulmonary manifestations, often start with nonspecific pulmonary symptoms and should pay attention to the diagnosis and differential diagnosis of interstitial lung disease. Hormone combination immunosuppressive therapy is effective; the change of creatine kinase (CK) value after treatment may be related to the prognosis of the disease.