抗JO-1抗体阴性皮肌炎并急性肺间质病变1例

Dermatomyositis of Non-Anti-JO-1 Antibodies with Acute Interstitial Lung Disease:a case report and literature review

肺间质病变是多发性肌炎/皮肌炎的常见并发症,预后不良且病死率高,发病机制目前仍不清楚,治疗上仍以激素为主,尚缺乏有效的治疗方法。抗JO-1抗体阴性皮肌炎并急性肺间质病变临床表现的非特异性和多样性,给临床诊断带来极大困难。本文报道1例患者的临床表现、影像学特点、诊断过程和依据、治疗经过及随访,并结合文献对抗JO-1抗体阴性皮肌炎并急性肺间质病变病因及发病特点、诊断依据和症状相似疾病鉴别进行了讨论。提高临床医生对此类疾病的认识,提高诊断水平,有助于判断预后。

Interstitial lung disease is a common complication of polymyositis（PM）/Dermatomyositis（DM）,with bad outcomes and significant mortality.The pathogenesis remains unclear.Steroids remain the first-line therapy for DM/PMILD.However there is a lack of effective treatment.Clinical manifestations of Dermatomyositis of non-Anti-JO-1 antibodies with acute interstitial lung disease are non-specificity and diversity,which creates great difficulties for clinical diagnosis.A case diagnosed in our hospital was reported,including clinical manifestations,diagnosis,treatment and follow up.It was discussed about pathogenesis,clinical characters,diagnostic creteria and identify diseases with similar symptoms in this article accroding to literature research.Improving the ability of clinician to identify this kind of disease which will contribute to evaluate the prognosis.