临床无肌病性皮肌炎合并快速进展间质性肺疾病患者铁蛋白与疾病活动度二例报告及文献复习

The disease activity and serum ferritin in patients with clinically amyopathic dermatomyositis of rapidly-progressive interstitial lung disease: a report of two cases and literature review

目的通过分析2例临床无肌病性皮肌炎(CADM)合并快速进展间质性肺疾病(RP-ILD)死亡病例的临床资料,总结诊治经验。方法对2015年1月—2018年1月2例CADM合并RP-ILD死亡病例的临床表现、实验室及影像学检查、诊断及治疗进行回顾性分析,总结其临床特点、治疗方案及死亡原因。结果 2例CADM合并RP-ILD死亡病例抗黑素瘤分化相关基因5(MDA5)抗体结果均阳性,入院时2例患者的SF水平均有明显升高,但胸部CT仅提示散在炎症。随着患者的呼吸情况逐渐加重,SF水平进行性升高,胸部影像学表现进展,给予激素、他克莫司及环磷酰胺3种药物的联合治疗,2例患者分别于发病66 d、61 d因呼吸衰竭而死亡。结论高SF水平反映了抗MDA5抗体相关的DM患者ILD的病情恶化。在判断疾病的进展方面,SF水平比胸部CT检查具有更早的诊断价值。

Objective To summarize the experience of diagnosis and treatment of clinical data of 2 dead cases with clinically amyopathie dermatomyositis （CADM） and rapidly-progressive interstitial lung disease （RP-ILD）. Methods The clinical manifestations, laboratory and imaging examination resuhs, diagnosis and treatment of 2 dead cases with CADM of RP-ILD treated in our hospital from February, 2015 to January, 2018 were analyzed retrospectively. The clinical characteristics, treatment plan and cause of death were summarized. Results The 2 dead cases with CADM of RP-ILD were tested positive for the anti-melanoma differentiation-associated gene 5 （MDA5） antibody. The SF level was significantly increased in the 2 patients at admission, but the CT scan-chest only indicated the mild inflammation. With the progressively exacerbation of the patient respiration, the SF level was increased progressively, and the chest imaging progressed. The combination of hormone, tacrolimus and cyclophosphamide were given for treatment in the two patients. The 2 patients died of respiratol7 failure at 66 days and 61 days respectively. Conclusion The high SF level reflects the deterioration in patients with DM of ILD associated with anti-MDA5 antibody. In determining the progression of the disease, the SF level has earlier diagnostic value than chest CT scan.