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目的分析33例以间质性肺疾病(ILD)为首发表现的皮肌炎(DM)患者的资料。方法33例以ILD为首发表现的DM患者分为死亡组(12例)和存活组(21例),比较两组的临床表现、实验室检查及影像学结果。结果死亡组患者表现为急性/亚急性发病的病例高于存活组(41.7%vs.4.8%)(P<0.05)。死亡组与存活组病程(3个月vs.36个月)、入院时血沉[(60.33±26.52)mm/h vs.(39.52±22.27)mm/h]和乳酸脱氢酶[(804.85±470.71)U/L vs.(357.09±223.51)U/L]、入院时动脉氧分压[(56.75±13.90)mmHg vs.(86.71±14.99)mmHg]的差异均有统计学意义(P<0.05);而两组发病年龄、性别构成比、典型皮疹、技工手、骨骼肌受累、DM发生时间、入院时肌酸激酶、C反应蛋白、抗JO-1、抗核抗体阳性率和高分辨CT成像均无统计学差异(P>0.05)。结论以ILD起病的DM患者缺乏典型的皮肤和肌肉改变,应引起临床医生的高度重视。
Objective To analyze the data of 33 cases of dermatomyositis (DM) with interstitial lung disease (ILD) as the first manifestation. Methods Thirty-three DM patients with ILD as the first onset were divided into death group (n = 12) and survival group (n = 21). The clinical manifestations, laboratory tests and imaging findings were compared between the two groups. Results In the death group, the incidence of acute / subacute morbidity was higher than that of the survivors (41.7% vs 4.8%) (P <0.05). The duration of death (3 months vs.36 months) in death group and survivor group was significantly higher than those in survival group (60.33 ± 26.52 mm / h vs. 39.52 ± 22.27 mm / h) and lactate dehydrogenase [(804.85 ± 470.71 ) (U / L vs. (357.09 ± 223.51) U / L]. There were significant differences in arterial partial pressure of oxygen between the two groups (56.75 ± 13.90 mmHg vs. 86.71 ± 14.99 mmHg) The incidence of DM, C-reactive protein, anti-JO-1, antinuclear antibody positive rate and high-resolution CT imaging No statistical difference (P> 0.05). Conclusion The lack of typical skin and muscle changes in DM patients with ILD onset should be highly regarded by clinicians.