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Correlation between disease activity and serum ferritin in clinically amyopathic dermatomyositis with rapidly-progressive interstitial lung disease: a case report

机译:临床肌萎缩性皮肌炎合并快速进展性间质性肺疾病的疾病活动性与血清铁蛋白的相关性:病例报告

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BackgroundClinically amyopathic dermatomyositis with anti-Melanoma Differentiation-Associated gene 5 (MDA5) antibody often presents with severe interstitial lung disease. Although serum ferritin level is known to reflect interstitial lung disease activity, there are few case reports describing this association. Case presentationA 58-year-old man was referred to our outpatient clinic with a 3-week history of cough and respiratory distress. He had erythema over the V area of the neck and a Gottron’s sign. Chest computed tomography revealed diffuse ground-glass opacities and reticular shadows in both lungs. Test for anti-MDA5 antibody was positive. After admission, he received triple combination therapy (methylprednisolone pulse therapy, tacrolimus, and cyclophosphamide). However, his respiratory condition worsened as the serum ferritin level increased. Despite no apparent deterioration on chest radiography, he ultimately died due to respiratory failure. ConclusionsIn this case, triple combination therapy was not effective for the patient’s respiratory condition. The serum ferritin level was correlated with disease activity and was more useful than chest radiography for monitoring clinical status.
机译:背景带有抗黑素瘤分化相关基因5(MDA5)抗体的临床肌病性皮肌炎通常表现为严重的间质性肺疾病。尽管已知血清铁蛋白水平可反映间质性肺疾病的活动,但很少有病例报道描述这种关联。病例介绍一名58岁的男子被转诊至我们的门诊,有3周的咳嗽和呼吸窘迫病史。他的颈部V区有红斑,并有Gottron的体征。胸部计算机断层扫描显示双肺弥漫性有毛玻璃状混浊和网状阴影。抗MDA5抗体测试为阳性。入院后,他接受了三联疗法(甲基泼尼松龙脉冲疗法,他克莫司和环磷酰胺)。然而,他的呼吸状况随着血清铁蛋白水平的升高而恶化。尽管胸部X光片无明显恶化,但他最终因呼吸衰竭而死亡。结论在这种情况下,三联疗法对患者的呼吸状况无效。血清铁蛋白水平与疾病活动相关,并且比胸部X光片对监测临床状况更有用。

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