Histopathological evidence of small-vessel vasculitis within the skin and lungs associated with interstitial pneumonia in an adult patient with dermatomyositis
Histopathological evidence of small-vessel vasculitis within the skin and lungs associated with interstitial pneumonia in an adult patient with dermatomyositis
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DOI：
10.1111/j.1365-2230.2008.02729.x
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发表时间：
2008-07-01
影响因子：
4.1
通讯作者：
Soma, Y.
中科院分区：
文献类型：
--
作者：
Kawakami, T.;Mizoguchi, M.;Soma, Y.
皮肌炎（DM）由于与恶性肿瘤或间质性肺炎（IP）相关的并发症，通常预后不良。糖尿病患者的皮肤病变和肺毛细血管炎的组织病理学小血管炎是罕见的。一名64岁的女性被诊断为糖尿病的基础上存在的四肢轻瘫，典型的向日皮疹，Gottron丘疹，血清水平增加的肌肉酶和典型的肌肉活检结果。她还患有相关IP，随后发展为快速进展性疾病。我们在她的血清中发现了高滴度的Krebs von den Lunge（KL）-6。血清KL-6水平的测量被广泛接受为监测间质性肺疾病活动的诊断测试。皮肤和肺部相关特征的组织学检查显示小血管炎。我们建议，调查KL-6水平在糖尿病患者和相关的条件下，应进行，以确定是否提高KL-6水平是有用的预测结果或严重程度的各种功能。
Dermatomyositis (DM) often has a poor prognosis, due to complications associated with malignancy or interstitial pneumonia (IP). It is uncommon to find histopathological small-vessel vasculitis within cutaneous lesions and pulmonary capillaritis in patients with DM. A 64-year-old woman was diagnosed with DM based on the presence of quadriparesis, typical heliotropic rash, Gottron's papules, increased serum levels of muscle enzymes and typical muscle biopsy findings. She also had associated IP, which subsequently developed into a rapidly progressive condition. We found a high titre of Krebs von den Lunge (KL)-6 in her serum. Measurement of serum KL-6 level is widely accepted as a diagnostic test to monitor the activity of interstitial lung diseases. Histopathological examinations of the cutaneous and lung-associated features showed small-vessel vasculitis. We suggest that investigation of KL-6 levels in patients with DM and associated conditions should be carried out to determine if raised KL-6 levels are useful at predicting outcome or severity of various features.