Division of Allergy and Pulmonology, Department of Internal Medicine, Bucheon St. Mary's Hospital, The Catholic University of Korea School of Medicine, Seoul, South Korea.
Department of Hospital Pathology, The Catholic University of Korea School of Medicine, Seoul, South Korea.
Balkan Med J. 2017 Aug 4;34(4):374-377. doi: 10.4274/balkanmedj.2016.1061. Epub 2017 Apr 13.
Clinically, amyopathic dermatomyositis is a clinically distinct subgroup of dermatomyositis characterised by unique dermatological manifestations without muscle involvement. Clinically, amyopathic dermatomyositis is frequently associated with interstitial lung disease, which usually has a rapidly progressive, fatal clinical course. Although clinically, amyopathic dermatomyositis-related interstitial lung disease is well described, data on the histopathology of clinically, amyopathic dermatomyositis-interstitial lung disease are limited. Organising pneumonia and pulmonary vasculitis have rarely been reported.
A 54-year-old Korean woman presented with exertional dyspnoea and a dry cough. Chest computed tomography revealed subpleural ground-glass opacities suggesting interstitial lung disease, which was later pathologically confirmed to be a combination of organising pneumonia and pulmonary vasculitis. The patient improved markedly with prednisone treatment and has remained stable for a long time.
We hereby report a rare combination of organising pneumonia and pulmonary vasculitis in a patient with amyopathic dermatomyositis-interstitial lung disease.
临床上，无肌病性皮肌炎是皮肌炎的一个临床独特亚组，其特征为独特的皮肤表现而无肌肉受累。临床上，无肌病性皮肌炎常伴有间质性肺病，其通常具有快速进展、致命的临床病程。尽管临床上，无肌病性皮肌炎相关的间质性肺病已有很好的描述，但关于无肌病性皮肌炎-间质性肺病的组织病理学数据有限。机化性肺炎和肺血管炎很少有报道。
一名 54 岁韩国女性因劳力性呼吸困难和干咳就诊。胸部计算机断层扫描显示胸膜下磨玻璃影，提示间质性肺病，后来经病理证实为机化性肺炎和肺血管炎的组合。患者经泼尼松治疗后明显改善，长期保持稳定。
我们在此报告一例无肌病性皮肌炎-间质性肺病患者中罕见的机化性肺炎和肺血管炎的组合。
