与无肌病性皮肌炎相关的预后不良的非特异性间质性肺炎。
Nonspecific interstitial pneumonia with poor prognosis associated with amyopathic dermatomyositis.
作者信息
Sakamoto Noriho, Mukae Hiroshi, Fujii Takeshi, Yoshioka Sumako, Kakugawa Tomoyuki, Yamaguchi Hiroyuki, Hayashi Tomayoshi, Kohno Shigeru
机构信息
Second Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki.
出版信息
Intern Med. 2004 Sep;43(9):838-42. doi: 10.2169/internalmedicine.43.838.
Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the lack of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by interstitial pneumonia that shows a rapid progressive course associated with a poor prognosis. We report a 49-year-old patient who presented with nonspecific interstitial pneumonia (NSIP) associated with ADM. The patient failed to respond to prednisolone and immunosuppressive therapy and died. Although idiopathic NSIP is known to have a better prognosis, NSIP in ADM could be a fatal disease. Therefore, we should appropriately treat interstitial pneumonia in ADM even if it is NSIP.
无肌病性皮肌炎（ADM）是皮肌炎的一种临床亚型，其特征为无肌无力且肌肉酶水平正常。ADM有时会伴有间质性肺炎，该肺炎呈现快速进展病程且预后不良。我们报告一名49岁患有与ADM相关的非特异性间质性肺炎（NSIP）的患者。该患者对泼尼松龙和免疫抑制治疗无反应并死亡。尽管特发性NSIP已知预后较好，但ADM中的NSIP可能是一种致命疾病。因此，即使是NSIP，我们也应妥善治疗ADM中的间质性肺炎。
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