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[1例伴间质性肺炎的无肌病性皮肌炎]

[A case of amyopathic dermatomyositis associated with interstitial pneumonitis].

作者信息

Tahara K, Nishiya K, Hisakawa N, Hashimoto K, Hara I, Kodama H

机构信息

Second Department of Internal Medicine, Kochi Medical School.

出版信息

Ryumachi. 1997 Oct;37(5):714-8.

PMID:9396375
Abstract

A patient (47-year old female) who had erythema similar to Gottron's sign on bilateral finger joints since two years ago, started to have polyarthralgia on bilateral knee and shoulder on the spring of 1993. Polyarthralgia was extended to both wrist and hand joints on Oct. of 1995. On the middle of Dec. 1995, she began to have exertional dyspnea and was referred and admitted in our hospital on 18th, Dec., 1995. Chest X-ray and CT scan showed the shadow for active interstitial pneumonitis on bilateral lower lung fields. Blood gas analysis indicated hypoxia (PaO2: 62.8 mmHg) and low % DLCO (64.7%). Skin eruption of face (heliotrope-like erythema) and hands (Gottron's sign) and skin biopsy (right hand) findings were compatible with that in dermatomyositis. The analysis in blood biochemistry showed no elevation for muscle enzymes. The diagnosis for amyopathic dermatomyositis (ADM) was made according to the criteria proposed by Euwer & Sontheimer (1993). The steroid pulse therapy and 50 mg per day of cyclophosphamide were immediately administered. The dyspnea and dermatitis were improved within two weeks after therapy. She is presently in remission until Jan. 1997 with the maintenance dosis of 10 mg per day of oral prednisolone.

摘要

一名患者(47岁女性),自两年前起双侧手指关节出现类似Gottron征的红斑,1993年春季开始双侧膝关节和肩关节出现多关节痛。1995年10月,多关节痛扩展至双侧腕关节和手部关节。1995年12月中旬,她开始出现劳力性呼吸困难,并于1995年12月18日转诊至我院并入院。胸部X线和CT扫描显示双侧下肺野有活动性间质性肺炎阴影。血气分析提示低氧血症(PaO2:62.8 mmHg)和低DLCO百分比(64.7%)。面部(向阳性红斑)和手部(Gottron征)的皮疹及皮肤活检(右手)结果符合皮肌炎表现。血液生化分析显示肌酶无升高。根据Euwer和Sontheimer(1993年)提出的标准,诊断为无肌病性皮肌炎(ADM)。立即给予类固醇冲击疗法及每日50 mg环磷酰胺治疗。治疗后两周内呼吸困难和皮炎症状改善。目前她处于缓解期,口服泼尼松龙维持剂量为每日10 mg,直至1997年1月。

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