PDF(Pubmed)
Abstract：
UNASSIGNED: Lung involvement in the context of idiopathic inflammatory myopathies has significant impact on outcome; early and accurate diagnosis is important but can be difficult to achieve. In particular, patients without clinically evident muscle involvement pose a significant diagnostic challenge.
UNASSIGNED: A computer-assisted search was conducted to identify patients with amyopathic interstitial lung disease associated with the presence of myositis-specific autoantibodies. Medical records and chest imaging studies were reviewed to identify clinical and radiologic features at presentation.
UNASSIGNED: Of the 35 patients with amyopathic interstitial lung disease associated with myositis-specific autoantibodies, the median age was 65 years (range 43-78) and 20 were women (57%). Of the patients, 34% had previously visited the rheumatology department. Presenting symptoms consisted of dyspnea (94%), cough (43%), and arthritis (23%). Raynaud phenomenon, \"mechanic hands,\" Gottron papules, and inspiratory crackles were present in 23, 31, 9, and 74% of patients, respectively. After a detailed history, none of the patients reported muscle weakness, while four (11%) exhibited increased CK levels; of these four, two had a concomitant increase in aldolase levels. Median FVC was 79% predicted (range: 49-135) and median DLco was 50% predicted (range: 17-103). HRCT pattern was suggestive of an alternative to UIP pattern in 31/33 (94%) patients; the most common imaging patterns were NSIP (49%) and NSIP/OP (39%).
UNASSIGNED: In patients with NSIP and NSIP/OP pattern, the presence of amyopathic interstitial lung disease associated with myositis-specific autoantibodies should be considered even in the absence of clinical evident myositis.
UNASSIGNED: A computer-assisted search was conducted to identify patients with amyopathic interstitial lung disease associated with the presence of myositis-specific autoantibodies. Medical records and chest imaging studies were reviewed to identify clinical and radiologic features at presentation.
UNASSIGNED: Of the 35 patients with amyopathic interstitial lung disease associated with myositis-specific autoantibodies, the median age was 65 years (range 43-78) and 20 were women (57%). Of the patients, 34% had previously visited the rheumatology department. Presenting symptoms consisted of dyspnea (94%), cough (43%), and arthritis (23%). Raynaud phenomenon, \"mechanic hands,\" Gottron papules, and inspiratory crackles were present in 23, 31, 9, and 74% of patients, respectively. After a detailed history, none of the patients reported muscle weakness, while four (11%) exhibited increased CK levels; of these four, two had a concomitant increase in aldolase levels. Median FVC was 79% predicted (range: 49-135) and median DLco was 50% predicted (range: 17-103). HRCT pattern was suggestive of an alternative to UIP pattern in 31/33 (94%) patients; the most common imaging patterns were NSIP (49%) and NSIP/OP (39%).
UNASSIGNED: In patients with NSIP and NSIP/OP pattern, the presence of amyopathic interstitial lung disease associated with myositis-specific autoantibodies should be considered even in the absence of clinical evident myositis.
摘要：
■特发性炎症性肌病的肺部受累对预后有重大影响；早期和准确的诊断很重要，但可能难以实现。特别是,没有临床上明显的肌肉受累的患者构成了重大的诊断挑战。
■进行了计算机辅助搜索，以鉴定与肌炎特异性自身抗体存在相关的肌病性间质性肺病患者。回顾了病历和胸部影像学研究，以确定临床和影像学特征。
■在35例与肌炎特异性自身抗体相关的肌病性间质性肺病患者中，中位年龄为65岁(范围43~78岁),20例为女性(57%).在患者中，34%的人以前去过风湿病科。出现的症状包括呼吸困难（94%），咳嗽（43%），关节炎（23%）。雷诺现象,\"机械师的手，\"Gottron丘疹，23、31、9和74%的患者出现吸气裂纹，分别。经过详细的历史，没有一个病人报告肌肉无力,而四个(11%)显示CK水平升高；这四个中，两个人的醛缩酶水平随之增加。FVC中位数为79%预测(范围:49-135),DLco中位数为50%预测(范围:17-103)。HRCT模式提示31/33（94％）患者可替代UIP模式；最常见的成像模式是NSIP（49％）和NSIP/OP（39％）。
■在具有NSIP和NSIP/OP模式的患者中，即使在没有临床明显肌炎的情况下,也应考虑与肌炎特异性自身抗体相关的肌病性间质性肺病的存在.
■进行了计算机辅助搜索，以鉴定与肌炎特异性自身抗体存在相关的肌病性间质性肺病患者。回顾了病历和胸部影像学研究，以确定临床和影像学特征。
■在35例与肌炎特异性自身抗体相关的肌病性间质性肺病患者中，中位年龄为65岁(范围43~78岁),20例为女性(57%).在患者中，34%的人以前去过风湿病科。出现的症状包括呼吸困难（94%），咳嗽（43%），关节炎（23%）。雷诺现象,\"机械师的手，\"Gottron丘疹，23、31、9和74%的患者出现吸气裂纹，分别。经过详细的历史，没有一个病人报告肌肉无力,而四个(11%)显示CK水平升高；这四个中，两个人的醛缩酶水平随之增加。FVC中位数为79%预测(范围:49-135),DLco中位数为50%预测(范围:17-103)。HRCT模式提示31/33（94％）患者可替代UIP模式；最常见的成像模式是NSIP（49％）和NSIP/OP（39％）。
■在具有NSIP和NSIP/OP模式的患者中，即使在没有临床明显肌炎的情况下,也应考虑与肌炎特异性自身抗体相关的肌病性间质性肺病的存在.
