Interstitial lung diseases associated with amyopathic dermatomyositis
Interstitial lung diseases associated with amyopathic dermatomyositis
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DOI：
10.1183/09031936.06.00038806
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发表时间：
2006-11-01
影响因子：
24.3
通讯作者：
Chida, K.
中科院分区：
文献类型：
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作者：
Suda, T.;Fujisawa, T.;Chida, K.
为探讨间质性肺病（ILD）合并无肌病性皮肌炎（ILD-ADM）患者的临床特点及预后，对14例ILD合并无肌病性皮肌炎（ILD-ADM）患者的临床资料进行分析。比较两种形式的临床特征、对治疗的反应性和预后。9例ILD-ADM患者分为急性/亚急性型，5例为慢性型。急性/亚急性ILD患者的动脉血氧分压显著低于慢性ILD患者。在高分辨率计算机断层扫描中，毛玻璃样阴影在两种形式中经常被发现，但实变在急性/亚急性ILD中比慢性ILD更常见。支气管肺泡灌洗分析显示，急性/亚急性ILD的总细胞和淋巴细胞数量高于慢性ILD。组织学上，最常见的发现是两种形式的非特异性间质性肺炎，而弥漫性肺泡损伤仅见于急性/亚急性ILD。急性/亚急性ILD通常对治疗耐药，而慢性ILD反应良好。值得注意的是，急性/亚急性ILD的死亡率远高于慢性ILD（分别为67%和0%）。总之，与无肌病性皮肌炎相关的间质性肺病包括两种不同的形式，急性/亚急性和慢性形式，具有不同的发病率。
The aim of the present study was to clarify the clinical characteristics and prognosis of patients with interstitial lung disease (ILD) associated with amyopathic dermatomyositis (ILD-ADM).The study consisted of 14 consecutive patients with ILD-ADM. Patients were classified into two categories, acute/subacute and chronic forms, according to the clinical presentation of ILD. The clinical features, responsiveness to therapy, and prognosis between the two forms were compared.Nine ILD-ADM patients were categorised as the acute/subacute form, and five as the chronic form. Arterial oxygen tension was significantly lower in the acute/subacute ILD than chronic ILD patients. On high-resolution computed tomography, ground-glass opacities were frequently found in the two forms, but consolidation was more common in acute/subacute ILD than chronic ILD. Bronchoalveolar lavage analysis showed higher numbers of total cells and lymphocytes in acute/subacute ILD than chronic ILD. Histologically, the most common finding was nonspecific interstitial pneumonia in the two forms, while diffuse alveolar damage was only found in acute/ subacute ILD. Acute/subacute ILD was generally resistant to therapy, while chronic ILD responded well. Notably, the mortality of acute/subacute ILD was much higher than that of chronic ILD (67 versus 0%, respectively).In conclusion, interstitial lung disease associated with amyopathic dermatomyositis includes two different forms, the acute/subacute and chronic forms, with distinct prognoses.