Early assessment of rapidly progressive interstitial pneumonia associated with amyopathic dermatomyositis
Early assessment of rapidly progressive interstitial pneumonia associated with amyopathic dermatomyositis
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DOI：
10.1007/s10067-005-0147-4
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发表时间：
2007-03-01
影响因子：
3.4
通讯作者：
Kumamoto, Toshihide
中科院分区：
文献类型：
--
作者：
Miyazaki, Eishi;Ando, Masaru;Kumamoto, Toshihide
无肌病性皮肌炎（ADM）偶尔并发快速进行性间质性肺炎（RPIP），在这种情况下，弥漫性肺泡损伤（DAD）通常在尸检时诊断。在这里，我们提出了三个病人与RPIP伴随ADM在肺部疾病的早期评估。在呼吸困难发作前进行的高分辨率计算机断层扫描（HRCT）显示均匀的胸膜下网状阴影伴微弱的磨玻璃样衰减。在此阶段，两名患者的手术肺活检显示典型的细胞非特异性间质性肺炎（NSIP）的组织学模式。尽管脉冲甲基强的松龙和随后的大剂量口服泼尼松龙，肺部疾病进展，在所有患者中，广泛区域的磨玻璃样阴影和实变观察到的HRCT扫描。1例经组织学证实为DAD。给予环孢素、环磷酰胺冲击或大剂量静脉注射免疫球蛋白后，所有患者均得到了挽救。我们的数据表明，ADM相关的间质性肺炎即使在放射学和组织学特征与NSIP一致的情况下也会发生侵袭性过程。对于这种危及生命的疾病患者，需要尽早使用高剂量类固醇和免疫抑制剂进行积极的联合治疗。
Amyopathic dermatomyositis (ADM) is occasionally complicated by rapidly progressive interstitial pneumonia (RPIP), and in such cases, diffuse alveolar damage (DAD) is usually diagnosed at autopsy. Here, we present three patients with RPIP accompanied by ADM in whom lung disease was assessed at an early stage. High-resolution computed tomography (HRCT) carried out before the onset of dyspnoea revealed uniformly subpleural reticular opacity with faint ground-glass attenuation. At that stage, surgical lung biopsies from two patients showed histological patterns typical of cellular nonspecific interstitial pneumonia (NSIP). Despite pulse methylprednisolone and subsequent high-dose oral administration of prednisolone, lung disease progressed in all patients, with extensive areas of ground-glass opacity and consolidation observed in HRCT scans. DAD was confirmed histologically in one case. Additional administration of cyclosporine, pulse cyclophosphamide or high-dose intravenous administration of immunoglobulin rescued all patients. Our data suggest that ADM-associated interstitial pneumonia takes an aggressive course even when the radiological and histological features are consistent with NSIP. Aggressive combination therapy with high-dose steroids and immunosuppressive agents is required as early as possible for patients with this life-threatening disorder.