Short-Term and Long-Term Outcomes of Interstitial Lung Disease in Polymyositis and Dermatomyositis A Series of 107 Patients
Short-Term and Long-Term Outcomes of Interstitial Lung Disease in Polymyositis and Dermatomyositis A Series of 107 Patients
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DOI：
10.1002/art.30513
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发表时间：
2011-11-01
影响因子：
--
通讯作者：
Menard, J. -F.
中科院分区：
文献类型：
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作者：
Marie, I.;Hatron, P. Y.;Menard, J. -F.
客观的。本研究旨在评估多发性肌炎/皮肌炎 (PM/DM) 中间质性肺疾病 (ILD) 的特征和结果，并确定预测 PM/DM 中 ILD 恶化的变量。方法。在 348 名连续的 PM/DM 患者中，通过 4 个医疗中心的病历检索发现了 107 名 ILD 患者。所有患者均接受了肺功能测试（PFT）和肺部高分辨率计算机断层扫描（HRCT）扫描。结果。 20 名患者中 ILD 发病先于 PM/DM 临床表现，69 名患者中 ILD 与 PM/DM 同时发生，18 名患者中 ILD 发生在 PM/DM 发病后。根据肺部表现，ILD 患者可分为 3 组：急性肺部疾病患者 (n = 20)、有进展性肺部体征的患者 (n = 55) 以及 PFT 和 HRCT 扫描显示与 ILD 一致的无症状患者 (n = 32)。我们观察到 32.7% 的患者肺部疾病得到缓解，而 15.9% 的患者 ILD 恶化。预测 ILD 预后不良的因素包括年龄较大、有症状的 ILD、肺活量和一氧化碳弥散能力较低、HRCT 扫描和肺活检中常见的间质性肺炎模式以及类固醇难治性 ILD。 ILD恶化患者的死亡率高于ILD未恶化患者的死亡率（47.1% vs 3.3%）。结论。我们的研究结果表明，ILD 导致 PM/DM 的高发病率。我们的研究结果还表明，对于具有 ILD 不良预后预测因素的 PM/DM 患者，可能需要更积极的治疗。
Objective. This study was undertaken to assess the characteristics and outcome of interstitial lung disease (ILD) in polymyositis/dermatomyositis (PM/DM) and to determine variables predictive of ILD deterioration in PM/DM.Methods. Among 348 consecutive patients with PM/DM, 107 patients with ILD were identified by medical records search in 4 medical centers. All patients underwent pulmonary function tests (PFTs) and pulmonary high-resolution computed tomography (HRCT) scan.Results. ILD onset preceded PM/DM clinical manifestations in 20 patients, was identified concurrently with PM/DM in 69 patients, and occurred after PM/DM onset in 18 patients. Patients with ILD could be divided into 3 groups according to their presenting lung manifestations: patients with acute lung disease (n = 20), patients with progressive-course lung signs (n = 55), and asymptomatic patients with abnormalities consistent with ILD evident on PFTs and HRCT scan (n = 32). We observed that 32.7% of the patients had resolution of pulmonary disorders, whereas 15.9% experienced ILD deterioration. Factors that predicted a poor ILD prognosis were older age, symptomatic ILD, lower values of vital capacity and diffusing capacity for carbon monoxide, a pattern of usual interstitial pneumonia on HRCT scan and lung biopsy, and steroid-refractory ILD. The mortality rate was higher in patients with ILD deterioration than in those without ILD deterioration (47.1% versus 3.3%).Conclusion. Our findings indicate that ILD results in high morbidity in PM/DM. Our findings also suggest that more aggressive therapy may be required in PM/DM patients presenting with factors predictive of poor ILD outcome.