Interstitial lung disease in polymyositis and dermatomyositis: Longitudinal evaluation by pulmonary function and radiology
Interstitial lung disease in polymyositis and dermatomyositis: Longitudinal evaluation by pulmonary function and radiology
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DOI：
10.1002/art.23571
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发表时间：
2008-05-15
期刊：
影响因子：
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通讯作者：
Lundberg, Ingrid E.
中科院分区：
文献类型：
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作者：
Fathi, Maryam;Vikgren, Jenny;Lundberg, Ingrid E.
Objective.评估多发性肌炎（PM）和皮肌炎（DM）患者间质性肺病（ILD）的预测因素和长期预后。我们进行了一项前瞻性研究，其中新诊断的PM/DM患者，无论肺部疾病的临床症状如何，均进行了重复胸部X线摄影、肺部高分辨率计算机断层扫描（HRCT）和肺功能检查（PFT）。临床、影像学和肺功能结果基于最后一次随访结果。纳入了23例患者，平均随访期为35个月。在18例患者（78%）中记录了与ILD相容的影像学检查和/或PFT结果。与无ILD的患者相比，ILD患者的肺功能较低，放射学评分较高，肌酸激酶值较高。所有患者均接受大剂量糖皮质激素和其他免疫抑制剂治疗。2例患者死于ILD，均伴有活动性肌炎。在随访期间，33%的患者的总肺容量（TLC）改善，39%的患者保持稳定，28%的患者恶化。TLC的改变与HRCT结果仅部分相关，即使在肺功能正常化后仍持续存在。与PM/DM相关的ILD在大多数情况下是轻度、慢性的，在免疫抑制治疗期间没有进展。即使ILD的放射学体征持续存在，PFT也可以在免疫抑制治疗期间恢复正常。ILD的病程在首次检查时无法预测。因此，肌炎伴ILD患者在随访期间需要仔细评估临床特征以及PFT和放射学特征。
Objective. To estimate predictors and long-term outcome of interstitial lung disease (ILD) in patients with polymyositis (PM) and dermatomyositis (DM).Methods. We conducted a prospective study in which newly diagnosed PM/DM patients, regardless of clinical symptoms of pulmonary disease, were investigated with repeated chest radiography, high-resolution computed tomography (HRCT) of the lungs, and pulmonary function test (PFT). Clinical, radiologic, and lung function outcome was based on the last followup results.Results. Twenty-three patients with a mean followup period of 35 months were included. Findings on radiographic examination and/or PFT compatible with ILD were recorded in 18 patients (78%). Patients with ILD had lower lung function, higher radiologic scores, and higher creatine kinase values than those without ILD. All patients were treated with high-dose glucocorticoids and other immunosuppressive agents. Two patients died due to ILD, both with active myositis. During the followup, total lung capacity (TLC) improved in 33%, remained stable in 39%, and deteriorated in 28%. Changes in TLC correlated only partially with HRCT findings, which persisted even after normalizing for lung function.Conclusion. ILD associated with PM/DM is in most cases mild, chronic, and has a nonprogressive course during immunosuppressive treatment. PFT can be normalized during treatment with immunosuppressive therapy, even if radiologic signs of ILD persist. The course of ILD could not be predicted on the first examination. Therefore, myositis patients with ILD need careful evaluation of clinical features as well as PFT and radiologic features during followup.