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題名 | Interstitial Lung Disease in Polymyositis/dermatomyositis: Association between the Onset of Interstitial Lung Disease and Clinical Outcome=多發性肌炎與皮肌炎患者併發間質性肺病時序與臨床預後之分析 |
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作者姓名(中文) | 全以祖; 吳建陞; 蔡瑋儀; 許秉寧; | 書刊名 | 中華民國風濕病雜誌 |
卷期 | 26:1/2 2012.12[民101.12] |
頁次 | 頁23-30 |
分類號 | 415.762 |
關鍵詞 | 皮肌炎; 間質性肺病; 多發性肌炎; 存活分析; Dermatomyositis; Interstitial lung disease; Polymyositis; Survival analysis; |
語文 | 英文(English) |
中文摘要 | 目的:探討臺灣地區,多發性肌炎(polymyositis, PM)與皮肌炎(dermatomyositis, DM)合併間質性肺病(interstitial lung disease, ILD)之患者,其預後因子與存活之分析。方法:本回溯性研究分析於1999至2008年間,同時合併PM/DM與ILD,而在國立臺灣大學附設醫院接受治療之病患病歷資料。結果:分析285名PM/DM患者,發現有43名同時合併ILD(15%)。依發生ILD與PM/DM之時序,可分為三組病人:(1)ILD發生在PM/DM診斷之前(n = 8),(2)ILD與PM/DM同時診斷(n = 19),(3)ILD發生在PM/DM診斷之後(n = 16)。我們分析發現,第3組病人年紀較輕、併發間質性肺病時所用的類固醇劑量較高,表示第3組的病人可能疾病活性也較高。並且相較於第1組(75%)有較差的存活率(37%)。合併ILD之PM/DM病患平均死亡率為44%,然而在第三組的病人就高達63%(10/16)。三組大部分病患的死亡皆發生在診斷ILD的一年內死亡。結論:同時合併ILD之PM/DM病患通常臨床預後較差,並且死亡率高。而PM/DM患者發生ILD之時序,可能與其預後相關。PM/DM之後才發生的ILD,也許可作為預後不佳之重要指標。 |
英文摘要 | Objective: We assessed the clinical outcome and prognostic predictors in patients with polymyositis (PM)/dermatomyositis (DM) and interstitial lung disease (ILD) in Taiwan. Methods: Forty-three patients with PM/DM and ILD were enrolled from January 1999 to July 2008 at the National Taiwan University Hospital and their clinical data were reviewed and analyzed. Results: The proportion of ILD in our PM/DM patients was 15% based on high resolution computer tomography or pulmonary function test results. All patients were classified into 3 subgroups according to the onset sequence of ILD: Group 1 was comprised of patients in whom ILD was diagnosed preceding PM/DM (n = 8), Group 2 included patients in whom ILD was diagnosed concomitantly with PM/DM (n = 19), and Group 3 consisted of patients in whom ILD was diagnosed after PM/DM (n = 16). The patients in Group 3 were younger (p=0.04), and had received more corticosteroid medications (p=0.01) when ILD was diagnosed, which indicated more severe disease activity in Group 3 patients. These patients were also associated with a significantly decreased survival rate (37%) compared to those with ILD preceding PM/DM (75%; p=0.032). The overall mortality rate in patients with PM/DM and ILD was approximately 44%, however, the mortality rate in Group 3 patients was as high as 63% (10/16). Most mortality occurred within 1 year after the diagnosis of ILD among the 3 groups. Conclusion: PM/DM patients with ILD are associated with poor clinical outcome and a high mortality rate. The onset of ILD and PM/DM may be associated with prognostic outcome in these patients. PM/DM onset followed by ILD development may be an important indicator for poor prognosis. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。