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Formosan Journal of Rheumatology

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中華民國風濕病醫學會,正常發行

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Background: Polymyositis and dermatomyositis are idiopathic inflammatory myopathies and anti-Jo-1 is the most common autoantibody found in inflammatory myositis patients. We aimed to demonstrate clinical symptoms and signs related to anti-Jo-1 antibody in patients with polymoyositis and dermatomyositis.Materials and Methods: We retrospectively reviewed medical records of 151 patients with polymoyositis and dermatomyositis in Chang Gung Memorial Hospital from 2000 to 2007.Results: Eighty (53%) of 151 patients had identified anti-Jo-1 antibody. Interstitial lung disease, cardiac involvement, hypertension, and dysphagia were associated with the presence of anti-Jo-1 antibody according to univariate analysis (p=0.011, <0.001, 0.045, and 0.005, respectively). Multivariate analysis, after adjusting for gender, showed that only interstitial lung disease (odds ratio = 7.49, 95% CI = 1.12-49.91, p=0.037) and cardiac involvement (odds ratio = 9.39, 95% CI = 1.34-165.83, p=0.024) were associated with the presence of anti-Jo-1 antibody. Kaplan-Meier survival analysis was not significantly lower in anti-Jo-1 positive patients than that in those without anti-Jo-1 antibody (p=0.201).Conclusion: This study highlights the clinical symptoms and signs related to anti-Jo-1 antibody in polymoyositis and dermatomyositis. Clinical presentation of interstitial lung disease and cardiac involvement were significantly associated with the presence of anti-Jo-1 antibody although anti-Jo-1 antibody had no influence on survival.

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目的:探討台灣地區,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,而在國立台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。

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Background: This study examined the long-term safety of Etanercept treatment and predictors of the discontinuation and adverse events or events of therapy in patients with rheumatoid arthritis (RA). Material and Method: Adverse events or events were recorded and analyzed for 181 consecutive RA patients followed in our department from 2003 to 2010. All enrolled patients were treated with Etanercept reimbursed by National Health Insurance. Results: A total of 181 RA patients being treated with Etanercept were enrolled, of whom 153 (84.5%) were women. The mean age at diagnosis was 44.8 ± 13.9 years, and the mean age for starting Etanercept treatment was 51.4 ± 13.6 years. The mean duration of Etanercept treatment was 39.4 ± 27.5 months (median 32.6 months; range 1.4 to 87.0 months). At baseline, the mean DAS-28 score was 6.90 ± 1.06. Of the 181 patients, 34 cases (18.8%) developed adverse events or events during the treatment period (mycobacteria in 4, bacterial infections in 17, virus infection in 7, neuropathy in 3, malignancy in 2, and other drug related event in 1). One of the tuberculosis cases with left upper lobe lung lesion was possible due to reactivation. One case had left lower lobe nontuberculous mycobacterium (NTM) pneumonia. One mortality case with hepatitis B virus carrier had hepatitis B flare up with acute liver failure. Of these 34 cases, 16 (34%) discontinued Etanercept treatment. Concomitant treatment with corticosteroid increased the risk of adverse events or events (OR 4.83, 95% CI 1.05-22.19, p=0.043). Patients with an initial DAS-28 score greater than 8.0 (OR 3.20, 95% CI 1.59-6.46, p=0.001) and patients who experienced adverse events or events (OR 2.50, 95% CI 1.30-4.79, p=0.006) were more likely to discontinue Etanercept treatment. Conclusion: Despite the good clinical efficacy of Etanercept treatment, physicians must remain alert for adverse events or events in patients treated with this medication. Extra vigilance is required when treating patients with a combination of Etanercept and corticosteroid. Patients who had initial DAS-28 scores more than 8.0 were more likely to discontinue Etanercept treatment.

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Objective: To survey the prevalence of entheseal abnormality in the lower limbs in subclinical patients undergoing hemodialysis by musculoskeletal ultrasound (MSUS), rate the severity of abnormalities using the Glasgow Ultrasound Enthesitis Scoring system (GUESS), and correlate the score with other clinical factors.Methods: Forty-eight subclinical patients who had undergone hemodialysis for at least 4 months were divided into 2 groups. The groups were compared for the total GUESS score, thickness score, erosion score, enthesophyte score, and bursitis score. We then assessed the correlations between these scores and the duration of dialysis, sex, age, body mass index (BMI), and levels of serum biomarkers.Results: The duration of hemodialysis was found to be significantly correlated with the total GUESS score (p<0.001, r = 0.88) and enthesophyte score (p<0.001, r = 0.71). No significant correlation was detected between the scores and the following variables: age; sex; BMI; calcium-phosphorus product; and serum levels of calcium, phosphorous, parathormone, uric acid, triglycerides, cholesterol, and C-reactive protein. The prevalence of bilateral lower limb enthesopathy, determined on the basis of grayscale musculoskeletal ultrasound examination, was 21.8%.Conclusion: Hemodialysis patients may develop lower limb enthesopathy, which worsens with increase in the duration of hemodialysis, even in the absence of obvious clinical symptoms or signs. MSUS should be more widely employed by clinicians for the early diagnosis of enthesopathy in subclinical hemodialysis patients.

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目的:探討台灣僵直性脊椎炎(AS)患者,其臨床表現(包括年齡、性別、發病、年齡、病程等),與疾病活性度與功能之相關性。方法:收集門診344例AS確診病患,記錄其年齡、性別、發病、年齡、臨床表現(包括關節炎、虹彩炎等),及使用藥物(sulfasalagine, NSAID)等。另外,測量疾病活性度,我們使用BASDAI,測量功能性指標,我們使用BASFI。結果:在收集344AS病患中,255例為男性,89例為女性(男:女 = 2.9:1),在台灣有不同族群分佈比例,閩南AS為69.3%,客家13.2%,外省人12.4%,所有病患平均年齡為36.2,發病年齡為24.98。16歲以下發病者占所有病患35%。有關BASDAI分數,總平均為3.2(範圍0到8.5),而BASFI分數為2.55(0到9.32)。29.9%曾發生過虹彩炎,66.1%有周邊關節炎,47.1%有家族其他病患發生AS。將上述臨床及基本個人資料與BASDAI及BASFI進行相關性比較,結果顯示周邊關節炎與BASDAI有明顯相關。而BASFI與多種臨床資料包括年齡、年齡小於或大於35歲,不同年齡分布、疾病病程、周邊關節炎與非類固醇抗發炎藥物(NSAID)持續使用有明顯相關。結論:本研究顯示周邊關節炎之表現可影響BASDAI與BASFI,然更多之臨床資料包括年齡、疾病病程與NSAID會影響BASFI但不會影BASDAI。及早之診斷與治療,應可避免或減低AS病患晚期功能之喪失。

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目的:探研究在瀰漫性肺泡出血的系統性紅斑狼瘡患者使用血漿置換的角色。方法:我們對於在1985年2月及2012年1月之間系統性紅斑性狼瘡合併瀰漫性肺泡出血之病患,因危急情況而接受救援性血漿置換術,進行回顧性的分析。結果:我們的研究共包括28例瀰漫性肺泡出血的系統性紅斑狼瘡患者,其中22位病患接受血漿置換術,另外6位病患未接受血漿置換術。血漿置換術總共進行189次,每個病患每次療程的中位數為七次(範圍為2-33)。期間共有四位患者於治療期間死亡:其中一位因敗血症而死亡,一位因瀰漫性肺泡出血併急性呼吸衰竭,其他兩位併發心臟血栓而造成循環衰竭。而其接受血漿置換術的病患5年存活率為較未接受者好(分別為64%及33%)。治療期間沒有病患產生過敏性休克。且部分患者同時接受類固醇脈衝治療(1500-6000毫克)及血漿置換術。在所有療程中,平均系統性紅斑性狼瘡的疾病活動指數分別為在接受救援性血漿置換術的24.9在及在接受完血漿置換術3週後的9.1。結論:我們的資料顯示比較起使用救援性血漿置換術及同時併用或未併用類固醇脈衝治療,在系統性紅斑性狼瘡併危急瀰漫性肺泡出血病患未使用血漿置換術有較高的死亡率。

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目的:成人一般變異性免疫缺乏症很少在本國被報告出來。吾人調查南台灣某教學醫院此類病患的自體免疫表現。方法:對國立成功大學醫學院附設醫院於民國九十年至一百年間被診斷為一般變異性免疫缺乏症十八歲以上之成人進行病歷回溯性調查,吾人仔細分析這些病患的基本特徵、臨床表現、實驗室資料、放射影像和微生物檢查。結果:總共找出五女一男計六位病患,平均疾病開始年齡為 22.3 ± 19.0歲及診斷年齡為 27.1 ± 17.7歲,平均診斷延遲時間為 4.8 ± 2.9年。這些患者中四位具有臨床上自體免疫表現,分別是關節炎(類風濕性關節炎與幼年性關節炎)二位、病理切片證實腸炎二位與血小板減少一位。給予靜脈注射免疫球蛋白治療後二位病患之關節炎均有顯著改善。所有病患皆有具外殼的病原細菌之感染併發症,且一半患者產生支氣管擴張。目前已有二位病患死亡,其中一位死因為廣泛性感染。結論:自體免疫表現常見於在南台灣一般變異性免疫缺乏症的病患,積極靜脈注射免疫球蛋白治療可明顯改善這類病患臨床上自體免疫造成之關節炎。

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Background: To investigate the prevalence of Baker's cysts (BCs) in patients with gouty arthritis of the knee using high-resolution musculoskeletal ultrasound (MSUS). To the best of our knowledge, this is the first report in the literature.Methods: We retrospectively reviewed clinical and MSUS data of 103 consecutive patients with gouty arthritis of the knee and 150 patients with knee osteoarthritis examined as a control group. They all had crystal confirmation of gout after joint aspiration. The correlation of the presence of BCs with the presence of joint effusion, synovitis, the double-contour sign, tendinopathy, and tophi was analyzed.Results: BCs were demonstrated by MSUS in 28 (27.1%) of the 103 patients. Only 10 (35.7%) of the 28 cysts had been clinically diagnosed. Joint effusion, synovitis, the double-contour sign, tendinopathy, and tophi were respectively detected in 96.1%, 77.6%, 32.0%, 4.9%, and 7.8% of knees. There was a significant correlation between the presence of BCs and synovitis (p<0.05). There was no significant correlation between the presence of BCs with effusion, the double-contour sign, tendinopathy, and tophi (p>0.05). Two ruptured BCs were observed.Conclusions: These results indicated that BCs are common in patients with gouty arthritis, and that they can easily escape clinical detection. Therefore, MSUS should be more widely employed by clinicians for diagnosis and treatment selection for BCs, which may sometimes be accompanied by significant morbidity, and thus help choose an appropriate treatment.