Overlap of systemic lupus erythematosus and myositis is rare in anti-Ku antibody-positive patients
Overlap of systemic lupus erythematosus and myositis is rare in anti-Ku antibody-positive patients
复制标题
抗 Ku 抗体阳性患者很少同时患有系统性红斑狼疮和肌炎
DOI：
10.1136/annrheumdis-2019-216375
复制
发表时间：
2019
影响因子：
27.4
通讯作者：
Akiyama Masashi
中科院分区：
文献类型：
--
作者：
Ogawa-Momohara Mariko;Muro Yoshinao;Akiyama Masashi
抗ku抗体最初被报道为硬皮病-多发性肌炎（PM）重叠综合征相关的自身抗体。然而，它们也经常在各种结缔组织疾病（CTDs）中发现，其临床意义尚未最终确定。此外，亚洲CTD队列中抗ku的研究较少。最近，Spielmann等人1发表了一项法国单中心大队列研究的值得注意的报告，该研究试图对各种CTDs的抗ku阳性患者进行分类，并能够识别出两个不同的患者亚组：“血清肌酸激酶（CK）水平升高的抗ku阳性患者”和“抗双链DNA抗体（抗dsdna）的抗ku阳性患者”。前一组患者发生间质性肺病的风险较高，后一组患者发生肾小球肾炎的风险较高。在本研究中，我们回顾性地筛选了600名日本CTDs患者的血清，这些患者来我们的研究所2，通过免疫荧光模式检测抗ku阳性，与之前的研究一样。1用抗ku70和抗ku80酶联免疫吸附试验筛选疑似抗ku70阳性血清，并用免疫沉淀-免疫印迹法进行验证。我们发现了10例抗库库阳性患者，并分析了他们的临床和实验室结果（表1）。平均年龄47.2±23.9岁。女性9例，平均随访5.7年（0.5 ~ 26年）。5例患者CK升高，诊断为PM或皮肌炎（DM）。5例PM/DM患者中有2例同时发生系统性硬皮病（SSc）和PM。在其他5例没有CK升高的患者中，3例被诊断为系统性红斑狼疮（SLE），其中1例在20年的病史后发展为SSc。3例SLE患者中，10例抗ku阳性患者均未出现CK升高。这些结果与Spielmann等人报道的结果一致。1有两项研究支持抗ku阳性患者肌炎重叠SLE的发生率较低。46例抗ku阳性CTD患者中，17例有肌炎（PM或DM）， 9例有SLE谱（抗磷脂综合征或SLE），但PM与SLE重叠综合征仅1例。3另一项回顾性ctd筛查研究报道，在30例抗ku阳性患者中，仅有1例肌炎/SLE重叠患者，包括11例肌炎患者（炎症性肌病、包体体肌炎和PM）和8例SLE谱患者。4这些在肌炎患者或sled谱系患者中所见的特征与Spielmann等人报道的结果一致。1我们还收集了所有10例抗库阳性患者的抗dsdna数据。其中4例抗dsdna阳性。令人惊讶的是，在4名抗脱氧核糖核酸阳性患者中，有3名是ck升高的患者。相反，所有3例SLE患者抗dsdna均为阴性。因此，在目前的抗ku阳性患者中，抗dsdna阳性与SLE是相互排斥的。相比之下，先前的一项国际研究比较了22例抗钾阳性SLE患者和209例抗钾阴性SLE患者的临床和实验室特征。5在抗cu阳性和抗cu阴性SLE组中，抗dsdna的频率相似，分别为31.8%和32.2%。此外，Spielmann等人1报道，在抗库氏阳性SLE患者中经常发现抗dsdna（89%, 7/8）。这种差异可能是由于遗传背景的不同。由于抗- dsdna在抗- ku阳性SLE 5中并不常见（约30%），除了存在…
Anti-Ku antibodies were originally reported as scleroderma-poly myositis (PM) overlap syndrome-related autoantibodies. However, they are also frequently found in various connective tissue diseases (CTDs) and their clinical significance has not been conclusively determined. Moreover, there are few studies on anti-Ku in Asian CTD cohorts. Recently, Spielmann et al 1 published a notable report of a French single-centre large-cohort study which tried to classify anti-Ku-positive patients with various CTDs and was able to identify two distinct subgroups of patients:‘anti-Ku-positive patients with elevated serum creatine kinase (CK) levels’ and ‘anti-Ku-positive patients with anti-double-strand DNA antibodies (anti-dsDNA)’. Patients in the former group were at high risk of developing interstitial lung disease and those in the latter were at high risk of developing glomerulonephritis. In the present study, we retrospectively screened sera from 600 Japanese patients with CTDs who visited our institute 2 by immunofluorescence patterns for anti-Ku-positivity, as performed in a previous study. 1 Sera suspected of being anti-Ku-positive were then screened by anti-Ku70 and anti-Ku80 ELISAs and verified by immunoprecipitation-immunoblot. We found 10 anti-Kupositive patients and analysed their clinical and laboratory findings (table 1). Their average age was 47.2±23.9 years. Nine were female and their average follow-up period was 5.7 years (0.5–26 years). Five patients showed CK elevation and were diagnosed with PM or dermatomyositis (DM). Two of the five PM/DM patients had developed systemic scleroderma (SSc) and PM simultaneously. Of the other five patients without CK elevation, three had been diagnosed with systemic lupus erythematosus (SLE), one of whom had developed SSc after a 20 year disease history. None of the 3 SLE patients among the present 10 anti-Ku-positive patients showed CK elevation. These results are consistent with the findings reported by Spielmann et al. 1 There are two studies supporting the lower frequency of myositis overlapping SLE in anti-Ku-positive patients. 3 4 Among 46 anti-Ku-positive CTD patients, 17 had myositis (PM or DM) and 9 had SLE spectrum (anti-phospholipid syndrome or SLE), but there was only 1 case with overlap syndrome of PM and SLE. 3 Another retrospective CTD-screened study reported that only 1 myositis/SLE overlap patient was seen among 30 anti-Ku-positive patients, including 11 myositis patients (inflammatory myopathy, inclusion body myositis and PM) and 8 SLE-spectrum patients. 4 These characteristics seen in myositis patients or SLE-spectrum patients are consistent with the findings reported by Spielmann et al. 1 We also collected the data on anti-dsDNA for all 10 anti-Kupositive patients. Four of them were positive for anti-dsDNA. Surprisingly, of the four anti-dsDNA-positive patients, three were CK-elevated patients. In contrast, all three SLE cases were negative for anti-dsDNA. Thus, anti-dsDNA positivity and SLE were mutually exclusive in the present anti-Ku-positive patients. In contrast, a previous international study compared the clinical and laboratory characteristics of 22 anti-Ku-positive SLE patients with those of 209 anti-Ku-negative SLE patients. 5 In both anti-Ku-positive and anti-Ku-negative SLE groups, frequencies of anti-dsDNA were similar: 31.8% and 32.2%, respectively. Furthermore, Spielmann et al 1 reported that anti-dsDNA was very often found in anti-Kupositive SLE patients (89%, 7/8). This discrepancy might be due to a difference of genetic backgrounds. Since anti-dsDNA are not so frequently found (around 30%) in anti-Ku-positive SLE 5 in addition to the presence …
影响因子：
27.4
作者：
Spielmann, Lionel;Nespola, Benoit;Meyer, Alain
通讯作者：
Meyer, Alain
影响因子：
2.6
作者：
Mahler, M.;Swart, A.;Fritzler, M. J.
通讯作者：
Fritzler, M. J.