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Anti-Sm and Anti-U1-RNP Antibodies: An Update 抗sm和抗u1 - rnp抗体研究进展
Pub Date : 2016-12-20 DOI: 10.35248/2684-1630.16.1.E104
J. Lemerle, Yves Renaudineau
From their discovery anti-Sm autoantibodies (Ab) have been associated with systemic lupus erythematosus (SLE), while anti-U1-RNP Ab detected alone are predominant in patients with mixed connective disease (MCTD). However, the identification of anti-Sm/U1-RNP Ab in a patient may be challenging, and usually requiring a two-step process including a screening step performed by indirect immunofluorescence (IIF) on HEp-2 cells showing a coarse speckled nuclear staining at an elevated level, followed by a confirmatory assay using specific antigens. The recent development of novel assays and the characterization of the target epitopes have been beneficial to improve the sensitivity for anti-Sm/U1-RNP Ab detection, but, in some cases, the necessity to use a different assay remains mandatory. Another recent and unexpected observation is related to the suspected role played by environmental and epigenetic factors in the induction of anti-Sm/U1-RNP Abs. Altogether, better knowledge regarding anti-Sm/U1- RNP Ab will undoubtedly provide improvements for the management and treatment of these patients.
从他们的发现来看,抗sm自身抗体(Ab)与系统性红斑狼疮(SLE)有关,而单独检测到的抗u1 - rnp Ab在混合性结缔病(MCTD)患者中占主导地位。然而,在患者中鉴定抗sm /U1-RNP抗体可能是具有挑战性的,通常需要两步的过程,包括通过间接免疫荧光(IIF)对HEp-2细胞进行筛选,HEp-2细胞在高水平上显示粗糙的斑点状核染色,然后使用特定抗原进行确认试验。最近新检测方法的发展和目标表位的表征有助于提高抗sm /U1-RNP Ab检测的敏感性,但是,在某些情况下,使用不同检测方法的必要性仍然是强制性的。另一个最近的意外观察结果与环境和表观遗传因素在诱导抗sm /U1-RNP抗体中可能起的作用有关。总之,更好地了解抗sm /U1-RNP抗体无疑将为这些患者的管理和治疗提供改进。
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引用次数: 5
Lupus: Open Access for Incredible Lupus Research 狼疮:开放获取不可思议的狼疮研究
Pub Date : 1900-01-01 DOI: 10.35248/2684-1630.20.5.e151
Yves Renaudineau
Lupus is an Incurable auto immune disorder in which body immune system attacks its own tissues or organs. It affects many parts of the body including Skin (Subcutaneous/cutaneous Lupus), Kidneys (Lupus Nephrites), Brain (Cerebral/CNS Lupus) etc., with several symptoms of Rashes (Malar, Discoid or photosensitive), Musculoskeletal Problems, Serositis, Anemia, Seziures and several many more. We can find several Diagnostic approaches for Lupus.
狼疮是一种无法治愈的自身免疫性疾病,患者的免疫系统会攻击自身的组织或器官。它影响身体的许多部位,包括皮肤(皮下/皮肤红斑狼疮),肾脏(肾性红斑狼疮),大脑(脑/中枢神经系统红斑狼疮)等,具有皮疹(麻风疹,盘状或光敏),肌肉骨骼问题,浆液炎,贫血,癫痫发作等几种症状。我们可以找到几种狼疮的诊断方法。
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引用次数: 0
Hydralazine Induced Lupus Nephritis 肼嗪致狼疮性肾炎
Pub Date : 1900-01-01 DOI: 10.35248/2684-1630.20.5.147
H. Timlin, J. Shiroky, M. Wu, D. Geetha
Renal involvement is uncommon in the hydralazine induced systemic lupus erythematosus. We conducted a retrospective study to identify patients with biopsy proven Hydralazine induced lupus nephritis. Material and Methods: In this retrospective study, patients who had a diagnosis of hydralazine-induced lupus and were on hydralazine prior to their diagnosis of biopsy proven lupus nephritis were included. Clinical and laboratory data were obtained from review of medical records. The median follow-up time was 12 months. Results: Medical records were reviewed between 2013 to 2017. Four patients had a diagnosis of biopsy proven hydralazine-induced lupus nephritis and were on hydralazine prior to their diagnosis. The median age was 68 years at the time of diagnosis. The majority of patients were Caucasian (75%). Three were female (75%) and three (75%) were exposed to hydralazine 100mg three times daily. All four patients had biopsy proven lupus nephritis (class II, III, IV, III/IV) with elevated serum creatinine and were positive for ANA (titer of 640-1280, homogenous pattern). Of the three patients tested, all were positive for anti-Histone antibody. Two patients had positive anti-dsDNA, and one of them had low C3 and C4. The level of Anti-dsDNA normalized at 3 months while low C3 in one patient persisted at 12months. All had negative C-ANCA and 3 of the 4 had positive P-ANCA. All had strong positive MPO titer and 2 of the 3 tested had positive PR3.In addition to withdrawal of hydralazine, all four patients were treated with steroids, hydroxychloroquine and mycophenolate mofetil. Two of four patients received PLEX and two received Cytoxan and hemodialysis. Conclusion: A timely diagnosis of hydralazine induced lupus nephritis can be critical. In addition to withdrawal of hydralazine, all patients also require aggressive treatment similar to idiopathic lupus nephritis.
肾受累是罕见的在肼引起的系统性红斑狼疮。我们进行了一项回顾性研究,以确定活检证实肼嗪引起狼疮性肾炎的患者。材料和方法:在这项回顾性研究中,被诊断为hydralazine诱导狼疮的患者,在活检诊断为狼疮肾炎之前服用hydralazine。临床和实验室数据是通过查阅医疗记录获得的。中位随访时间为12个月。结果:回顾了2013 - 2017年的医疗记录。4例患者活检诊断证实肼致狼疮性肾炎,并在诊断前服用肼。确诊时的中位年龄为68岁。大多数患者为白种人(75%)。3名女性(75%),3名(75%)暴露于肼嗪100mg,每日3次。所有4例患者活检证实狼疮性肾炎(II、III、IV、III/IV级),血清肌酐升高,ANA阳性(滴度640-1280,同质模式)。3例患者抗组蛋白抗体均呈阳性。2例患者抗dsdna阳性,1例患者C3、C4低。抗dsdna水平在3个月时恢复正常,而有1例患者在12个月时持续低C3水平。C-ANCA阴性,P-ANCA阳性3例。所有患者MPO滴度均为强阳性,3例患者中2例PR3阳性。除停用肼外,所有4例患者均接受类固醇、羟氯喹和霉酚酸酯治疗。4例患者中2例接受PLEX治疗,2例接受环磷酰胺和血液透析治疗。结论:及时诊断肼肼所致狼疮性肾炎至关重要。除了停用肼外,所有患者还需要类似于特发性狼疮性肾炎的积极治疗。
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引用次数: 1
P-glycoprotein Expressing-B cell associated Active True Renal Lupus Vasculitis in Lupus Nephritis 狼疮性肾炎中p糖蛋白表达与b细胞相关的活动性真肾狼疮血管炎
Pub Date : 1900-01-01 DOI: 10.35248/2684-1630.20.5.150
S. Tsujimura, Akio Kawabe, Yoshiya Tanaka
True renal lupus vasculitis (TRLV), a rare form of vascular lesion usually associated with proliferative lupus nephritis  (LN), is resistant to conventional treatments and associated with poor renal outcome among renal vasculopathies.  Evidence suggests the involvement of P-glycoprotein (P-gp) expressing-activated B cells in the pathogenesis and  treatment resistance of TRLV through the production of autoantibodies and direct infiltration into the inflammatory  lesion of small vessels. Therapies targeting activated B cells might overcome refractory TRLV. Identification of  the subsets of peripheral activated B cells that express P-gp in TRLV patients might help the selection of suitable  treatment strategy.
真性肾性狼疮性血管炎(TRLV)是一种罕见的血管病变,通常与增殖性狼疮性肾炎(LN)有关,在肾脏血管病变中,常规治疗无效,且与预后差有关。有证据表明,表达p -糖蛋白(P-gp)的活化B细胞通过产生自身抗体和直接浸润到小血管炎性病变,参与TRLV的发病和治疗抵抗。靶向活化B细胞的治疗可能克服难治性TRLV。鉴定TRLV患者表达P-gp的外周活化B细胞亚群可能有助于选择合适的治疗策略。
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引用次数: 2
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Lupus: Open Access
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