老年性系统性红斑狼疮的临床特征

目的 探讨老年性系统性红斑狼疮患者的临床特征。方法 总结56例老年性系统性红斑狼疮患者临床表现、自身抗体分布特点及治疗效果，并与中青年患者对比分析。结果老年组蝶形红斑、盘状红斑、光过敏、脱发、雷诺现象等皮肤表现及肾损害和神经系统损害较中青年组明显少见(P＜0、05)，肺部病变、肌痛和肌无力、疲乏明显高于中青年组，而血液系统损害和狼疮的两项特异性抗体(抗ds—DNA和抗Sm抗体)、非特异性抗体(抗SS—A和抗SS—B抗体)以及补体C3下降的阳性率都低于中青年组(P＜0．05)。结论 老年性与中青年性系统性红斑狼疮相比在临床表现、实验室检查等方面有其自身特点，在临床诊断和治疗中应给予足够重视。     

抗精子抗体对精子顶体酶活性的影响

本文旨在观察抗精子抗体对精子顶体酶活性的影响。选择男性不育者50例与正常生育者20例，采用固相酶染色法测抗精子抗体，以固定明胶薄膜法测精子顶体酶活性。结果发现50例不育者抗精子抗体阳性率为52％。不育者精子顶体酶活性明显低于生育者；抗精子抗体阳性者须体酶活性明显低于阴性者，表明抗精子抗体可降低精子顶体酶活性。     

抗精子抗体对精子顶体酶活性的影响

本文旨在观察抗精子抗体对精子顶体酶活性的影响。选择男性不育者５０例与正常生育者２０例，采用固相酶染色法测抗精子抗体，以固定明胶薄膜法测精子顶体酶活性。结果发现５０例不育者抗精子抗体阳性率为５２％，不育者精子顶体酶活性明显低于生育者；抗精子抗体阳性者顶体酶活性明显低于阴性者，表明抗精子抗体可降低精子顶体酶活性。     

抗精子抗体对精子顶体酶活性的影响

目的：观察抗精子抗体对精子顶体酶活性的影响。方法：选择男性不育者５０例，与正常生育者２０例。采用固相酶染色法测抗精子抗体，固定明胶薄膜法测精子顶体酶活性。结果：５０例不育者抗精子抗体阳性率为５２％。不育者精子顶体酶活性明显低于生育者；抗精子抗体阳性者顶体酶活性低于阴性者。结论：抗精子抗体可降低精子顶体酶活性。     

SLE患者血清对人脐静脉内皮细胞ICAM-1和MCP-1表达的影响及氟伐他汀的干预作用

目的：观察抗核抗体（ANA）和抗ds-DNA抗体对人脐静脉血管内皮细胞（HUVEC）细胞间黏附分子-1（ICAM-1）、单核细胞趋化因子-1（MCP-1）表达的影响及他汀类药物氟伐他汀（fluvastatin,flu）干预后的变化,以探讨ANA和抗ds-DNA抗体在系统性红斑狼疮（SLE）血管炎中的致病机制和flu对血管内皮保护作用。方法：体外培养HUVEC,收集女性SLE患者血清（以抗核抗体全套为依据,分3组：ANA阴性、ANA滴度1：80、ANA滴度1：80和抗ds-DNA抗体均阳性的患者每组各5例）及女性健康对照者血清作为干预因素,分为空白对照组、正常对照组、SLE血清组1、2、3和SLE血清与氟伐他汀共同干预组。应用免疫细胞化学和双抗体夹心ELISA法检测不同干预因素对HUVECICAM-1和MCP-1的蛋白表达影响。结果：正常对照组和SLE血清组1与空白对照组相比,ICAM-1和MCP-1蛋白表达差异无统计学意义（P〉0.05）;SLE血清组2和SLE血清组3的ICAM-1与MCP-1表达高于正常对照组和SLE血清组1（P均〈0.01）。氟伐他汀（1×10^-5mol/L）能显著降低SLE血清组2和SLE血清组3刺激的HUVEC表达ICAM-1与MCP-1（P〈0.01,P〈0.05）。结论：ANA阳性（滴度为1：80）,尤其是ANA和抗ds-DNA抗体均阳性的SLE血清可激活HUVEC,使其细胞内和细胞培养上清液中ICAM-1和MCP-1的蛋白表达增加;ANA阴性的SLE血清不能激活HUVEC,不能使其细胞内和细胞培养上清液中ICAM-1和MCP-1的蛋白表达增加。氟伐他汀可下调激活的HUVEC表达ICAM-1和MCP-1。     

24例男性系统性红斑狼疮临床特点分析

目的：了解男性系统性红斑狼疮的临床特点.方法：回顾分析我院自2005年~2012年收治的24例男性SLE患者的临床资料、血清免疫检查及肾组织病理.结果：24例男性系统性红斑狼疮患者发热11例（45.8%）,尿检异常20例（83.3%）,肾衰竭5例（20.8%）,血液系统损害22例（91.6%）,面部红斑11例（45.8%）,浆膜腔炎症12例（50%）,光敏感6例（25%）,关节痛11例（45.8%）,口腔溃疡7例（29.1%）,低补体3血症15例（62.5%）,高血压12例（50%）,脱发2例（8.3%）.免疫血清学指标：ANA阳性24例（100%）,ds-DNA阳性11例（45.8%）,抗sm抗体阳性7例（29.1%）,抗ss-A阳性14例（58.3%）,抗ss-B抗体阳性6例（25%）,肾组织活检病理：狼疮性肾炎Ⅳ或Ⅴ型.结论：男性系统性红斑狼疮发病机制尚不清楚,男性体内女性激素水平升高是导致男性SLE的发生的原因之一.从我们收集的24例男性SLE患者的病例资料分析,男性SLE发生年龄与女性基本相同,以脱发、血液系统损害、高血压为主的男性患者构成比高于女性患者,口腔溃疡男性少于女性,而发热、面部红斑、关节疼痛、光敏感、尿检异常、肾功能异常与女性表现相似（P＞0.05）;ANA、抗SS-A高于女性组而抗sm抗体、ds-DNA、C3 降低的阳性率与女性相似,肾组织活检病理提示男性肾组织病理以Ⅳ和Ⅴ型多见,预后不良.诊断依靠综合多方面因素加以考虑,了解了男性SLE的临床特点,尽早查ANA、ds-DNA、抗SS-A、抗sm抗体和C3可提高对男性SLE早期诊断率,以便早期发现,及时治疗.     

Luminex技术与ELISA方法检测抗HLA抗体在肾移植急性排斥反应中的应用

目的 探讨用Luminex技术与酶联免疫吸附试验(ELISA)方法检测肾移植受者抗HLA抗体的结果差异及其与移植后早期急性排斥反应(AR)的关系.方法 以2010年10月至2012年10月接受亲属活体肾移植的34例受者为研究对象,术前采集受者血清,同时应用Luminex技术与ELISA方法进行抗HLA-Ⅰ类、Ⅱ类抗体检测,并且记录术后早期AR发生情况.结果 用Luminex技术检测抗HLA-Ⅰ类抗体阳性率为41.2％(14/34),检测抗HLA-Ⅱ类抗体阳性率为38.2％(13/34);用ELISA方法检测抗HLA-Ⅰ类抗体阳性率为2.9％(1/34),检测抗HLA-Ⅱ类抗体阳性率为8.8％(3/34);两种方法检测抗HLA-Ⅰ类、Ⅱ类抗体阳性率的差异有统计学意义(x2=14.46,P＜0.05;x2 =8.17,P＜0.05).用Luminex技术检测18例术前抗体阳性者中有8例术后早期发生AR(占44.4％);16例术前抗体阴性者中,有2例术后早期发生AR(占12.5％).用ELISA方法检测4例术前抗体阳性者中,有3例术后早期发生AR(占75.0％);30例术前抗体阴性者中,有7例术后早期发生AR(占23.3％).结论 Luminex技术检测肾移植受者抗HLA抗体的检出阳性率高于ELISA方法,对于移植后早期AR的监测可以提供有用的信息.     

自身免疫异常对不明原因性不孕女性IVF妊娠结局的影响

目的:探讨自身免疫异常对不明原因性不孕女性IVF妊娠结局的影响。方法:选择2002年1月～2006年7月由于不明原因性不孕而接受IVF-ET治疗的女性236例,其中血清抗磷脂抗体(APA)阳性者34例,抗滋养细胞膜抗体(ATA)阳性者33例,hCG抗体(hCGAb)阳性者35例,抗子宫内膜抗体(EmAb)阳性者32例,阴性对照102例,两种或两种以上抗体阳性患者排除本研究之外。比较抗体阳性组和阴性对照组女性胚胎种植率、临床妊娠率、流产率、生化妊娠率及持续妊娠率。结果:各抗体阳性组虽较阴性对照组的临床妊娠率、胚胎种植率低,但差异无显著性(P>0.05);APA、ATA、hCGAb阳性组生化妊娠率高于EmAb阳性组及阴性对照组(P<0.05);EmAb阳性组与阴性对照组之间生化妊娠率差异无显著性(P>0.05);各抗体阳性组流产率均高于阴性对照组(P<0.05);持续妊娠率均低于阴性对照组(P<0.05)。结论:APA、ATA、hCGAb、EmAb的存在会引起不明原因IVF妊娠流产率增高,从而降低了持续妊娠率,有必要对此类患者进行自身抗体的检测。     

抗核抗体阳性的不典型膜性肾病与V型狼疮性肾炎的临床与病理比较

目的:探讨抗核抗体(ANA)阳性的不典型膜性肾病(Atypical Membranous Nephropathy,AMN)与V型狼疮性肾炎(V-LN)的临床与病理特点,寻找对V-LN具有较高预测价值的临床与病理指标。方法:回顾性分析膜性肾病登记数据库中21例ANA阳性AMN患者、46例V-LN患者的临床与病理资料。结果:AMN组抗ds DNA抗体、抗Sm抗体阳性率及低补体血症、皮疹、关节炎、免疫系统异常发生率较V-LN组低(P均0.05)。在肾脏病理方面,AMN组患者肾组织中Ig A、C1q阳性率均不同程度低于V-LN组(P均0.01),PLA2R阳性率高于V-LN组(P0.01)。V-LN患者在基底膜部位比AMN组患者更易出现电子致密物沉积(P0.01)。结论:ANA阳性的AMN在临床表现、实验室及病理表现与V-LN相似,它可能是V-LN中较为隐匿的的一个亚型。     

系统性红斑狼疮患者血清CCL19的表达及临床意义

目的:研究系统性红斑狼疮(SLE)患者血清趋化因子配体19(CCL19)的表达情况,并分析其临床意义。方法:选取2015年07月～2017年12月重庆市开州区人民医院门诊及住院的SLE患者60例为研究对象,记为研究组。另取同期于我院接受体检的健康人员30例记为对照组。分别采用酶联免疫吸附法检测两组人员血清CCL19表达情况,同时检测各项免疫球蛋白指标,补体C3、C4、抗双链DNA水平情况,分析血清CCL19水平与免疫球蛋白水平、补体C3、C4、抗双链DNA水平的相关性。此外,分析SLE患者光过敏、关节炎以及继发干燥综合征情况与血清CCL19水平的关系。结果:研究组的血清CCL19水平明显高于对照组,组间对比差异有统计学意义(P 0. 05)。研究组的血清免疫球蛋白G(Ig G)、免疫球蛋白M(Ig M)、免疫球蛋白A(Ig A)、抗双链DNA水平均分别明显高于对照组,而C3、C4水平均明显低于对照组,组间对比差异有统计学意义(均P 0. 05)。经Preason法分析相关性发现:SLE患者的血清CCL19水平与Ig G、Ig MIgA抗双链DNA均呈正相关,而与C3、C4水平呈负相关(均P 0. 05)。有光过敏、关节炎以及继发干燥综合征的SLE患者血清CCL19水平均明显高于无光过敏、无关节炎、无继发干燥综合征患者,组间对比差异有统计学意义(均P 0. 05)。结论:SLE患者血清CCL19水平存在明显高表达,在有光过敏、关节炎以及继发干燥综合征的患者中水平更高,且与免疫球蛋白水平、补体C3、C4、抗双链DNA水平存在密切相关。CCL19可能是通过调节免疫球蛋白水平、补体C3、C4、抗双链DNA水平参与SLE的发生、发展过程,值得重视。     

Serological and histological study of lupus nephritis with special reference to anti-SSA antibody

Systemic lupus erythematosus (SLE) is a disease of unknown etiology in which many organs are damaged by deposition of pathogenic autoantibodies and immune complexes Clinically lupus nephritis occurs about 50% in SLE Many studies revealed the association between autoantibodies and lupus nephritis However, the pathogenetic role of autoantibodies in lupus nephritis remains obscure. To elucidate the pathogenetic role of anti-SSA antibody in lupus nephritis, 32 patients with SLE were evaluated by serological and histological methods. Enzyme-linked immunosorbent assay for anti-SSA antibody was developed for this study. It was confirmed that this assay was specific, did not detect autoantibodies other than anti-SSA antibody. The levels of anti-SSA antibody determined by this assay significantly correlated with the levels determined by double immunodiffusion (p less than 0.01). The level of anti-SSA antibody greater than or equal to 200 units was regarded as positive. The serum levels of antinuclear antibody, anti-DNA antibody, anti-RNP antibody, anti-SSA antibody, anti-SSB antibody, C3, and C4 were also determined. Renal biopsy materials were evaluated according to the WHO criteria, and activity index (AI), chronicity index (CI), and pathologic score (PS) were calculated according to Austin et al. The patients were divided into group A (AI greater than or equal to 4, n = 17) and group B (AI less than or equal to 3, n = 15) The levels of anti-DNA antibody were significantly higher in group A than in group B (p less than 0.05). The frequency of positive anti-SSA antibody in group A (70.6%) was greater than in group B (23.3%) significantly (p less than 0.05). However, there were no differences in the levels of anti-nuclear antibody, anti-DNA antibody, anti-RNP anti-body, anti-SSA antibody, anti-SSB antibody, C3, and C4 between group A and group B. Then these patients were divided into group I (anti-SSA greater than or equal to 200 units, n = 17) and group II (anti-SSA less than 200 units, n = 15). AI and CI were greater than in group I than in group II significantly (p less than 0.05). The frequency of pericarditis in group I (35.3%) was greater than group II (6.7%) (p = 0.061), but the frequencies of the other clinical manifestations were not different. AI was correlated with anti-DNA antibody significantly (p less than 0.01), but there were no correlations between other serological data and parameters.(ABSTRACT TRUNCATED AT 400 WORDS)     

Clinical and prognostic significance of antinuclear antibodies in primary antiphospholipid syndrome: A multicenter retrospective study

IntroductionThe antiphospholipid syndrome (APS) (1) is defined by the development of vascular thrombosis, or pregnancy morbidity in the presence of persistent antiphospholipid antibodies (aPL). Antinuclear antibodies (ANA) can be detected in primary APS patients without any clinical systemic autoimmune disease. The presence of ANA antibodies could confer a specific phenotype in primary APS.ObjectiveTo evaluate the characteristics of APS patients with antinuclear antibodies without other autoimmune disease (ANA positive APS patients) in comparison with primary APS without ANA or secondary APS patients with associated systemic lupus erythematosus (SLE).MethodsClinical and biologic data from 195 APS were retrospectively collected and patients were classified as primary APS with positive ANA (ANA-positive APS), primary APS without any ANA (ANA-negative APS), and SLE-associated APS (SLE-APS).ResultsFourty patients (21%) were classified into ANA-positive APS group, 77 (39%) in ANA-negative APS and 78 (40%) in SLE-APS. In ANA-positive APS patients, 20 patients (51%) had arterial thrombosis, 14 (41%) had veinous thrombosis and 19% had obstetrical complications. There was no difference between the three groups for the frequency of thrombotic manifestations and obstetrical complications. ANA-positive APS patients had more non-criteria manifestations than ANA-negative APS (48% versus 25%; P ≤ 0.01). ANA-positive APS had more triple aPL positivity (59% versus 18%; P < 0.001) and more thrombosis and obstetrical recurrences (63% versus 36%; P < 0.01) in comparison with ANA-negative APS patients. ANA-positive APS had more triple aPL positivity than SLE-APS patients (54% versus 33%; P < 0.05). ANA-positive APS and SLE-APS patients had similar clinical manifestations, and recurrences. Despite a limited follow-up (28 months (11–50)) none of the ANA-positive APS develop SLE. Antiplatelet and anticoagulant therapies were similar for the three groups. SLE-APS patients received more immunomodulatory therapies.ConclusionANA positivity in patients with APS enables to individualize a subset of patients with a more severe phenotype. Whereas the ANA positivity does not seem to be associated with the risk to develop SLE, prospective studies with a longer follow-up are necessary, in particular to evaluate the effect of additional therapies in this subset of APS.     

Doença de Kikuchi‐Fujimoto antes do diagnóstico de lúpus eritematoso sistêmico juvenil

Kikuchi‐Fujimoto disease (KFD) is a self‐limiting histiocytic necrotizing lymphadenitis of unknown origin. Of note, KFD was infrequently reported in adult systemic lupus erythematosus (SLE), with rare occurrence in childhood‐SLE (C‐SLE) patients. To our knowledge, the prevalence of KFD in the paediatric lupus population was not studied. Therefore, in a period of 29 consecutive years, 5,682 patients were followed at our institution and 289 (5%) met the American College of Rheumatology classification criteria for SLE, one had isolated KFD (0.03) and only one had KFD associated to C‐SLE diagnoses, which case was reported herein. A 12 year‐old female patient had high fever, fatigue and cervical and axillary lymphadenopathy. The antinuclear antibodies (ANA) were negative, with positive IgM and IgG herpes simplex virus type 1 and type 2 serologies. Fluorine‐18‐fluoro‐deoxy‐glucose positron emission tomography/computed tomography (PET/CT) imaging demonstrated diffuse lymphadenopathy. The axillary lymph node biopsy showed necrotizing lymphadenitis with histiocytes, without lymphoproliferative disease, compatible with KFD. After 30 days, she presented spontaneous regression and no therapy was required. Nine months later, she developed malar rash, photosensitivity, oral ulcers, lymphopenia and ANA 1:320 (homogeneous nuclear pattern). At that moment the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI‐2K) score was 10 and she was treated with prednisone (1.0 mg/kg/day) and hidroxychloroquine showing progressive improvement of hers signs and symptoms. In conclusion, KFD is a benign and rare disease in our paediatric lupus population. We also would like to reinforce the relevance of autoimmune diseases diagnosis during the follow‐up of patients with KFD.     

老年系统性红斑狼疮临床特点分析

目的：探讨老年系统性红斑狼疮（systemic lupus erythematosus,SLE）39例患者的临床表现、实验室检查及肾脏损害情况,与同期确诊的青年SLE40例患者的上述指标进行对比。方法：观察两组患者临床特点、血常规、自身抗体、蛋白尿、肾功能、活动指数（SLEDAI Score）、肾脏病理改变及治疗后感染情况。结果：老年组狼疮SLEDAI评分明显低于青年组,皮疹、狼疮脑病发生率和狼疮特异性抗体（抗ds-DNA）的阳性率显著低于青年组（P〈0.05）,但发热、关节炎高于青年组（P〈0.05）,肾脏是老年SLE的最常见累及脏器,且重型狼疮性肾炎（LN）（Ⅳ、Ⅴ型）的发病率与青年组发病的SLE无差别。老年组治疗1个月内继发感染率显著高于青年组（P〈0.05）。结论：老年SLE与青年SLE临床特征有许多不同之处,且起病隐匿,易误诊,治疗时并发症高于青年人,须谨慎用药。     

Appropriateness of laboratory tests in the diagnosis of inflammatory rheumatic diseases among patients newly referred to rheumatologists

IntroductionAutoantibody tests are commonly ordered when screening for rheumatic diseases. Rheumatoid factor (RF) and antinuclear antibody (ANA) have low positive predictive values in general practice. Overuse of diagnostic tests can result in an increase in unnecessary referrals, patient anxiety, and further costs.ObjectiveThe objective was to evaluate the utilization patterns, appropriateness, and associated costs of tests including ANA, extractable nuclear antibodies (ENA), anti-double stranded DNA (anti-dsDNA), RF, and HLA-B27 in patients referred to rheumatologists.MethodsA review was conducted of consecutive referrals (accepted and rejected) using university rheumatologists’ practices over one year. Inappropriate investigations, and associated costs were analyzed. Tests were considered appropriate if at least one criterion for a specific disease was provided.ResultsOf 638 referrals the most common reported reasons for referral were: spondyloarthropathies (SpA), rheumatoid arthritis (RA), and lupus (SLE). Prior to referral: 61% had undergone ANA testing at least once, ANA was repeated in one third; 19% had ENA and 21% had anti-dsDNA. 20% had ANA testing with no clinical indication. Half of ENA and anti-dsDNA testing was in the context of a negative ANA. RF was requested in 65% and in close to one third, there was no clinical suspicion of inflammatory arthritis.ConclusionDespite the recommendations by CRA Choosing Wisely Campaign, at least 50% of laboratory investigations, including RF, ANA, ENA, and anti-dsDNA, are inappropriately ordered. More selective ordering of the above tests would lead to marked cost reduction.     

Two cases of lupus cystitis complicated by lupus nephritis treated successfully with steroid therapy

In patients with systemic lupus erythematosus(SLE), interstitial cystitis(lupus cystitis) is an uncommon, but important manifestation. We report two Japanese patients with lupus cystitis. Case 1 was a 49-year-old woman diagnosed as having rheumatoid arthritis and membranous nephropathy. She was treated with prednisolone(5 mg daily). Case 2 was a 41-year-old woman also diagnosed as having rheumatoid arthritis previously and treated with a non-steroidal anti-inflammatory drug. Both cases presented abdominal pain, vomiting, dysuria and frequency of micturition. We diagnosed these cases as SLE on the basis of arthritis, renal disorder(proteinuria and hematuria), and positive antinuclear and anti-dsDNA antibodies. In addition, bilateral hydronephrosis was found in both cases. Thus, they were also diagnosed as probable lupus cystitis. The patients were treated with one cycle of methylprednisolone pulse therapy. Thereafter they were treated with 60 mg/day of prednisolone and their symptoms resolved promptly. Furthermore, no abnormal finding was found by abdominal ultrasonography and/or the intravenous pyelogram after therapy. Renal biopsies were performed and both cases showed lupus glomerulopathy (case 1: WHO class Vb, case II: WHO class IVb). Abdominal pain and/or dysuria, which is common in SLE patients, requires further examinations to evaluate the lupus cystitis.     

老年性系统性红斑狼疮的临床特点及预后

目的探讨老年性系统性红斑狼疮(SLE)的临床特点及预后。方法58例老年性SLE的主要临床表现、实验室检查、诊断、疗效及预后与非老年组76例对比分析。结果与非老年组比较,老年组浆膜炎、肺部病变、肌病、体重减轻、贫血、白细胞和血小板计数减少的发生率较高(P<0.05),发热、面颊皮疹、光过敏、口腔溃疡、脱发、雷诺现象、肾损害、神经精神症状、补体C3低下、抗ds-DNA(+)、抗SM(+)的发生率较低(P<0.05),初诊误诊率和男性发病率较高(P<0.05),治疗有效率较高,死因并非全是SLE。结论老年性SLE临床表现不典型、重症型较少、易误诊,应避免过度治疗。     

Antineutrophil cytoplasmic autoantibodies in patients with systemic lupus erythematosus recognize a novel 69 kDa target antigen of neutrophil granules

OBJECTIVE: Antineutrophil cytoplasmic autoantibodies (ANCA) were found in patients with systemic lupus erythematosus (SLE). Cathepsin G and lactoferrin were the major target antigens. However, some ANCA-positive sera did not recognize either of them. The present study was to investigate the unknown target antigens of ANCA in patients with SLE and their clinical significance. METHODS: Sera were collected from 72 patients with SLE. ANCA were detected in both indirect immunofluorescence and antigen-specific enzyme-linked immunosorbent assay (ELISA). Mixed neutrophil granules were separated from normal human peripheral neutrophils; soluble acid extracts in non-reducing conditions were used as antigens in western blot analysis to detect ANCA. RESULTS: SLE sera could blot a few bands. Interestingly, 14/72 (19.4%) sera recognized a novel 69 kDa protein band and 10/72 (13.9%) sera recognized the 55 kDa protein band, which might be bactericidal/permeability-increasing protein (BPI). The 69 kDa target antigen was different from the known target ANCA antigens such as cathepsin G and lactoferrin. Further study revealed that the percentages of patients with photosensitivity and oral ulcer in the anti-69 kDa autoantibodies-positive group were significantly higher than those in the anti-69 kDa autoantibodies-negative group (57.1%vs 10.3%, P < 0.005 and 50.0%vs 12.1%, P < 0.05, respectively). CONCLUSIONS: A 69 kDa protein in human neutrophil granules was identified as a novel target antigen of ANCA in patients with SLE. The anti-69 kDa autoantibodies might be associated with photosensitivity and oral ulcer in patients with SLE.     

The underlying diseases and follow-up in Taiwanese children screened by urinalysis

To date, the underlying diseases and follow-up of Taiwanese children screened by urinalysis have not been reported. The grading of urine abnormalities varied from grade A (microscopic hematuria only), grade B (light proteinuria only), grade C (light proteinuria and microscopic hematuria) to grade D (heavy proteinuria). From January 1991 to August 1998, 630 students, aged 6-15 years and with positive urinary screening, were admitted to our hospital for further evaluation. Of these, 573 students had confirmed abnormal findings, 298 were boys, 275 were girls, and 294 students received a renal biopsy and have had regular follow-up visits. This study was designed to retrospectively elucidate: (1) the relationship between grading of urine abnormality and underlying disease; (2) the relationships among hypertension, grading of urine abnormality, and underlying disease; (3) the underlying disease of low serum C3 level; and (4) to determine whether urinary screening progressively decreased the number of students with end-stage renal disease (ESRD) annually. The results show that glomerular nephritis (GN) is still one of the major causes of urinary abnormalities. The most-important secondary GN was systemic lupus erythematosus (SLE) with lupus nephritis. One-quarter of the patients fulfilled at least four of the revised American Rheumatology Association (ARA) criteria for SLE at first administration, while the others who fulfilled only two to three of the revised ARA criteria had gradually developing signs and symptoms of SLE at follow-up. The percentage of SLE patients amongst anti-nuclear antibody (ANA) positive children was 72%. Membranoproliferative GN is very rare. The distribution of hypertension was 8.2% in grade A, 10.7% in grade B, 9.7% in grade C, and 28.9% in grade D urinary abnormality. There were statistical differences between grade D and either grade A or B or C (P<0.05). Lower serum C3 levels were found only in a minority of patients, including those with SLE. In this series, focal segmental glomerular sclerosis (FSGS) and active class IV lupus nephritis patients were found early enough to receive methylprednisolone pulse plus cyclosporine A therapy. To date there have been only 2 cases (5%) of FSGS with impaired renal function, and none of the lupus nephritis patients are in the predialysis stage. In conclusion, GN is still the major cause of urinary screening abnormality. ANA study is indicated in all Chinese students with abnormal urinary screening. The correlations between the severity of proteinuria and hypertension showed more-severe proteinuria in patients with nephritis as well as in those with hypertension.     

血清抗核抗体阳性的不典型膜性肾病与狼疮膜性肾病及特发性膜性肾病的临床病理比较

目的 探讨血清抗核抗体（ANA）阳性的不典型膜性肾病（AMN）与狼疮膜性肾病（LMN）、特发性膜性肾病（IMN）的关系，寻找对诊断LMN具有较高预测价值的临床和病理学指标。 方法 2003年1月至2006年12月期间在北京协和医院住院并行肾活检，临床、病理资料保存完整的患者为对象。分组：AMN组（n = 28）：血清ANA滴度≥1∶80，少于4条美国风湿病学会（ARA）修订的系统性红斑狼疮分类标准，病理呈肾小球基底膜病变伴系膜增生和（或）免疫荧光C1q阳性；IMN组（n = 100）；LMN组（n = 45）。回顾性分析各组病例的临床表现、病理学特点。应用免疫组化法，对各组部分病例肾活检组织行IgG亚型染色，半定量分析染色强度。对各组部分病例肾活检组织行免疫荧光双染色（IgG-TRITC，C3-FITC），用激光扫描共聚焦显微镜观察肾小球沉积的IgG和C3的空间分布。 结果 (1)AMN组起病年龄（38±17）岁，女∶男比为2.5∶1，介于LMN和IMN之间。3组起病年龄差异有统计学意义（P < 0.01），AMN组女∶男比高于IMN组（P = 0.017）。AMN组血液系统异常、血抗SSA抗体阳性百分比较高（21.4%、40.7%）。(2)AMN组系膜增生、系膜区及内皮下电子致密物出现的百分比高于IMN组（P < 0.01）。(3)AMN和LMN组肾小球IgG3沉积占优势的百分比分别为78.9%、73.9%；IMN组IgG4沉积占优势的百分比为61.1%，差异均有统计学意义（IMN组与AMN、LMN组比较, P < 0.01）。(4)IMN组常出现IgG和C3在上皮下的共沉积现象，而在AMN和LMN组中少见。(5)在鉴别LMN和IMN的指标中，敏感性较高的有肾小球IgG4不占优势（91.3%）；特异性较高的有内皮下电子致密物（100.0%）、血抗SSA抗体（95.5%）、肾小球IgG3占优势（94.4%）。 结论 AMN的临床表现与IMN类似，而各项病理学特点，尤其是肾小球IgG亚型沉积特点与LMN更为接近。它有可能是狼疮肾炎中较为隐匿的一个亚型。