不同性别系统性红斑狼疮的临床特征分析

系统性红斑狼疮 (ＳＬＥ)是常见病 ,在我国发生率高达0 .1% ,肾脏损害是其显著的特点 ,其发病有着明显性别差异 ,男女的比例为 1∶5～ 12 〔1,2〕。据文献报道 ,男性ＳＬＥ的临床表现、治疗及预后均有别于女性患者〔2～ 4〕。作者比较分析了收集到的 2 40例女性 ,32例男性ＳＬＥ患者起病及进展期的临床表现、自身抗体分布以及首始起病时的病理分型 ,以探讨两性ＳＬＥ的临床特点。资料和方法1　病例来源　 1983年～ 1999年我院收治的住院患者 ,以及作者在进修期间收集到的住院患者 ,女性 2 40例 ,男性 32例 ,发病年龄 5岁～ 78岁 ,全部 2 72…     

重症肌无力血清抗乙酰胆碱受体抗体和抗突角前膜抗体监测的…

用ＡＢＣ－ＥＬＩＳＡ法对５０例重症肌无力（ＭＧ）病人血清中抗乙酰胆碱受体抗体（ＡｃｈＲａｂ）和抗突触前膜抗体（ＰｒＭａｂ）进行检测，结果发现，（１）ＭＧ患者抗体总阳性３７例（阳性率７４％），其中ＡｃｈＲａｂ阳性３３例（６６％），ＰｒＭａｂ２９例（５８％），二者均显示重症患者阳性率高于轻症。（２）血清中两种抗体滴度重症患者均明显高于轻症，胸腺肿瘤ＡｃｈＲａｂ滴度明显高于胸腺增生病例，抗体滴度与胸腺病     

重症肌无力血清抗乙酰胆碱受体抗体和抗突触前膜抗体监测的临床意义

用ＡＢＣ－ＥＬＩＳＡ法对５０例重症肌无力（ＭＧ）病人血清中抗乙酰胆碱受体抗体（ＡｃｈＲａｂ）和抗突触前膜抗体（ＰｒＭａｂ）进行检测。结果发现：①ＭＧ患者抗体总阳性３７例（阳性率７４％），其中ＡｃｈＲａｂ阳性３３例（６６％），ＰｒＭａｂ２９例（５８％），二者均显示重症患者阳性率高于轻症。②血清中两种抗体滴度重症患者均明显高于轻症，胸腺肿瘤ＡｃｈＲａｂ滴度明显高于胸腺增生病例，抗体滴度与胸腺病理关系呈肿瘤＞萎缩或正常＞增生。③ＡｃｈＲａｂ和ＰｒＭａｂ高度相关。结果表明ＡｃｈＲａｂ与ＭＧ病情轻重和胸腺病理有一定关系，可用于ＭＧ的病情监测，ＰｒＭａｂ同样可以作为ＭＧ的一项免疫学诊断指标，有重要的临床应用价值。     

狼疮性肾炎中抗内皮细胞抗体和抗心磷脂抗体的关系

目的 进一步探讨狼疮性肾炎（ＬＮ）中抗内皮细胞抗体（ＡＥＣＡ）和抗心磷脂抗体（ＡＣＡ）的关系。方法 采用ＥＬＩＳＡ方法对５８例ＬＮ患者血清进行了ＡＥＣＡ和ＡＣＡ检测，并应用免疫印迹方法对ＡＥＣＡ的抗原进行分析。结果 在狼疮性肾炎中ＡＥＣＡ与ＡＣＡ阳性率分别为３６．２％和３９．７％，在２３例ＡＣＡ阳性患者中，１７例ＡＥＣＡ阳性，而３５例ＡＣＡ阴性患者中只有４例ＡＥＣＡ阳性，两者比较具有显著性差异（Ｐ     

血清前列腺特异性抗原的BA－ELISA测定法的建立及临床应用

建立一种灵敏的生物素－亲和素酶联免疫吸附测定法（ＢＡ－ＥＬＩＳＡ）用于血清前列腺特异性抗原（ＰＳＡ）的测定。用自制ＰＳＡ免疫家兔制备抗ＰＳＡ血清，用亲合层析技术纯化抗ＰＳＡ抗体，将生物素－亲和素系统引入普通ＥＬＩＳＡ以增加其灵敏度。本法灵敏度０．２μｇ／Ｌ，工作范围０．２～５０μｇ／Ｌ，批内变异系数２．８％～５．０％，批间变异系数４．０％～９．６％，回收率９４．６％。本法与血清中其它成分无交叉反应。本法测定９２例正常男性血清ＰＳＡ水平为１．１０±１．１５μｇ／Ｌ，４６．５％（３１／６７）的前列腺增生症患者及９１．１％（４１／４５）的前列腺癌患者血清ＰＳＡ水平升高。本法快速、重复性好、特异性高，能够达到常用的放免法的灵敏度，可用于临床血清ＰＳＡ浓度的测定     

前列腺癌患者血清及尿液唾液酸测定的意义

为观察前列腺癌患者血唾液酸（ＳＡ）与病情、疗效及血清ＰＳＡ的关系，按血ＰＳＡ高低将病人分为３组：Ａ组（治疗前，１７例）血ＰＳＡ≥５０μｇ／Ｌ，７０７％为临床Ｃ、Ｄ１、Ｄ２期，病情进展，前列腺症状或骨痛明显。Ｂ组（治疗前，６例）血ＰＳＡ８～３７μｇ／Ｌ，临床分期为Ｃ、Ｄ期，占５０％。Ｃ组（治疗后，８例）血ＰＳＡ＜４μｇ／Ｌ，除临床Ｃ期１例，余均为临床Ｂ期术后，病愈或明显好转。血ＳＡ均值，Ａ组为３０８ｍｍｏｌ／Ｌ，大于Ｂ组２８１ｍｍｏｌ／Ｌ及Ｃ组２１４ｍｍｏｌ／Ｌ；Ａ、Ｃ间有非常显著性差异（Ｐ＜０００１），尿ＳＡ结果同血ＳＡ，Ｐ＜００５。此外，血ＰＳＡ与血ＳＡ（或尿ＳＡ）密切相关，Ｐ＜００１。同为阳性（或阴性）一致率血ＳＡＰＳＡ为８１６％，尿ＳＡＰＳＡ为７２２％。本实验所用“一步法”简便快速，血ＳＡ敏感性１０００％，特异性７８１％，不需进口试剂或设备，有助于前列腺癌的检出与病情疗效追踪观察。     

微型结节性多动脉炎患者血清抗髓过氧化物酶抗体的检测意义

抗髓过氧化物酶（抗ＭＰＯ）抗体是抗中性粒细胞浆抗体（ＡＮＣＡ）中的一种，其对结节性多动脉炎（ＰＡＮ）的诊断价值日益受到重视。我们应用ＥＬＩＳＡ方法对１９例ＰＡＮ患者血清进行检测，６／１９例抗体阳性（３２％），其中５例临床表现合并肺、肾损害，１例合并皮肤、肾脏病变；另外６例中的１例动态监测，当病变处于急性活动期时，抗ＭＰＯ抗体阳性；而病变趋于慢性化及临床症状趋于缓解时，抗ＭＰＯ抗体阴性。此外，对２２     

男性不育精浆抗弓形虫抗体检测结果的140例临床分析

为了探讨弓形虫感染对男性生殖的影响，我们随机选择了１４０例男性不育病人，对其精浆进行了抗弓形虫抗体ＩｇＧＩＨＡ法检测和抗精子抗体（ＡＳＡ）ＥＬＩＳＡ法检测，检测结果有３８例抗弓形抗体阳性，阳性率为２７％，明显高于我国正常人群的平均感染率，表明弓形虫感染可能是引起男性不育的一个因素，并对弓形虫感染引起男性不育的途径和发病机理进行了探讨。     

高渗氯化钠对心钠素及内源性洋地黄样物质的影响

比较３０例硬膜外阻滞择期手术病人，输入７．５％ＨＳ和５％ＧＳ后血浆ＡＮＰ、ＥＤＬＳ及血流动力学的变化。输入ＨＳ后ＡＮＰ明显升高，ＥＤＬＳ短暂降低，６０ｍｉｎ完全恢复，主动脉顺应性快速增加。输入ＧＳ后ＡＮＰ有增加趋势，但无统计学意义。ＥＤＬＳ显著降低，６０ｍｉｎ时仅为基础值的。ＴＰＲ持续增加，ＣＯ和ＳＶ短暂降低，并伴有血压明显下降。揭示ＨＳ可明显刺激ＡＮＰ分泌，减缓ＥＤＬＳ变化。     

肺癌患者血清与支气管肺泡灌洗液四项放免肿瘤指标同步检测的结果 …

对３８例肺癌患者的血清和支气管肺泡灌洗液（ＢＡＬＦ）同步进行ＣＥＡ，ＣＡ－５０，ＡＦＰ，ＳＦ的检测。结果显示：肺癌ＢＡＬＦ的ＣＥＡ浓度测值７３％明显高于血清中ＣＥＡ浓度，ＣＡ－５０浓度测值６８％，高于血清ＳＦ９２％明显低于轿清中测值，ＡＦＰ在肺癌患者无论血清及灌洗液中，阳性率均低于７％，但仍是ＢＡＬＦ的浓度测值敏感于血清，提示支气管肺泡灌洗液的ＣＥＡ，ＣＡ－５０与血清的同步检测是目前肺癌早期诊断的     

48例晚发型系统性红斑狼疮的临床分析

目的：分析48例晚发型系统性红斑狼疮患者的临床特征。方法：回顾性分析1995年7月～2008年6月间解放军174医院及解放军175医院住院及门诊的48例发病年龄≥50岁的SLE患者的年龄、性别、诊断时间、临床表现及诊治经过等特点,并与随机抽取的同期100例发病年龄〈50岁的SLE患者进行比较。结果：晚发组女性与男性患者的比例显著低于对照组（P〈0.01）;发病至确诊的间隔时间略长于对照组,但差异尚无统计学意义。晚发组关节炎、蝶形红斑发生率显著低于对照组（P〈0.01,P〈0.05）,而发热、高血压发生率略高于对照组,但差异无统计学意义;晚发组重要脏器受累较少（P〈0.05）,肾衰竭的发生率较低（P〉0.05）,增生性狼疮性肾炎的发生率低于对照组（P〈0.05）,尤其是弥漫增生性肾炎的发生率更低（P〈0.01）;晚发组平均每个病人的严重复发次数少于对照组（P〈0.01）;晚发组低补体血症较少见（P〈0.01）,而类风湿因子阳性率高（P〈0.05）;晚发组需要接受大剂量糖皮质激素（P〈0.01）及免疫抑制剂（P〈0.01）治疗的患者较少。结论：晚发型SLE患者病情相对较轻,较少出现严重复发,但易误诊,临床医师应加强对这一类型SLE的认识。     

70例男性系统性红斑狼疮患者临床及肾脏病理分析

目的：探讨男性系统性红斑狼疮（SLE）患者的临床及肾脏病理特点。方法：回顾性分析了我院2003年4月～2009年4月收治的70例男性SLE患者的临床表现及肾脏病理,并对部分患者进行了随访。结果：70例男性SLE患者年龄16岁～72岁,平均（34.8±14.1）岁,临床表现肾病综合征25例、慢性肾炎19例、急性肾衰竭8例、隐匿性肾炎14例,以及尿常规检查和肾功能正常者4例。42例患者进行了肾活检,肾脏病理结果为Ⅱ型3例、Ⅲ型5例、Ⅳ型20例、Ⅴ型5例,Ⅲ＋Ⅴ型4例,Ⅳ＋Ⅴ型5例。在肾病综合征患者中21例进行了肾活检的,病理类型分别为Ⅲ型1例、Ⅳ型9例、Ⅴ型4例、Ⅲ＋Ⅴ型2例、Ⅳ＋Ⅴ型5例;在8例急性肾衰竭患者中,7例进行了肾活检,病理均为Ⅳ型。结论：男性SLE在各个年龄段均可患病,临床和肾脏病理表现多样,多数患者病情较重,但也有部分患者肾脏损害较轻。     

Evans syndrome and systemic lupus erythematosus: clinical presentation and outcome

ObjectiveTo review the clinical, laboratory and outcome features of Evans syndrome (ES) in systemic lupus erythematosus (SLE) patients.MethodsWe reviewed the charts of 953 SLE patients followed up regularly at our service. ES was defined as the presence of hemolytic anemia and thrombocytopenia concomitantly or sequentially. Clinical and laboratory manifestations occurring during the disease course, as well as concomitant diseases and survival was carefully reviewed.ResultsWe identified ES in 26 of 953 (2.7%) SLE patients. Twenty-three were women with mean age at SLE diagnosis of 25.7 years. Four (15%) patients had disease onset before the age of 16. In the majority of patients (92%), immune thrombocytopenia and AIHA appeared simultaneously at the beginning of SLE. Active features of SLE were a frequent finding concomitant to ES, especially arthritis (77%), malar rash (61.5%), photosensitivity (57.6%), oral ulcers (34.6%), nephritis (73%), serositis (54%), neuropsychiatric (19%) and pulmonary (15%) manifestations. In addition to this multisystemic disease, 34.6% of our patients had an association with another autoimmune disease such as antiphospholipid syndrome. Recurrence of ES was observed in only four (15%) patients. After follow-up time of 8.72 years, 19 patients (73%) were in remission and seven (27%) patients died.DiscussionES is a rare manifestation in SLE, occurring in patients with severe multisystemic SLE manifestations. Treatment strategies frequently used in SLE contribute to longer disease remission and less frequent exacerbation than observed in the general population with ES.     

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。     

Systemic lupus erythematosus: A follow-up study of Japanese patients with end-stage renal failure treated with haemodialysis

Summary: In order to explore the clinical course of Japanese patients with systemic lupus erythematosus (SLE) in end-stage renal failure, the clinical findings from 26 patients who had received haemodialysis were analysed. Each patient was followed for 72 months from the onset of clinical lupus nephritis to the initiation of haemodialysis. In most patients, renal disease progressed to end-stage renal failure despite clinical quiescence of SLE, which remained inactive throughout haemodialysis treatment. Seven patients (27%) had clinically active SLE with high dose prednisolone (mean; 49.3 mg per day) at initiation of haemodialysis. These patients had relatively rapid progression of their renal failure and 2 patients died within 1 month of their first haemodialysis. During the follow-up period from starting haemodialysis for an average of 44 months, most patients received ongoing haemodialysis while their SLE remained clinically inactive. Six patients (23%) died, 5 of those within 1 month from starting hemodialysis. the results of this long-term follow up of a large number of haemodialysis patients with lupus nephritis indicate that: (i) most patients with lupus nephritis undergoing haemodialysis have an excellent survival rate; and (ii) patients with active SLE at initiation of haemodialysis have a high mortality rate (within 1 month). We therefore conclude that more effective treatment for SLE in the presence of renal failure is required for these patients.     

Clinical and genetic characters of 8 Chinese children with ADCK4-associated glomerulopathy

Objective To investigate the clinical and genetic character of Chinese children with the aarF domain containing kinase 4 (ADCK4)-associated glomerulopathy. Methods Applying next generation sequencing to detect possible gene mutation(renal disease associated monogene was pooled as one panel) in 69 children with steroid-resistant nephrotic syndrome (SRNS) or persistent proteinuria of unknown origin. Sanger sequencing was used to confirm the significant mutations found in the children and to validate these mutation sites in their patients. Using online software (PolyPhen2, SIFT, Mutation Taster) to predict whether the detected missense mutations were disease causing or not. Collecting and analyzing clinical data of children with ADCK4-associated glomerulopathy, which included onset age, clinical manifestation, and renal pathology. Results The ADCK4 gene mutation was detected in 8 children with a positive rate of 11.6% (8 out of 69), among which 3 patients carried homozygous c.748G>C mutation, 3 patients carried homologous c.737G>A mutation, 1 patient carried compound heterozygous mutation(c.748G>C and c.737G>A), and 1 patient carried compound heterologous mutation(c.551A>G and c.737G>A). Collectively, there were only 3 mutation sites found in total 8 patients, in which the mutation sites of c.748G>C and c.737G>A had high detection frequency in these 8 patients. These 3 mutation sites were all missense mutation which were predicted to be disease causing by online software and not reported before. The average onset age was 6.5 years (2 years-11.75 years). Four patients presented with SRNS and the other 4 presented with persistent proteinuria. All 8 patients had no extrarenal manifestation, renal biopsy revealed focal segmental glomerulosclerosis (FSGS) in most patients, among which 3 cases had gone to end-stage renal disease (ESRD) at disease onset, and 2 cases progressed to ESRD 2 and 5 years after onset respectively. Seven patients had received glucocorticoid and /or immunosuppressive drug while only one patient getting partial response. All 8 patients were treated with large amount of coenzyme Q10 (15 mg?kg-1?d-1) after definite diagnosis of ADCK4 mutation-some patients had acquired encouraging curative effect. Conclusions ADCK4-associated glomerulopathy is not rare especially in the children with SRNS. The onset age is relatively old and the extrarenal manifestation is less common. FSGS is a main pathology type. Patients usually have no response to immunosuppressive therapy, but may benefit from addition of large amount of coenzyme Q10. Some patients may only manifest with insidious proteinuria, causing the early diagnosis to be difficult, which deserves more attention. Three new missense mutations expand disease causing mutation repertoire of ADCK4 gene, among which the two sites of c.748G>C and c.737G>A may be mutation hotspot of ADCK4-associated glomerulopathy in Chinese population, and need further study.     

Colony-Stimulating Factor-1: A Potential Biomarker for Lupus Nephritis

A noninvasive means to predict the onset and recurrence of lupus nephritis (LN) before overt renal injury is needed to optimize and individualize treatment. Colony-stimulating factor-1 (CSF-1) is expressed by kidney tubules at the onset of LN, increases with disease progression, and spills into the circulation in lupus-prone mice. We tested the hypothesis that amplified expression of CSF-1 detected in the serum or urine correlates with intrarenal CSF-1 expression and histopathology (increased macrophage accumulation, activity indices) and clinical kidney disease activity and predicts the onset and recurrence of nephritis in patients with systemic lupus erythematosus (SLE). We found increased serum or urine CSF-1 levels in patients with cutaneous, serositis, and musculoskeletal disease; however, the increase in CSF-1 levels was far greater in LN. Moreover, an elevation in serum or urine CSF-1 levels correlated with increasing intrarenal CSF-1 expression and histopathology. By longitudinally tracking patients, we found that elevated serum CSF-1 heralded the initial onset of disease, and a rise in serum or urine CSF-1 predicted recurrences of LN before clinical evidence of glomerular dysfunction and conventional serologic measures, even in patients with other manifestations of SLE. These findings indicate that serial monitoring for a rise in serum or urine CSF-1 levels in patients with SLE reflects kidney histopathology and may predict renal disease activity and the onset and recurrence of LN more accurately than conventional laboratory measures.     

Clinical and therapeutic studies in mesangial immunoglobulin a glomerulonephritis

A long-term clinical and therapeutic study was performed in 47 patients with mesangial IgA glomerulonephritis. The male to female ratio was 2.9∶1. An episode of gross haematuria or the incidental discovery of asymptomatic microscopic haematuria with associated mild proteinuria heralded the apparent onset of renal disease. At the onset of observation 18 patients (38.2%) had high blood pressure. Other 17 patients developed hypertension during observation. Anaemia was uncommon. No essential abnormalities in serum protein and lipid patterns were found. Twenty-nine patients (61.6%) had higher levels of serum immunoglobulins—most frequently of IgA (42.5%). Twenty-two patients had low serum C₃ levels (46.8%). The percentage of patients with renal failure increased from 21.2 to 36.1 during observation. Male sex, hypertension, proteinuria higher than 2 g/24 h, elevated ESR, high serum IgA levels, longer duration of the disease and older age of patients suggest an unfavourable outcome. Long-term treatment with a combination of azathioprine/acenocumarol, or indomethacin, or levamisole has no effect on the clinical manifestation and evolution.     

Silent renal disease in systemic lupus erythematosus

Several recent studies have focused on the discrepancy between lupus nephropathy and clinical renal involvement and, consequently, question the relevance of renal biopsy in these patients. We analyze the clinical characteristics, histological renal findings and subsequent course of patients with silent renal disease. Renal biopsy was performed in 15 patients with systemic lupus erythematosus (SLE) who had no clinical signs of renal involvement (no urinary sediment abnormalities, absence of proteinuria and serum creatinine less than 1.3 mg/dl). All biopsies were classified according to a modified classification proposed by the WHO. Six cases (40%) showed no histological or immunofluorescence changes (type I), 7 (47%) had mesangial nephropathy (3 type IIa and 4 type IIb) and 2 (13%) had focal proliferative glomerulonephritis (type III). None of the patients had previous evidence of neurological abnormalities. Patients with type I only had arthritis, skin lesions and Raynaud's phenomenon. By contrast, 7 patients with histological renal involvement had serositis or hemolytic anemia. All cases with silent nephropathy were treated with steroids and showed a benign clinical course with stable renal function and absence of urinary abnormalities during follow-up. We concluded that in the absence of clinical renal abnormalities, renal involvement is not uncommon in SLE. We believe that a renal biopsy should be performed mainly in those SLE patients presenting with clinical manifestations other than arthritis or cutaneous lesions since this policy may allow detection of significant silent renal injury.     

系统性红斑狼疮并发继发性抗磷脂综合征肾损害11例临床病理分析

目的 分析系统性红斑狼疮（SLE）并发继发性抗磷脂综合征（APS）肾损害的临床病理表现,旨在提高对该类疾病的认识。 方法 回顾性分析北京协和医院2000年至2010年期间确诊SLE并发继发性APS（SLE伴APS）并行肾组织学检查的11例患者的资料,分析其临床病理特点,并比较其和SLE不伴APS患者在肾损害的临床病理及预后上的差异。 结果 11例SLE伴APS患者均有肾脏受累,突出表现为高血压（54.5%）、大量蛋白尿（≥3.5 g/d）（72.7%）和肾功能异常（45.5%）。SLE伴APS患者的舒张压、平均动脉压以及肾小球滤过率（eGFR）均明显高于SLE不伴APS患者（均P < 0.05）。8例（72.7%）SLE伴APS患者存在肾内血管的“血管闭塞性表现”,即符合抗磷脂综合征肾病（APSN）的病理表现,包括肾小血管、肾小球毛细血管血栓形成以及肾小动脉内膜增生、局灶性肾皮质萎缩、肾小管甲状腺样化,其中慢性APSN表现5例（45.5%）,急性APSN表现4例（36.4%）（其中1例同时有急性和慢性表现）;其APSN的发生率以及急性APSN的发生率明显高于SLE不伴APS患者（P < 0.05）。 结论 SLE并发APS肾损害患者除狼疮肾炎外,多并发APSN,临床上高血压和肾功能异常更为突出。