狼疮性肾炎临床与病理的关系

对55例狼疮性肾炎进行了病理组织学研究，其中26例为尸检病例，29例为肾穿刺材料；轻微病变型2例（3．6％），系膜增生型11例（20．0％），局灶型10例（18．2％），弥漫增生型17例（30．9％），膜型13例（23．6％）及增生硬化型2例（3．6％）。肾小球病变的多样性和非典型性，肾小球内巨块状免疫复合物沉积乃至毛细血管壁的白金耳样改变，纤维素样坏死，微血栓形成，小动脉炎，肾间质炎及苏木素小体形成等，均为狼疮性肾炎的常见病变，具有诊断意义。这些病变也是狼疮性肾炎活动进展的指征。     

58例狼疮性肾炎病理与临床分析

目的 分析肾活检及相关实验室检测结果，探讨狼疮性肾炎（LN）病理类型与临床表现的关系。方法 对58例LN患者进行肾活检及常规实验室检查。根据WHO 1982年标准进行病理分型，并分别进行活动性指数（AI），慢性指数（CI）和肾小管间质病变（TIL）评分。结果 病理类型以Ⅳ型LN最多，占38％，其次Ⅱ型（26％）和Ⅴ型（24％），Ⅱ型与Ⅲ型多表现为肾炎综合征，Ⅳ型和Ⅴ型多表现为肾病综合征。Ⅳ型和LN的血,高血压，肾功能不全发生率最高，其AI亦显著高于其他类型。LN肾间质受累以Ⅳ型最为显著。血Cr水平与肾间质受累程度呈正相关。与TIL0－1级相比，2－3级病程相对要长。1－2级者尿蛋白排泄较0级与3级为高。结论 LN的病理类型与临床表现有一定关系，根据其临床表现和实验室检查可大致推测其病理类型。肾活检对判断疾病活动性，指导治疗与估计预后有重要意义。     

狼疮性肾炎患者病理改变和肾小管功能障碍的关系

作者测定了２０例狼疮性肾炎（ＬＮ）患者和２０例正常人的尿酸化功能和血、尿渗透压。结果显示：ＬＮ患者的尿ＨＣＯ３－与正常对照组无显著性差异（Ｐ＞０．０５）；尿ＴＡ，ＮＨ４－和ＮＡＣ的排泄量，ＬＮ患者中病理改变呈多灶状肾小管萎缩组（Ａ组）显著低于对照组和局灶肾小管萎缩组（Ｂ组）（Ｐ＜０．０１）。     

狼疮性肾炎的病理形态发病机制及治疗

狼疮性肾炎(LN)传统地按照其肾活检组织形态进行治疗,近来研究认为单纯依靠形态学分类,而不触及病变的发生机制往往带来认识上的偏见.本文将已知LN的病理变化机制概括为4类:循环免疫复合物沉积;原位免疫复合物形成;血管炎性病变;血栓性微血管样病变.建议按照病变发生机制选择适当的免疫抑制药物(如激素、环磷酰胺、霉酚酸酯、普乐克复等),从而可以提高疗效,同时建议推广多靶点疗法,在减少各种免疫抑制荆剂量的条件下,多种药物并用,可以提高疗效,减轻副反应.     

男性狼疮性肾炎的临床和病理：附69例分析

目的：观察男性狼疮性肾炎（ＬＮ）的流行病学、临床表现、肾脏病理、免疫学异常、治疗及预后，与女性患者对比，以期提高对男性ＬＮ的认识。 方法：回顾分析６９例男性ＬＮ及同期４９５例女性患者的是、临床及随访资料。 结果：①男性患者占ＬＮ总数的１２．２６％，发病的高峰年龄为１５￣３５岁；②男性ＬＮ合并心脏、肺、肝、浆膜腔、中枢神经、血液系统病变较女性多见（或严重），而发热、并节肿痛、皮疹、雷诺征较少见；③根     

迟发狼疮性肾炎的临床与病理分析

目的:分析迟发狼疮性肾炎(LN)患者的临床与病理特点. 方法:21例发病年龄≥50岁,有肾脏损害的临床表现并行肾活检病理检查的LN患者作为迟发组,随机选取同期发病年龄<50岁的132例LN患者作为对照组.肾活检病理按照2003年ISN/RPS分型方案.回顾性分析迟发LN患者的临床与病理特点.结果:迟发组平均年龄(54.8±5.4)岁,对照组平均(28.7±7.5)岁;迟发组男性比例相对增多,女性与男性比例为6∶1,对照组为7.3∶1;高血压、肾功能损害的比例高于对照组,肾脏病理类型分布与对照组无差异;面部红斑、脱发的发生率低于对照组,白细胞减少的比例升高;血清A-dsDNA、抗心磷脂抗体阳性率、低C3血症的发生率较对照组高. 结论:迟发LN临床表现与对照组存在一定差异.     

重症活动性狼疮性肾炎的临床病理特征

重症活动性狼疮性肾炎(LN)进展迅速，病情凶险，如不及时治疗，病死率高。1993～2004年，我院收治38例重症活动性LN。现报告如下，并探讨其临床病理特点。     

弥漫增殖性狼疮性肾炎按血管病变为基础的病理分型探讨

目的：根据肾脏血管病变的特点，建立弥漫增殖型狼疮性肾炎的病理分型，评价其对判断病情，指导治疗及评价预后的意义。方法：根据肾活检病理肾小球毛细血管及间质血管病变特点第３４１例弥漫增殖型狼疮性肾炎为４组，弥漫增殖组（Ａ组１２８例）袢坏死组（Ｂ组８６例）袢坏死伴间质血管病变组（Ｃ组４８例），血栓形成组（Ｄ组７９例），回顾性分析各组患者临床表现，对不同免疫抑制治疗的的近期疗效及长期预后。结果：（１）四组患     

狼疮性肾炎的诊断及治疗

1概念 系统性红斑狼疮(SLE)是一类临床表现多系统、多器官损伤及血清中存在一种或多种自身抗体尤其抗-dsDNA抗体为特征的临床症候群,是典型的自身免疫性疾病.60%～80%的SLE患者可有肾脏受累的临床征象,表现有蛋白尿、血尿,或伴肾功能不全(即狼疮性肾炎,LN).LN是我国最常见的继发性肾炎,好发于育龄期女性.也可见于儿童、男性和老人,LN有种临床和病理类型,治疗应分别对待.     

Ⅴ型狼疮性肾炎的临床及病理研究

本文回顾性地分析了４４例经肾活检诊断为Ⅴ型狼疮肾炎患者的临床及病理资料，并与５０例Ⅳ狼疮性肾炎患者进行比较。Ⅴ型狼疮肾炎患者中，男７例，女３８例，干均年龄２９．６±９．２岁，肾活检光镜下表现为纯膜性改变者１８例，非纯膜性改变者２６例。纯膜型与非纯膜狼疮性肾炎临床表现的比较表明、纯膜型组起病年龄大于非纯膜型组，肾外表现相对较少（关节受累率３８％∶７３％，血液系受累３３％∶５０％），纯膜型组血清肌酐升高患者的比例（０％）明显低于非纯膜型组（１１％），而非纯胰回组与Ⅳ型狼疮组的临床表现无显著差异：在肾病综合征比例、蛋白尿程度、镜下血尿比例上各组之间差异不显著，病理上除肾小球的基本改变外，纯膜型组小管间质改变较轻，较少伴有间质大量淋巴细胞浸润，而非纯膜型多伴系增殖及系膜插入，同时伴明显间质淋巴细胞浸润，血管周围炎细胞浸润，小管上皮细胞脱落、坏死、萎缩及慢性硬化性改变。免疫学检查表明纯膜型组患者高ＩｇＧ血症程度、补体Ｃ３，Ｃ４下降幅度以及血清Ａｎｔｉ－ＤｓＤＮＡ阳性滴度均不及非纯膜型组。通过以上结果的比较，我们认为Ⅴ型狼疮肾炎是一组不均一的疾病，至少可分成纯膜型与非纯膜型两大亚型，治疗上应区别对待。     

Clinical and Laboratory Predictors of Distinct Histopathogical Features of Lupus Nephritis

The authors aimed to explore whether distinct clinical, serological, and urinalysis findings are associated with specific histological classes of lupus nephritis. Clinical and laboratory features were recorded at the time of clinical diagnosis from 297 consecutive patients with biopsy-confirmed lupus nephritis. Univariate and logistic regression analyses were performed and a risk score was developed to estimate the risk for developing different classes of lupus nephritis. Variables independently associated with class II included absence of malar rash, negative anti-dsDNA, and ≤5 urine leucocytes/high power field (hpf); with III/IV: age at nephritis diagnosis ≤32 years old, presence of musculoskeletal features, new-onset hypertension, positive anti-dsDNA, >5 urine leucocytes/hpf, creatinine >1.2 mg/dL, cellular casts >1/hpf, and absence of nephrotic range proteinuria; with V: age at nephritis diagnosis >32 years, malar rash, absence of musculoskeletal complaints or serum C3 hypocomplementemia, nephrotic range proteinuria, and ≤9 urine erythrocytes/hpf. A risk predictive score of specific histological classes was calculated for each patient. Associations between 2, 3 or more risk factors with specific histological classes were also revealed [Odds ratios (95% confidence interval) (≥2 risk factors) was 6.7 (2.8–17.4) for class II nephritis, 15.6 (5.1–47.8), and 8.2 (3.6–19.0) for classes III/IV and for class V, respectively (≥3 risk factors)]. The identification of independent factors associated with specific classes of lupus nephritis can provide guidance in selecting specific therapeutic modalities, particularly in cases in which renal biopsy is contraindicated.     

Lupus Nephritis

Lupus nephritis (LN) is one of the common manifestations of systemic lupus erythematosus. Kidney biopsy remains a mainstay of LN diagnosis, which is usually prompted by abnormal urinary sediment, proteinuria, or elevated creatinine. New International Society of Nephrology/Renal Pathology Society classification of LN tends to remove some of the ambiguities of World Health Organization classification and results in better categorization of patients. Although prognosis of LN has improved with the combined use of cytotoxic and steroid therapy, up to 20% of these patients progress to renal failure. Moreover, toxicity of the current regimens remains a major concern. Last few decades have seen a tremendous progress being made in understanding the pathogenesis of LN, but a little has been added to armamentarium against LN, leaving physicians with a few choices. Fortunately, with unfolding of molecular processes involved in disease pathogenesis, new targets for drug therapy have emerged. Whether these medications will prove to be more efficacious and less toxic remains a matter of debate and will be answered by several ongoing trials and future studies.     

15例表现为恶性高血压的IgA肾病临床与病理分析

恶性高血压(MHT)常危及心、脑、肾等重要脏器,据统计,未获得有效治疗的MHT患者存活1年者不足25%,而存活5年以上者仅有1%[1].在MHT的病因中,15%为慢性肾小球肾炎[2],而在慢性肾小球肾炎中以IgA肾病占首位[3].现将我院近6年来,以MHT为主要表现的15例IgA肾病患者的临床资料进行回顾性分析,报道如下.     

静脉滴注免疫球蛋白治疗狼疮性肾炎并发感染的临床观察

目的 :探讨采用静脉滴注免疫球蛋白 (IVIG)治疗狼疮性肾炎 (LN)并发感染的疗效及适应证。方法 :13例LN并发感染患者 ,采用IVIG 4 0 0mg/ (kg·d) ,连用 3～ 5d ,此后给予泼尼松 (pred) 1mg/ (kg·d) ,4周后 ,按系统性红斑狼疮活动指数比较治疗前后病情控制情况、2 4h尿蛋白、尿红细胞数、C3 、C4、抗ds DNA抗体、血小板数以及抗核抗体。结果 :IVIG治疗 4周后 ,13例肺部感染全部治愈 ,上述指标于治疗前、后均有显著或非常显著差异 (P <0 .0 5或P <0 .0 1) ,5 / 13例ANA转阴性。结论 :IVIG联合 pred治疗LN并发感染是一有效治疗措施 ,尤其对LN并发感染并有血小板明显减少、体质差 ,暂时无法使用大剂量甲泼尼龙及 /或免疫抑制剂治疗的患者更为适宜     

Clinical and Serologic Features in Patients With Incomplete Lupus Classification Versus Systemic Lupus Erythematosus Patients and Controls

Objective

Incomplete lupus erythematosus (ILE) involves clinical and/or serologic manifestations consistent with but insufficient for systemic lupus erythematosus (SLE) classification. Because the nature of ILE is poorly understood and no treatment recommendations exist, we examined the clinical manifestations, medication history, and immunologic features in a diverse collection of ILE and SLE patients.

Methods

Medical records of subjects enrolled in the Lupus Family Registry and Repository were reviewed for medication history and American College of Rheumatology (ACR) classification criteria to identify ILE patients (3 ACR criteria; n = 440) and SLE patients (≥4 ACR criteria; n = 3,397). Participants completed the Connective Tissue Disease Screening Questionnaire. Anticardiolipin and plasma B lymphocyte stimulator (BLyS) were measured by enzyme‐linked immunosorbent assay, antinuclear antibodies (ANAs) by indirect immunofluorescence, and 13 autoantibodies by bead‐based assays.

Results

On average, ILE patients were older than SLE patients (46.2 years versus 42.0 years; P < 0.0001), and fewer ILE patients were African American (23.9% versus 32.2%; P < 0.001). ILE patients exhibited fewer autoantibody specificities than SLE patients (1.3 versus 2.6; P < 0.0001) and were less likely to have ANA titers ≥1:1,080 (10.5% versus 19.5%; P < 0.0001). BLyS levels were intermediate in ILE patients (controls < ILE; P = 0.016; ILE < SLE; P = 0.008). Pericarditis, renal, or neurologic manifestations occurred in 12.5% of ILE patients and were associated with non–European American race/ethnicity (P = 0.012). Hydroxychloroquine use increased over time, but was less frequent in ILE than SLE patients (65.2% versus 83.1%; P < 0.0001).

Conclusion

Although usually characterized by milder symptoms, ILE manifestations may require immunomodulatory treatments. Longitudinal studies are necessary to understand how ILE affects organ damage and future SLE risk, and to delineate molecular pathways unique to ILE.

     

Treatment of Lupus Nephritis

The treatment of lupus nephritis has evolved over the past few decades. Standard practice is to define the first 6 months of therapy as an induction phase, during which the goal of therapy is to achieve renal remission, usually with bolus intravenous infusions of the cytotoxic cyclophosphamide or the immunosuppressant mycophenolate mofetil (MMF). Following induction, therapy is continued, with some decrease in aggressive dosing for a more prolonged period of time—typically 24 months—that is aimed at preventing renal flares and smoldering disease, which could lead to continuous deterioration of renal function. During maintenance, the usual therapeutic option is immunosuppression with MMF or azathioprine. In recent years, MMF has been increasingly replacing intravenous cyclophosphamide as an initial standard of care. The current paper reviews data on these treatment strategies and suggests a possible treatment algorithm for clinical care.     

男性狼疮性肾炎的临床研究

目的探讨男性狼疮性肾炎的临床特点并与女性狼疮性肾炎患者进行比较。方法收集2004年1月—2008年9月住院的31例男性狼疮性肾炎患者和同期263例女性患者的临床资料进行统计学对比分析。结果（1）在肾脏受损方面：男性患者肾损害发生早且有较严重的肾损害;（2）肾外临床表现：男性关节疼痛、发热、多浆膜腔积液多于女性,而女性则以面部红斑、光过敏、雷诺现象、脱发为常见;（3）在实验室检查方面：男性有较严重的贫血、血小板减少而自身抗体阳性率、阳性滴度较低。结论男性与女性狼疮性肾炎的临床表现有较明显差别,临床上要注意男性狼疮性肾炎的早期诊断,及时治疗。