100例新月体肾炎的免疫病理分型及临床病理分析

目的：了解新月体性肾炎的免疫病理分型及其临床病理特点。方法：对我科近10年来经肾活检确诊的100例新月体性肾炎进行回顾性分析,对患者血清进行抗中性粒细胞胞浆抗体（ANCA）和抗肾小球基底膜（GBM）抗体的检测。结果：100例患者中21％为抗GBM抗体型,其中6／21例同时合并ANCA阳性;47％为免疫复合物型,其中9／47型ANCA阳性;32％为少免疫沉积型,其中17／32例为ANCA阳性小血管炎。3种类型相比,抗GBM抗体型以青年男性为主,多有少尿或无尿（P＜0．05）,肾小球受累受为广泛（P＜0．001）,预后差（P＜0．001）。免疫复合物型以中青年为主,多表现为肾病综合征（P＜0．01）,强化免疫抑制治疗有效。少免疫沉积型以中老年为主,有多系统受累（P＜0．05）,治疗效果相对较好。结论：我国新月体肾炎中虽仍以免疫复合物型为主,但抗GBM抗体型和ANCA阳性小血管炎并不少见。肾活检免疫病理和血清自身抗体的联合应用对新月体肾炎进行分类更接近病因学诊断。     

乙型肝炎病毒相关性肾炎临床病理分析

我国是乙型肝炎病毒（HBV）流行的高发区[1],HBV感染可引起乙型肝炎病毒相关性肾小球肾炎（hepatitis B virus associated glomerulo nephritis,HBV-GN）[2,3]。目前国内外学者认为[4],HBV感染与多种病理类型肾炎相关,如膜性肾病（HBVMN）、膜增生性肾小球肾炎（HBV-MPGN）、系膜增生性肾小球肾炎（HBV-MsPGN）、IgA肾病（HBV-IgA）     

大鼠新月体肾炎中的细胞增殖与凋亡

目的探讨大鼠新月体肾炎时肾脏细胞凋亡与细胞增殖。方法分别用免疫组化（ＰＣＮＡ）和原位末端标记法检测增殖和凋亡的细胞。结果浸润肾脏的巨噬细胞及肾脏固有细胞，包括肾小管上皮细胞均发生凋亡，在细胞性、细胞－纤维素性新月体中及肾小管间质损害严重处凋亡明显，凋亡的肾小管上皮细胞数与肾小管病理损害程度高度相关。结论大鼠新月体肾炎时肾脏巨噬细胞和固有细胞均发生凋亡，凋亡可能在进行性肾损害中起着重要作用     

肾小球肾炎合并急性间质性肾炎的临床病理分析

目的 探讨肾小球肾炎合并急性间质性肾炎(AIN)患者的临床和病理特点。 方法 回顾性分析21例经肾活检证实的肾小球肾炎合并AIN患者的资料，以同期无肾小球疾病的AIN患者 35例作为对照。 结果 肾小球肾炎合并AIN占同期AIN的37.5%。β内酰胺类抗生素、中药是引起AIN的主要病因。76.2%的患者就诊原因为发现血肌酐升高。肾组织学检查同时存在肾小球肾炎和AIN的病理改变，肾间质与肾小球病变程度不平行，前者普遍重于肾小球损害，肾间质中嗜酸性粒细胞对诊断有提示价值。随访中，64.7%的患者肾功能最终恢复正常或基线水平，中位恢复时间为150 d（单纯AIN组为60 d），两组患者在2年内肾功能恢复情况方面无明显差异。 结论 肾小球肾炎合并AIN的患者临床表现不典型，AIN的症状易被肾小球肾炎掩盖。肾活检对诊断和鉴别有重要价值。早期诊治的患者预后较好。     

IgA肾病伴部分性新月体形成的临床病理分析

目的：探讨原发性IgA肾病伴部分性新月体形成的临床和病理特点。方法：选取79例经肾活检确诊伴部分性新月体形成IgA肾病患者,分析其临床和病理特点,并根据新月体形成所累及肾小球的比例分组：≥10%为A组,31例;≤10%为B组,48例。结果：（1）临床表现：79例均有血尿＋蛋白尿,蛋白尿〉1g/24h者48例（60.8%）;两组比较,A组蛋白尿〉1g/24h28例（89.3%）,B组蛋白尿〉1g/24h20例（41.7%）,A组大量蛋白尿、肉眼血尿、高血压、肾衰竭发生率均高于B组（P〈0.05）。（2）病理表现：79例新月体形成累及肾小球3.3%～29.0%,均以细胞性为主,几乎均有肾小球硬化、内皮细胞及系膜细胞增生、球囊黏连、灶性肾小管萎缩、以及炎性细胞浸润;两组比较A组中、重度系膜细胞及内皮细胞增生、炎性细胞浸润,细胞新月体所占比例均较B组明显;病理改变硬化肾小球占55例（69.6%）。结论：IgA肾病伴部分性新月体形成患者临床均有血尿＋蛋白尿,尤其大量蛋白尿;病理改变以局灶节段性肾小球硬化常见;炎性细胞浸润、内皮细胞及系膜细胞增生等活动性病变易见并影响新月体形成;新月体的多少及纤维化程度影响临床病理表现,≥10%较≤10%严重。     

Azurocidin-specific-ANCA-related idiopathic necrotizing crescentic glomerulonephritis

An 80-year-old woman who had rapidly progressive glomerulonephritis unaccompanied by systemic vasculitis is described. On renal biopsy, she showed necrotizing crescentic glomerulonephritis by light microscopy and pauci-immune glomerular lesions by immunofluorescent study. No dense deposits were present on electronmicroscopic study. On serum examination, indirect immunofluorescent study showed perinuclear pattern antineutrophil cytoplasmic antibody (ANCA), but myeloperoxidase-ANCA and proteinase 3-ANCA were both negative. Her serum reacted only to azurocidin excluding other ANCA antigens: bactericidal permeability-increasing protein, cathepsin G, elastase, lactoferrin, or lysozyme. Serum creatinine level decreased, and C-reactive protein turned negative after steroid therapy. Azurocidin-ANCA also turned negative. It is suggested that azurocidin-ANCA might have been related to the inflammatory process of pauci-immune necrotizing crescentic glomerulonephritis in this patient.     

新月体肾炎患者肾组织中浸润细胞的分布特点及临床意义

目的研究肾组织各种浸润细胞在新月体肾炎的发病机制中的作用。方法分析５６例各类月体肾炎患者肾组织中ＣＤ６８＋及ＰＣＮＡ＋细胞在各类新月体肾炎患者肾组织中的分布特点,并就它们与肾组织中ＣＤ＾＋及ＣＤ８＋细胞浸润的关系进行分析探讨。结果本组新月体肾炎患者肾组织中ＣＤ６８＋、ＰＣＮＡ＾＋、ＣＤ４＋及ＣＤ８＋细胞均明显高于正常供肾组织。在Ⅰ型新月体肾炎患者肾组织中ＣＤ６８＾＋及ＰＣＮＡ＋细胞数高于Ⅱ型及Ⅲ型     

口服舒洛地特联合缬沙坦治疗原发性慢性肾小球肾炎的临床研究

目的 观察口服舒洛地特联合血管紧张素受体拮抗剂缬沙坦治疗原发性慢性肾小球肾炎的临床效果.方法 选取本院97例原发性慢性肾小球肾炎患者经常规治疗后分成两组,Ⅰ组（联合治疗组）：给予舒洛地特250LSU 2次/天口服＋缬沙坦80mg 1次/天口服;Ⅱ组（缬沙坦组）：给予缬沙坦80mg 1次/天口服.治疗4个月后检测治疗前后24h尿蛋白定量、白蛋白、血浆纤维蛋白原、凝血酶原时间、活化部分凝血酶时间、甘油三酯、总胆固醇、血压、血肌酐、尿素氮、胱抑素C等指标.结果 白蛋白、凝血酶原时间（PT）、活化部分凝血酶时间（APTT）、甘油三酯（TG）、总胆固醇（TC）、血压（SBP）、血肌酐（Cr）、尿素氮（Ur）治疗前后比较差异无统计学意义（P＞0.05）.治疗后24h尿蛋白定量、血浆纤维蛋白原、胱抑素C较治疗前显著下降（P＜0.05）.治疗后联合治疗组较缬沙坦组24h尿蛋白定量、血浆纤维蛋白原、胱抑素C下降更明显（P＜0.01）.结论 采用舒洛地特联合缬沙坦较单独使用缬沙坦对慢性肾小球肾炎的治疗效果更为显著,为原发性慢性肾小球肾炎的治疗提供了新的方向.     

Rapidly progressive glomerulonephritis (crescentic glomerulonephritis) in the course of type I idiopathic membranoproliferative glomerulonephritis

Membranoproliferative glomerulonephritis is a chronic glomerulopathy with a generally progressive course toward end-stage renal disease and a high recurrence rate in renal allografts. Described herein is the appearance of rapidly progressive glomerulonephritis (crescentic glomerulonephritis) in the course of type I membranoproliferative glomerulonephritis. This transition occurred within 6 weeks, documented histologically by an initial and subsequent renal biopsy. No recurrence of the disease was noted in a living donor graft 18 months posttransplantation.     

210例原发性IgA肾病临床病理分析

目的 探讨原发性IgA肾病患者的临床表现、病理特点及其相关性。方法 回顾性总结分析本院1999年1月至2010年6月经肾活检确诊为原发性IgA肾病的210例患者的临床表现及病理特点。结果 506例肾活检患者中IgA肾病210例,检出率为41.5％。患者平均年龄为28.9±10.3岁,以20 ～ 39岁为高发,占59.5...     

原发性IgA肾病与非IgA系膜增生性肾小球肾炎的临床病理分析

目的 比较原发性IgA肾病与非IgA系膜增生性肾小球肾炎（non-IgA mesangial proliferative glomerulonephritis,non-IgA MsPGN）的临床及肾脏病理改变特点.方法 选择我科经肾活检确诊的原发性IgA肾病患者（A组）和non-IgA MsPGN患者（B组）进行临床与病理资料对比分析.结果 A、B组的性别、前驱上呼吸道感染诱因、起病时伴发高血压、镜下血尿、血肌酐无统计学差异（P＞0.05）.B组较A组起病年龄小,起病时伴发肉眼血尿比率低,肾病综合征发生率高,血IgG水平低,差异均有统计学意义（P＜0.05）.A组肾小球、肾小管间质、肾小动脉病理改变发生率高于B组（P＜0.05）,IgM、C3沉积、系膜区电子致密物沉积、大块状致密物、足细胞微绒毛化、肾小球基底膜分层发生率均较B组高（P＜0.01）.结论 IgA肾病与non-IgA MsPGN在临床表现、病理改变上存在明显差异,IgA肾病较non-IgA MsPGN病理损伤重.     

Clinicopathological features and prognosis of IgA nephropathy with crescentic formation in children

Objective To observe the clinical, pathological features and prognosis of IgA nephropathy in children with crescent formation. Methods A total of 38 cases of children with IgA nephropathy diagnosed by renal biopsy at Qilu Hospital of Shandong University were retrospectively analyzed. According to whether there were crescent formation, they were divided into crescents formation group (crescent formation≥10%, n=18) and control group consisting of children with complete clinical data and matching age, gender and course of disease (no crescent formation, n=20). The clinical, pathological features and prognosis of the two groups were compared. Results Fourteen cases (77.78%) of crescents formation group were associated with nausea hematuria, and 12 cases (66.67%) had hypertension, 8 patients in control group (40%) had gross hematuria, and 6 cases (30%) had high blood pressure. The differences were statistically significant (P＜0.05). In crescents formation group, Alb and eGFR were significantly lower than those of control group. 24 h urine protein and Scr were significantly higher than those of control group and there were significant statistically differences between two groups (P＜0.05). Nephropathological changes in crescent formation group were mainly in grade Ⅱ and grade Ⅲ and were usually combined with tubular interstitial damages. In addition to IgA immunoglobulin deposition, they often accompanied by IgG and IgM immunoglobulin deposition. In control group, nephropathological changes were mainly in grade Ⅰ and grade Ⅱand tubular interstitial damages were rare. IgA was the main form of deposition in control group. After 12 weeks steroid treatment and (or) immunosuppressive therapy, urinary protein turned negative in 7 cases in crescent formation group, 24 h urinary protein level declined by half than before in 7 cases and no obvious change in 4 cases. In control group, urinary protein turned negative in 12 cases, 6 cases showed a more than 50% decline in urinary protein level and 2 cases had no obvious change. During the 1-3 years of follow-up, renal function of 14 cases and 19 cases was normal in the crescent formation group and control group respectively, serum creatinine of 3 cases and 1 case increased mildly in the crescent formation group and control group respectively, 1 case needed dialysis treatment in the crescent formation group and the one in the control group who had slightly increasement of Scr did not reach the standard of dialysis treatment. Conclusions The clinical and pathological manifestations of children with IgA nephropathy with crescent formation are more severe and more likely to manifest renal insufficiency, suggesting that crescent formation is one of the indicators of poor prognosis.     

Treatment of crescentic glomerulonephritis

Summary: Acute crescentic-rapidly progressive glomerulonephritis is an uncommon but devastating disease which comes in several forms. That associated with anti-glomerular basement membrane (GBM) antibodies is most vexing to treat and has by far the worst prognosis. Despite conventional wisdom that plasma exchange and immunosuppression are optimal therapy, the cost constraints and issues raised in the present review suggest that this needs to be readdressed. Non-anti-GBM disease appears to respond well to aggressive immunosuppression and/or plasma exchange. A possible zided benefit of plasma exchange in patients with vasculitis on dialysis needs to be further addressed, especially in terms of a possible differential effect of very large doses of pulse methylprednisolone (30 mg/kg) as opposed to smaller doses.     

A case of anti-myeloperoxidase antibodies-associated idiopathic crescentic glomerulonephritis with pulmonary hemorrhage]

We report a case of idiopathic crescentic glomerulonephritis with pulmonary hemorrhage associated with anti-myeloperoxidase antibodies (anti-MPO ab). A 74 year-old female was admitted to our hospital because of rapidly progressive glomerulonephritic syndrome and dyspnea with bloody sputum. On admission anti-MPO ab, one of anti-neutrophil cytoplasmic antibodies, were detected but anti-GBM antibodies and immune complexes were not detected. Renal biopsy showed crescentic glomerulonephritis and lung biopsy showed massive alveolar hemorrhage. Both tissue had pauci-immune deposit by immunofluorescence microscopy. Hemodialysis and steroid administration were started. Pulmonary hemorrhage was improved remarkably, but renal failure progressed rapidly to end stage kidney, then hemodialysis was continued. Although subsequent 3 years uneventful maintenance hemodialysis had been performed, she admitted to our hospital again because of progressive dyspnea with hemoptysis after upper respiratory tract infection. On admission anti-MPO ab were detected again and steroid administration was started. Pulmonary hemorrhage was improved with decreased anti-MPO ab titer. While tapering the dosis of steroid, anti-MPO ab again increased and pulmonary hemorrhage recurred. Although pulse methylprednisolone therapy and plasma exchange were performed, respiratory failure progressed rapidly and she died of sepsis. Postmortem examination showed no evidence of systemic vasculitis. In this case, titer of anti-MPO ab was associated with not only idiopathic crescentic glomerulonephritis but also with pulmonary hemorrhage. We tried to detect enzymatically active MPO in serum. Titer of serum MPO was also associated with disease activity and anti-MPO ab. It is suggested that both anti-MPO ab and serum MPO are closely related to the pathogenesis of idiopathic crescentic glomerulonephritis and pulmonary hemorrhage.