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。     

环磷酰胺联合激素治疗血肌酐升高的IgA肾病的安全性和有效性分析

目的:探讨环磷酰胺联合激素疗法治疗血肌酐升高的IgA肾病的安全性及有效性。方法:纳入我院2002年01月~2015年11月血肌酐升高的IgA肾病的患者88例,随机平均分为2组,每组44例,其中激素+环磷酰胺+贝那普利组,连续1年;贝那普利组,连续1年,比较治疗前后2组的有效率,并比较2组患者的不良发应发病率及不良反应。结果:重复实验方差显示,实验组24 h尿蛋白效果、血清白蛋白及血肌酐各指标均优于对照组,差异具有统计学意义(P0.05);对照组与实验组治疗有效率分别为70.45%和86.36%,实验组显著高于对照组(Z=2.833,P0.05);实验组发生感染及肝功能损害患者的数量显著高于对照组(P0.05);实验组的恶心、厌食的不良反应发生率显著高于对照组(P0.05)。结论:激素+环磷酰胺+贝那普利较单用贝那普利对于IgA肾病的临床缓解率更高,尿蛋白及血肌酐下降程度更明显,而继发感染例数稍多,但无严重感染,肝功能损害多。     

中医及中西医结合治疗IgA肾病疗效分析

目的：探讨与中医及中西医结合治疗IgA肾病疗效相关的肾组织病理因素。方法：对58例经肾活检确诊的IgA肾病患者按治疗方法分为单纯中药治疗组及中西医结合治疗组,对两组患者的肾组织病理资料进行回顾性分析。结果：中药组中细胞性新月体组、肾小管萎缩组、肾小血管病变组治疗后24h尿蛋白定量较治疗前明显减少,差异有统计学意义（P均〈0.05）。中西医组：肾小球无硬化组、肾小球硬化组、细胞性新月体组、间质纤维化组、肾间质炎症组、肾小管萎缩组、肾小血管病变组、无肾小血管病变组治疗后24h尿蛋白定量均较治疗前明显减少（P〈0.05）,有统计学差异。不同病理表现对中药组、中西医组治疗前后血清肌酐水平均无明显改善（P〉0.05）。结论：中西医结合治疗对于肾脏病理表现为活动性病变及慢性病变的IgA肾病患者均具有疗效;而单纯中药辨证治疗对于肾脏病理表现以慢性病变为主者（肾小管萎缩、肾小血管病变）具有疗效。对于西药无法干预治疗的重症型IgAN患者,单纯中药辨证治疗仍能改善部分患者24h尿蛋白排泄,临床治疗总有效率仍有60.7%,结合中药辨证治疗IgAN可与西药治疗优势互补,从而延缓疾病的进展。     

不同类型血脂异常对IgA肾病临床及病理特征的影响

目的探讨不同类型血脂异常对IgA肾病临床及病理特征的影响。 方法共纳入283例原发性IgA肾病患者,收集临床资料及病理资料进行回顾性分析。将患者分为：高胆固醇(H－TC)组48例( TC≥ 6.22 mmol/L,TG<1.7 mmol/L);高甘油三脂(H－TG)组48例(TG≥2.26 mmol/L,TC<5.18 mmol/L);低高密度脂蛋白(L－HDL)组34例(HDL－C<1.04 mmol/L,TC<5.18 mmol/L,TG<1.7 mmol/L],同时收集IgAN血脂正常者(153例,TG<1.7 mmol/L、TC<5.18 mmol/L、HDL－C>1.04 mmol/L)作为对照组。对各组患者的一般情况、临床指标及病理分型(牛津分型)进行比较,并采用逐步回归分析方法进行多重线性回归分析探讨影响患者肾小球滤过率(eGFR)的因素。 结果H-TC组24 h尿蛋白量高于血脂正常组(Z＝－2.979, P＝0.003),血清白蛋白低于血脂正常组(t＝2.606,P<0.001)。H-TG组,身体质量指数(BMI)、血清尿酸高于血脂正常组(t＝3.982,t＝5.056;P<0.001), eGFR低于血脂正常组(t＝2.011,P＝0.045)。L-HDL组BMI高于血脂正常组(t＝2.946,P＝0.004),eGFR低于血脂正常组(t＝2.498,P＝0.013),肾小管萎缩/间质纤维化程度高于血脂正常组(χ²＝8.284,P<0.017)。回归方程结果显示年龄、收缩压、尿酸与eGFR呈负相关关系(P<0.05),血清TC与eGFR亦呈负相关关系(95%CI：－5.228～－0.312,t＝－2.220,P＝0.027)。血清HDL与eGFR呈正相关关系( 95%CI：1.469～7.468,t＝2.935,P＝0.004),该模型经检验有统计学意义(F＝11.838,P<0.001)。 结论H-TC、H-TG、L-HDL血症均可能加重IgA肾病的临床损害,且L-HDL血症者肾小管间质受损更严重。降低血清TC,提高血清HDL可能有助于IgA肾病患者改善预后。     

伴高尿酸血症IgA肾病的临床、病理特点及预后的相关分析

目的：研究伴高尿酸血症IgA肾病患者的临床、病理特点及预后的相关因素。方法：对2006年～2009年经肾活检确诊为IgA肾病的72例患者的临床与病理资料进行分析。根据血尿酸水平,将72例IgA患者分为高尿酸血症组与血尿酸正常组,比较两组间临床指标、肾功能指标及病理学参数。结果：临床指标中年龄、血尿酸及尿蛋白定量均与血肌酐、肾小球滤过率有显著相关性（P〈0.05）。多元逐步回归分析血尿酸对IgA肾病肾功能进展的预测优于尿蛋白定量。病理参数中肾小球积分、肾小管间质积分与血肌酐、肾小球滤过率显著相关（P〈0.05）。高尿酸血症组肾功能指标均差于血尿酸正常组。高尿酸血症组肾小管间质损害均高于血尿酸正常组。结论：IgA肾病的预后主要与尿蛋白定量、血尿酸、肾小管间质损害、球性硬化有关,IgA肾病37.5%患者合并高尿酸血症、其临床尿蛋白、肾功能损害及病理肾小管间质损害均高于血尿酸正常组IgA肾病患者,应重视血尿酸在IgA肾病中的作用。     

伴和不伴新月体形成的IgA肾病的临床与病理研究

目的：探讨伴和不伴新月体形成的原发性IgA肾病（IgAN）临床和病理特点。方法：分析128例经肾活检确诊IgAN患者,分析其临床特点,并根据新月体形成所累及肾小球的比例分组：无新月体形成为A组,69例;≤10%为B组,25例;〉10%〈50%为C组,34例。根据Katafushi积分分析肾脏病理。结果：（1）临床方面：B组和C组的血尿素氮（BUN）较A组高（P〈0.05）;C组血肌酐（Scr）较A组高（P〈0.01）;B组和C组的收缩压（SBP）较A组高（P〈0.01）;C组舒张压（DBP）较A组高（P〈0.01）。（2）病理方面：C组肾小球总积分比A组、B组高（P〈0.01）,B组肾小球总积分比A组高（P〈0.01）;B组球性硬化积分比A组高（P〈0.01）,C组球性硬化积分比A组高（P〈0.01）;C组节段损害、肾小管间质、炎症细胞浸润积分比A组高（P〈0.01）。结论：原发性IgAN随着新月体形成所累及肾小球的比例增加,血压有逐渐升高趋势;肾脏病理损害逐渐加重,故对有新月体形成原发性IgAN应早期干预治疗,以延缓肾病进展。     

不同CKD分期、大量蛋白尿型IgA肾病患者的临床、病理和预后分析

目的:分析不同慢性肾脏病(chronic kidney disease,CKD)分期的大量蛋白尿型IgA肾病患者的临床表现和病理改变,并随访观察患者的预后。方法:回顾性分析2012年01月~2016年12月CKD 1~5期大量蛋白尿型IgA肾病患者80例。比较CKD 3~5期大量蛋白尿型IgA肾病组(A组)及CKD 1~2期大量蛋白尿型IgA肾病组(B组)患者的临床和病理特点、治疗疗效及随访中肾存活率。结果:A组患者肾活检时肾功能较差,其血尿素氮(BUN)、血肌酐(Scr)、血尿酸(Sua)、高血压发病率均显著高于B组,血红蛋白、肾小球滤过率水平显著低于B组;肾脏病理上,A组患者活动性病变更明显,肾小管间质疾病及由于血管病变所致的肾损伤均重于B组,A组病理Lee氏分级均≥Ⅲ级,B组病理Lee氏分级均≤Ⅳ级。经糖皮质激素和/或免疫抑制剂治疗后,A组患者血白蛋白水平升高[(42.57±5.86)vs.(33.71±8.08)g/L,P0.01],肾小球滤过率升高[(54.69±16.35)vs.40.13±17.67)m L·min-1·1.73 m-2,P0.01],尿蛋白较治疗前显著下降[(2.77±1.98)vs.(6.59±4.17)g/24 h,P0.01],但有效率低于B组(55.56%vs.88.09%,,P0.01),治疗后A组患者的血肌酐、血尿素氮、尿蛋白水平仍高于B组[(2.77±1.98)vs.(1.19±1.07)g/24 h,P0.01],e GFR水平及肾存活率低于B组(84.23%vs.100.00%,P0.05)。结论:大量蛋白尿型IgA肾病患者中CKD 3~5期的患者与CKD 1~2期的患者相比,病理上活动性病变及慢性化病变均更明显,肾存活率更低;激素及免疫抑制剂治疗可使部分患者的尿蛋白减少,肾功能改善。CKD3~5期大量蛋白尿型IgA肾病患者仍需行肾活检以便明确诊断、及时治疗。     

骨形成蛋白7和转化生长因子β1在不同病理类型IgA肾病的变化

目的 观察转化生长因子β1（TGF-β1）和骨形成蛋白7（BMP-7）在不同病理类型IgA肾病的变化,并探讨其意义。方法 89例IgA肾病患者分成3组：A组为47例轻度系膜增生性IgA肾病;B组为29例中重度系膜增生性IgA肾病;C组为13例增生硬化或硬化性IgA肾病。检测患者的血压、尿蛋白量（24 h）、Scr和Ccr。免疫组化和ELISA方法测定患者肾组织冰冻切片及其血、尿中TGF-β1和BMP-7水平。计算患者病理切片硬化肾小球数、新月体数和间质纤维化面积百分比。结果 随着IgA肾病患者肾小球病变的加重,肾小管萎缩和肾间质纤维化增多,其血压、尿蛋白量（24 h）、Scr逐渐增加,除B、C两组间尿蛋白量（24 h）无显著差异外,其余各组间差异均有统计学意义（P＜0．05）。与A组比较,B组肾组织及血、尿TGF-β1明显增多,C组显著降低（P＜0．01）。肾组织冰冻切片及血、尿BMP-7随着肾脏病变的加重,水平逐渐下降（P＜0．01）;而且与Ccr呈正相关;与血压、Scr、尿蛋白量（24 h）、硬化肾小球数、新月体数、肾间质纤维化面积呈负相关。结论 TGF-β1在IgA肾病系膜增生严重时明显增加,肾脏广泛纤维化时明显降低,可能参与了IgA肾病肾间质纤维化的发生。BMP-7随肾脏病变的加重而明显降低,可能导致其抗肾纤维化作用减弱。     

糖皮质激素和环磷酰胺治疗IgA肾病疗效及相关因素分析

目的 分析糖皮质激素和环磷酰胺治疗IgA肾病患者的疗效,寻找与疗效相关的临床和病理指标.方法 217例IgA肾病患者(尿蛋白定量≥1.0/d,且Scr＜250μmol/L),应用糖皮质激素和环磷酰胺治疗1年后,对比分析治疗有效组和无效组临床及病理资料.将尿蛋白定量＜0.5g/d,或eGFR上升＞15％视为治疗有效.结果 糖皮质激素和环磷酰胺治疗IgA肾病有效者占82.49％,疗效与年龄、肾功能水平、尿蛋白、尿渗透压、肾小管萎缩和肾间质纤维化以及肾小球缺血硬化程度等有关.结论 糖皮质激素和环磷酰胺是治疗IgA肾病有效方法,但选择该方案时,应考虑患者的临床和病理特点.     

伴部分新月体形成IgA肾病临床病理特征分析

目的:探讨伴部分新月体形成的Ig A肾病患者临床、病理特征及预后情况。方法:采用回顾性分析方法,比较115例伴部分新月体形成的Ig A肾病患者与91例不伴新月体形成的患者临床表现、肾脏病理等方面异同。结果:(1)一般情况:伴部分新月体形成的Ig A肾病患者病程短于不伴新月体形成组(P0.05),且伴部分新月体形成组扁桃体肿大出现的比例高(P0.05)。(2)理化指标:伴部分新月体形成组血肌酐水平、Ig A/C3比值显著高于不伴新月体形成组(P0.05),e GFR、血红蛋白水平低于不伴新月体形成组(P0.05);两组间24小时尿蛋白定量、Alb、UA、TC、TG、LDL、血清Ig A、补体C3及收缩压比较差异无统计学意义(P0.05)。(3)肾脏病理:两组病理类型均以局灶增生型为最多,伴部分新月体形成组病理类型其次为局灶增生硬化型,而不伴新月体形成组其次为轻度系膜增生型(P0.05);伴部分新月体形成组炎细胞浸润积分、肾间质纤维化积分、肾小管萎缩积分明显高于不伴有新月体组(P0.05)。结论:伴部分新月体形成的Ig A肾病患者病情进展较快,基础肾功能(血肌酐、e GFR)偏差,肾小管间质病变重,提示预后不佳。     

Crescentic, proliferative IgA nephropathy: clinical and histological response to methylprednisolone and intravenous cyclophosphamide.

BACKGROUND: IgA nephropathy is an immune-complex glomerulopathy that can result in capillary or extra-capillary proliferation. Previous attempts to correlate specific histological findings including cellular crescents or endocapillary proliferation, with clinical outcomes, have produced conflicting results. METHODS: We conducted a prospective open-labelled trial of 12 patients with crescentic, proliferative IgA nephropathy and clinically progressive disease and treated them with pulse steroids and intravenous cyclophosphamide. Therapy included pulse solumedrol at 15 mg/kg/day for 3 days, followed by monthly intravenous cyclophosphamide at 0.5 g/m(2) body surface area for 6 months. Clinically significant proteinuria (>1.0 g/24 h) was present in all patients, while nephrotic-range proteinuria (>3.0 g/24 h) was observed in eight of 12 patients. All patients were hypertensive (BP >140/90 mmHg). RESULTS: After 6 months of treatment, the mean serum creatinine was reduced from a maximum of 2.65+/-0.39 to 1.51+/-0.10 mg/dl (P<0.03), while proteinuria was reduced from 4.04 to 1.35 g/24 h (P<0.01). The mean slope of 1/serum creatinine increased from -0.0398+/-0.02 to 0.0076+/-0.01 after 6 months of therapy, but this trend did not reach statistical significance (P<0.08). A repeat kidney biopsy was performed in all treated patients. Endocapillary proliferation, cellular crescents and karyorrhexis were eliminated in all 12 patients after 6 months of therapy, while interstitial fibrosis and tubule dropout remained unchanged. To determine the long-term efficacy of the treatment, treated patients were compared to 12 historical controls matched for severity of IgA on initial biopsy. After 36 months, the rate of end-stage renal disease in the treated group was lower (1/12) than in the historical controls (5/12). CONCLUSIONS: We conclude that steroids and intravenous cyclophosphamide reduce proliferative lesions, reduce proteinuria and stabilize renal function in patients with crescentic IgA nephropathy.     

Steroid and cyclophosphamide therapy for IgA nephropathy associated with crescenteric change: an effective treatment

BACKGROUND: IgA nephropathy is the most common form of idiopathic glomerulonephritis. There is no current consensus on treatment for this condition. We report on the effect of immunosuppression with corticosteroids and cyclophosphamide for the treatment of IgA nephropathy associated with crescenteric change. METHODS: The effect of oral prednisolone (0.8 mg/kg initially, reducing to 0.4 mg/kg after 4 weeks) and cyclophosphamide (1.5 mg/kg) given until a plateau of response was obtained was studied in 9 patients with IgA nephropathy associated with severe inflammatory change and crescents. The initial diagnostic renal biopsies of these patients revealed 25-70% of the glomeruli effected with active cellular crescents. When response to therapy, plateaued cyclophosphamide was discontinued and prednisolone reduced from 0.4 mg/kg. Follow-up renal biopsy was performed in 8 of the 9 patients. Patients were maintained on prednisolone (5- 7.5 mg) and azathioprine (1 mg/kg) for further 2 years. RESULTS: The mean time until discontinuation of cyclophosphamide was 17.8 weeks (+/-1.23, range 12-25 weeks). There were no serious complications of therapy. There was an improvement in renal function in all patients with serum creatinine falling from a mean of 149.6+/-16.5, range 81-227 micromol/l to 116.4+/-8.6, range 80-158 micromol/l, p=0.01. Creatinine clearance improved from a mean of 57.1+/-9.9, range 21-104 ml/min to 87.2+/-10.1, range 39-125 ml/min, p=0.004. 24-hour urinary total protein fell over the same time m period from a mean of 4.54+/-1.1, range 1.0-11.27 g to 1.2+/-0.27, range 0.01-2.65 g, p=0.004. There were no significant differences in blood pressure during this time. Repeat renal biopsies showed significant degrees of histological improvement with healing of crescents and a reduction in acute inflammatory change in all except one patient. The mean period of follow-up after cessation of cyclophosphamide was 17.4+/-2.8 months, range 10-36 months. There was no significant change over this period in serum creatinine, creatinine clearance or urinary protein losses. CONCLUSION: These data suggest that IgA nephropathy associated with severe inflammatory and crescenteric change can be effectively and safely treated with a low-cost regime based on oral corticosteroids and cyclophosphamide tailored to a plateau of treatment response in individual patients.     

Discriminant analysis of clinical markers before renal biopsy in patients with IgA nephropathy

Discriminant analysis of clinical markers before renal biopsy in patients with IgA nephropathy is described. Sixty eight patients with IgA nephropathy (IgA nephropathy group) and 66 patients with other chronic glomerulonephritis (non-IgA nephropathy group) were examined. The discriminant analysis was applied to separate those two groups by using twenty clinical parameters as well as binding capacity of serum IgA to the glomeruli of renal specimens. Binding of serum IgA of patients to the glomeruli obtained from patients with IgA nephropathy was performed using avidin-biotin immunofluorescence. Among twenty clinical markers, the levels of serum IgA and creatinine, and degree of microhematuria in IgA nephropathy group were significantly higher than those in non-IgA nephropathy group Furthermore, the positive incidence of serum IgA binding of IgA nephropathy group was significantly higher than that of serum IgA binding of non-IgA nephropathy group. The correct classification rate were 79.10% using five clinical markers including serum IgA, microhematuria, serum C4, quantitation of proteinuria and degree of proteinuria. It is indicated that the levels of serum IgA and the binding of serum IgA to the glomeruli were considered to be major markers for clinical diagnosis of patients with IgA nephropathy It was concluded that the discriminant analysis before renal biopsy was useful for diagnosis of IgA nephropathy.     

贫血加重IgA肾病患者的临床和病理改变

目的 分析IgA肾病合并贫血患者的临床病理特征.方法 收集经肾活检确诊的IgA肾病患者临床资料409例,按照贫血与否分为非贫血组和贫血组,回顾性分析两组患者的临床和病理资料.结果 与非贫血组比较,贫血组患者的肾小球损伤和肾小管间质萎缩程度较重、24 h尿蛋白增多和eGFR降低.Spearman相关分析结果显示,血红蛋白、eGFR与肾脏病理损伤呈负相关(P＜0.05),血尿酸、24h尿蛋白与肾脏病理损伤呈正相关(P＜0.05).多因素Logistic回归分析发现贫血是肾小管间质萎缩的独立危险因素.结论 IgA肾病合并贫血患者的临床和病理损伤重于IgA肾病非贫血的患者,贫血参与IgA肾病的进展.     

Clinical presentation, natural history, and treatment of crescentic proliferative IgA nephropathy

IgA nephropathy is one of the most common causes of glomerulonephritis in the world and is characterized histologically by the deposition of polymeric forms of IgA within the mesangium and in some cases along the glomerular capillary wall.(1) Proliferative and crescenteric forms of IgA are associated with nephrotic range proteinuria, accelerated hypertension, and a more rapid decline toward end-stage renal disease. Previous attempts to categorize the incidence and clinical significance of proliferative IgA nephropathy have given conflicting results. This is in part the result of the lack of a uniform nomenclature and the failure of clinical therapies to prolong renal survival in specific subgroups. In the present study, we performed a prospective open-label trial of pulse solumedrol and intravenous cyclophosphamide in 20 patients with IgA nephropathy and at least 10% cellular crescents or endocapillary proliferation on renal biopsy. Seventeen patients underwent repeat kidney biopsies after 6 months of therapy, and the morphologic response to treatment was assessed using a modified systemic lupus erythematosis (SLE) histologic activity and chronicity index score. To determine the long-term efficacy of intravenous cyclophosphamide on renal survival, the results of the treated patients were compared with 12 untreated historical controls. Pulse solumedrol and intravenous cyclophosphamide effectively reduced peak serum creatinine, degree of proteinuria, the rate of decline in renal function, and the incidence of end-stage renal disease at 36 months.     

Loss of renal graft due to recurrent IgA nephropathy with rapidly progressive course: an unusual clinical evolution

Recurrence of IgA nephropathy following renal transplantation has been described in 40-50% of patients, and it usually has a good outcome. We present the case of a 54-year-old man with IgA nephropathy who developed terminal renal failure in 1985, 3 years after the onset of the disease. In March 1986 he received a cadaveric renal allograft following treatment with ciclosporin and steroids. Eight months later he developed microhaematuria and proteinuria and 10 months later he developed acute nephritic syndrome and rapidly progressive renal failure. Renal biopsy disclosed an IgA nephropathy with epithelial crescents in 60% of glomeruli. Treatment with plasma exchange and cyclophosphamide was unsuccessful and the patient lost his graft and returned to regular haemodialysis 15 months after renal transplantation.     

Crescents proportion no lower than 14% were significantly associated with renal function and outcomes of IgA nephropathy patients

Objective To explore the clinicopathological features and outcomes of IgA nephropathy patients with different proportions of crescents. Methods A total of 270 patients who were diagnosed as IgA nephropathy by renal biopsies from January 2010 to December 2015 in the First Affiliated Hospital of Shenzhen University were enrolled. All patients were divided into 3 groups according to the optimal cutoff level of crescents proportion in the Receiver Operating Characteristic Curve (ROC) as follows: 0%, ＜14%; ≥14%. The endpoint was defined as the doubling of baseline serum creatinine (Scr) and/or end-stage renal disease (ESRD). Kaplan-Meier curve and Cox regression model were used to analyze the renal survival among three groups. Results One hundred and four patients (38.5%) without any crescents; 84 patients (31.1%) with crescents proportion＜14% and 82 patients (31.4%) with crescents proportion ≥14%. Patients with crescents proportion ≥14% group were older and had higher level of systolic blood pressure and diastolic blood pressure, 24-hour urine protein and serum uric acid level; more patients treated with RAS blocker, glucocorticoid and immunotherapy, but lower eGFR, hemoglobin and serum albumin level than those with crescents proportion＜14%. Compared with those without crescents and crescent proportion＜14%, patients with crescent proportion ≥ 14% also had higher proportion of global glomerulosclerosis, more endocapillary hypercellularity and severe tubulointerstitial lesions, higher degree of IgA and C3 depositions in renal. 24-hour proteinuria, serum uric acid level, low hemoglobin level, endocapillary hypercellularity and renal C3 depositions were risk factors for crescents formation. Patients were followed-up for a median of (31.7±21.0) months, and Kaplan-Meier analysis revealed that renal survival rate was significantly lower in patients with crescents proportion ≥14% compared with other groups (P=0.001). But there was no significant difference between no crescent group and crescents proportion＜14% group. However, multivariate Cox analysis showed no significant difference between crescents proportion and renal survival. Conclusion Crescents proportion is associated with higher risk of renal function and renal progression.     

Steroid and cyclophosphamide in IgA nephropathy.

BACKGROUND: IgA nephropathy is associated with a wide spectrum of possible lesions. Therefore, different responses to anti-inflammatory or immunosuppressive therapies should be expected with acute inflammatory changes, which are predominantly reversible, and with prevalently sclerotic lesions. METHODS: The effects of a combined schedule of prednisone and cyclophosphamide was analysed in the specific subset of IgA nephropathy patients with acute inflammatory histologic changes associated with haematuria and proteinuria. Two groups of patients, with similar histologic lesions and clinical presentation, were considered. The first group (12 patients) was treated within 1 week after renal biopsy; starting with three pulses of methylprednisolone (1 g) followed by oral prednisone (0.8 mg/kg body weight for 2 weeks, 0.6 mg/kg for another 2 weeks, 0.4 mg/kg for an additional 4 weeks, then slowly tapered by 5 mg each month until discontinuation) and 1.5 mg/kg cyclophosphamide for 2 months. A second sample of eight untreated patients served as a control group. Treated and untreated patients had diffuse mesangial proliferation with florid crescents (8-60% in treated and 10-40% in untreated patients) with mild degree of glomerular sclerosis and interstitial changes. Basal creatinine (167 micromol/l, range 79-371 vs 132 micromol/l, range 79-256) and proteinuria (3.0 g/24 h, 1.0-4.9 vs 3.3 g/24 h, 1.0-13.7) were not statistically different between treated and untreated patients respectively. Nine treated and six untreated patients were hypertensive. Blood pressure treatment did not include ACE-inhibitors. RESULTS: Untreated patients' 5-year renal survival, as assessed by the Kaplan-Meier method, was found to be significantly lower than treated patients (37.5 vs 91.6%, log-rank P=0.01 and Breslow test P=0.008; relative risk to reach the endpoint of a 100% increase in serum creatinine=3.58, P=0.03). CONCLUSION: This short course of therapy with prednisone and cyclophosphamide has been effective in a subset of IgA nephropathy patients with florid glomerular changes and major urinary abnormalities, turning off phlogistic activity and preventing subsequent progression toward renal failure.     

伴有慢性肾衰竭的马兜铃酸肾病与IgA肾病的配对研究

目的:了解伴有慢性肾衰竭的马兜铃酸肾病患者与IgA肾病患者的临床病理差异.方法:分析11例马兜铃酸肾病患者的临床病理资料,并与经肾活检确诊的IgA肾病患者进行配对比较.结果:两组患者的年龄、性别、血肌酐水平无明显差异(P>0.05),马兜铃酸肾病患者镜下血尿发生率低于IgA肾病患者(P<0.05).在血肌酐水平无明显差异时,马兜铃酸肾病患者血红蛋白浓度明显低于IgA肾病患者(P<0.01),尿蛋白定量和肾脏的长径少于IgA肾病患者(P<0.05).间质纤维化程度重于IgA肾病患者(P<0.05),而间质炎细胞浸润少(P<0.05).结论:伴有慢性肾衰竭的马兜铃酸肾病患者临床发展隐匿,与IgA肾病比较,肾小管间质损伤是造成肾功能损害的主要原因,因此要重视对马兜铃酸肾病的早期防治.