老年慢性间质性肾炎78例临床及病理分析

慢性间质性肾炎(CIN)早期临床表现较隐匿,以肾小管功能障碍为主,常被漏诊.为探讨老年慢性间质性肾炎的临床及病理特点,我们对78例CIN患者的临床及病理资料进行回顾性分析如下.     

狼疮性肾炎48例临床表现与病理改变分析

目的 总结狼疮性肾炎的临床表现及病理改变.方法 对48例狼疮性肾炎患者临床资料作以回顾总结.结果 48例患者临床表现以多系统损害最为常见,多数患者同时出现多种临床症状.48例患者经肾组织活检发现其中狼疮肾炎病理分型为Ⅰ型者1例,占2.1％；为Ⅱ型者6例,占12.5％；为Ⅲ型者14例,占29.2％；为Ⅳ型者20例.占41.7％;为V型者7例,占14.6％.结论 狼疮性肾炎患者的临床症状一定程度上可以预测患者的病理分型,在狼疮肾炎患者条件及病情允许的情况下,应对患者进行肾活检病理检查,并根据其病理结果进行确诊与相应治疗.     

狼疮性肾炎并急性肾衰的临床及病理分析

目的：探讨狼疮性肾炎并急性肾衰的临床、病理特点及治疗措施。方法：收集符合狼疮性肾炎合并急性肾衰者共22例进行分析研究。结果：病人均属狼疮活动状态,肾脏病理改变以IV型较多见,与预后相关因素为大量蛋白尿、血清补体显著下降,肌酐清除率（Ccr）降低,累及脏器多少等。大剂量甲基强的松及环磷酰胺联合冲击治疗,总有效率为63.6%,死亡率为27.3%。结论：狼疮性肾炎并急性肾衰是肾科的危重症,而早期强有力的治疗可改善本病的预后。     

老年毛细血管内增生性肾小球肾炎病理与预后分析

目的分析老年毛细血管内增生性肾小球肾炎患者临床、病理特点及其预后。方法回顾性分析5例经肾组织活检组织病理确诊的毛细血管内增生性肾小球肾炎老年患者临床、病理资料及治疗与转归。结果4例呈肾病综合征表现，1例呈慢性肾炎综合征表现；2例有前驱皮肤感染史；均符合毛细血管内增生性肾小球。肾炎病理改变；经抗感染、降压、糖皮质激素及血液透析等治疗，均好转出院；随访1a，患者仍存在高血压及镜下血尿。结论提高对老年毛细血管内增生性肾小球肾炎的认识，及时进行肾组织活检有助于早期诊断、合理治疗及改善患者预后。     

狼疮性肾炎并急性肾衰25例的临床及病理分析

系统性红斑狼疮(SLE)几乎全部有肾脏病变,而以急性肾功能衰竭(ARF)为主要表现者不多见,此型病情发展凶险,预后严重,早期诊断和合理治疗可大大改善预后.作者回顾分析了近年来收治的25例病人的临床及病理,旨在提高对狼疮性肾炎(LN)合并ARF的诊治认识.     

过敏性紫癜性肾炎早期诊断研究进展

过敏性紫癜肾脏受累的程度直接影响患者预后,早期及时诊断,可指导临床治疗.肾组织活检对诊断具有重要意义,但为有创检查.过敏性紫癜性肾炎的早期实验室诊断可为临床诊断提供依据.本文将过敏性紫癜性肾炎相关的反映肾小球、肾小管损伤的各种血、尿指标及辅助检查资料作一综述,为过敏性紫癜肾脏损伤的早期诊治提供依据.     

慢性肾小球肾炎并药物性间性肾炎（附8例临床分析）

药物性急性间质性肾炎是由药物引起的一组临床病理综合征 ,起病急骤 ,其主要病理改变为肾间质的炎细胞浸润 ,肾小管不同程度的退行性变 ,出现肾功能不全。目前临床用药非常广泛 ,故药物性急性间质性肾炎已引起临床重视。由于慢性肾脏疾病易并发药物间质性肾炎 [1 ] ,且容易漏诊与误诊 ,为提高二者并存时临床表现及治疗的认识 ,对我院 1 996～ 1 999年8例慢性肾小球肾炎并药物性间质性肾炎患者的临床资料总结 ,报告如下。1　临床资料1 .1　一般资料　病例均符合以下标准 :1先有肾小球疾病的临床表现 ,包括肉眼血尿 1例 ,蛋白尿并镜下血尿 3例…     

狼疮性肾炎病理分析

目的分析肾活检中狼疮性肾炎的病理与病变发展关系,为临床治疗提供可靠的实验依据。方法参照WHO(1982年及1995年)肾小球疾病组织分型方法及补充修订方案,按临床表现、实验室诊断及病理特征分型。结果本组肾活检继发性肾病321例,确诊为狼疮性肾炎81例,占继发性肾病的39.63%。结论实验结果显示,肾组织活检病理学是诊断狼疮性肾炎最可靠的方法,它能为不同类型狼疮性肾炎患者提供临床治疗及判断预后提供可靠的形态学依据。     

85例新月体肾炎的临床与病理分析

目的 观察新月体肾炎临床、病理特点、治疗及预后。方法 对我科1981～2005年3月经肾活检确诊的85例患者发病年龄、性别、临床表现及相关实验室检查，肾脏病理特点，治疗方法与预后进行回顾性分析，近10年来对患者血清学进行抗中性粒细胞抗体(ANCA)和抗肾小球基底膜(GBM)抗体的检测。结果 85例中，男性47例(55．3％)，女性38例(44．7％)；平均年龄为39．2岁(5～77岁)，5～15岁11例(12．9％)，16～40岁43例(50．5％)，41～77岁31例(36．4％)。原发性急进性肾炎26例(30．5％)；原发性小血管炎25例(29．4％)，狼疮性肾炎11例(12．9％)，紫癜性肾炎7例(8．2％)，IgA肾病7例(8．2％)，肺出血肾炎综合征7例(8．2％)，急性链球菌感染后肾炎和亚急性细菌性心内膜炎各1例(1．2％)。临床表现肾脏表现主要浮肿，蛋白尿，血尿，管型尿，少尿或无尿，不同程度的贫血与高血压，均有急性肾功能衰竭。肾外表现有发热，皮损，咯血，关节痛等系统疾病表现，病理肾脏大小正常或稍大，光镜下有大新月体形成，免疫复合物肾炎，中青年男性多见，早期强化治疗有效。抗GBM抗体型以青年男性多见，强化血浆置换治疗效果差，预后不良。原发性小血管炎，Ig沉积阴性或弱阳性以中老年男性多见，强化治疗有效。本院21例缓解，3例正在透析，3例作肾移植，其余3个月～2年内死亡。结论 在我国新月体性肾炎以免疫复合物沉积者多见，ANCA阳性小血管炎肾炎并非少见，本组占第二位。早期强化治疗可完全缓解或脱离透析。     

急进性肾小球肾炎的治疗进展

1引言 RPGN是一组以急性肾炎综合征(血尿、蛋白尿、水肿、高血压)、肾功能急剧减退、早期出现少尿或无尿为主要临床表现,以新月体肾小球肾炎(50%以上肾小球出现大新月体,早期为细胞新月体,晚期为纤维新月体)为病理特征的原发性肾小球疾病.病情危重,需要尽早治疗.     

Apheresis for MPO-ANCA-associated RPGN-indications and efficacy: lessons learned from Japan nationwide survey of RPGN

A national survey concerning rapidly progressive glomerulonephritis (RPGN) was conducted in Japan between 1989 and 2000 and resulted in the registration of 715 patients with RPGN. Among the documented patients, the most frequent primary disease was primary pauci-immune crescentic glomerulonephritis (n = 283), and the second most frequent was microscopic polyangitis (n = 127). Overall, 370 patients had MPO-ANCA, and 23 patients had PR3-ANCA. We found that both renal and patient survivals were significantly worse in patients with MPO-ANCA-associated RPGN than patients with PR3-ANCA. Fifty-three patients received apheresis therapy with various combinations of immunosuppressive regimens. They had higher serum creatinine, higher CRP, and a higher frequency of complicated pulmonary involvements as compared to the controls without apheresis therapy. In dialysis-dependent patients, no additional benefit from apheresis therapy was observed. Only pulmonary renal syndrome patients with CRP > 6 mg/dl at presentation showed a slightly better prognosis (patient survival with apheresis; 66.7%, without apheresis; 56.7%). Furthermore, a rapid MPO-ANCA titer reduction was observed in patients treated with apheresis. Patients with MPO-ANCA-associated RPGN were older, and had more chronic and sclerotic lesions than patients with PR3-ANCA-associated RPGN. Based on these findings, we suggest that a lower dose of immunosuppressant should be considered in order to avoid opportunistic infection. In this situation, cytapheresis is the treatment of choice. Nevertheless, in patients with an aggressive form of RPGN with rapid deterioration of renal function like the PR3-ANCA-associated RPGN, or pulmonary renal syndrome complicated severe inflammation, or relapses with high MPO-ANCA titer, we conclude that apheresis therapy should be considered.     

Plasmapheresis in the treatment of rapidly progressing glomerulonephritis

The authors are of the opinion that plasmapheresis (PP) combined with immunosuppressant therapy is an effective and a relatively safe method for the treatment of rapidly progressing glomerulonephritis (RPGN). Introduction of PP in multimodality treatment of RPGN made it possible to arrest rapidly progressing renal failure in all 6 treated patients. After PP treatment was over, renal function was recovered completely in 3 patients. One patient manifested the retention of renal failure of medium degree while rare hemodialysis sessions permitted one to control water-electrolyte disorders. In two patients the discontinuation of PP treatment resulted in the progress of renal failure. The data obtained do not make it possible to relate the improvement of renal function exclusively to the action of PP, since all the patients received immunosuppressants. Nevertheless, in 2 cases, the improvement could be attributed to PP, for its discontinuation in these patients (without any changes in the remaining treatment) brought about again the progress of renal failure.     

Cytapheresis for the treatment of myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis: report of five cases.

To minimize the adverse effects of high-dose administration of steroids and cyclophosphamide in patients with myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA), granulocytapheresis (GCAP) or leukocytapheresis (LCAP) was performed to reduce inflammation. Four patients with rapidly progressive glomerulonephritis (RPGN) and one patient with pulmonary hemorrhage due to MPO-ANCA-associated vasculitis were treated by cytapheresis. The prednisolone (PSL) dose was 0.28 +/- 0.15 mg/kg/day (mean +/- SD) (range 0.18-0.50 g/kg/day). In the 4 RPGN patients, the peak serum creatinine level was 3.7 +/- 1.9 mg/dl (range 1.7 to 5.6 mg/dl). GCAP was performed in 3 RPGN patients and in 1 pulmonary hemorrhage patient. LCAP was performed in 1 RPGN patient. In the 4 RPGN patients, renal function improved after combined therapy with cytapheresis and corticosteroids. In the pulmonary hemorrhage patient, evidence of pulmonary hemorrhage on chest computed tomography scanning diminished after combined therapy with cytapheresis and corticosteroids. Cytapheresis, when combined with a low-dose or intermediate-dose PSL regimen, is effective in the treatment of ANCA-associated vasculitis.     

The partial recovery of kidney function in chronic uremia patients during hemodialysis treatment

Out of 432 patients placed on the treatment with hemodialysis (HD) for terminal renal failure (TRF) at the All-Union Nephrologic Center from January 1, 1978 to December 31, 1987, 17 patients manifested partial recovery of renal function, which enabled dialysis treatment to be discontinued for a time. Among the 17 patients with noticeable improvement of renal function, 8 presented with lupoid rapid-progressing glomerulonephritis (RPGN), 2 with RPGN associated with hemorrhagic vasculitis, 1 with idiopathic RPGN, 4 with chronic glomerulonephritis (CGN), 1 with chronic pyelonephritis, and 1 with polycystic kidneys. In 11 patients with RPGN, the rate of renal failure progression, expressed by the regression coefficient, was much higher among those in whom HD treatment was discontinued that in the group of patients without renal function recovery. In the 4 patients with CGN, renal function was recovered after the correction of marked disorders of purine metabolism, whereas in the 1 patient with chronic pyelonephritis and in the 1 with polycystic kidneys after urinary infection elimination. According to the ultrasonography data, out of the 17 patients with partial recovery of renal function, the size of the kidneys turned out normal in 14 patients.     

Efficacy of plasma exchange in severe idiopathic rapidly progressive glomerulonephritis. A report of ten cases

The vast majority of patients with idiopathic rapidly progressive glomerulonephritis (RPGN) develop irreversible renal failure within weeks to months. We report a retrospective study of 10 patients with idiopathic RPGN who were treated with plasma exchange in addition to steroids and immunosuppression. Renal biopsies were obtained in nine patients. RPGN without immune complexes was present in four, immune complex disease in four, and anti-glomerular basement membrane disease in two. Renal function did not recover in those patients with anti-glomerular basement membrane disease. In contrast, seven of eight patients without auto-antibodies to basement membranes responded to therapy, and four did not need dialysis for two years or more. The sustained improvement in renal function in these seven patients suggests the need for evaluation of plasma exchange as an adjunct to steroids and immunosuppression in a prospective, controlled study in RPGN.     

Endocarditis-associated rapidly progressive glomerulonephritis mimicking vasculitis: a diagnostic and treatment challenge

BackgroundInfective endocarditis (IE)-associated rapidly progressive glomerulonephritis (RPGN) is rarely reported. Sporadic case reports have noted the diagnostic and therapeutic challenge in IE-associated glomerulonephritis because it may masquerade as idiopathic vasculitis.MethodsPatients with clinical diagnosis of IE-related RPGN in a tertiary hospital in China between January 2004 and May 2021 were identified and retrospectively reviewed.ResultsTwenty-four patients with IE-associated RPGN were identified. All patients presented with fever and multiorgan system involvement on top of heart and kidneys, spleen (79%, 19/24), skin (63%, 15/24), lung (33%, 8/24) and nervous system (17%, 4/24). Six of the 24 patients (25%) were initially suspected to have ANCA-associated or IgA vasculitis. Forty-five percent of patients are seropositive for ANCA. Renal histology showed mesangial and/or endocapillary hypercellularity with extensive crescents in most patients. C3-dominant deposition was the predominant pattern on immunofluorescence and pauci-immune necrotising crescentic glomerulonephritis was observed in one case. All patients received antibiotics with or without surgery. Six patients received immunosuppressive therapy before antibiotics due to misdiagnosis and seven patients received immunosuppressive therapy after antibiotics due to persistence of renal failure. Three of the 24 patients died due to severe infection. All the surviving patients had partial or complete recovery of renal function.ConclusionIE-associated RPGN is rare and the differential diagnosis from idiopathic vasculitis can be challenging due to overlaps in clinical manifestations, ANCA positivity and absence of typical presentations of IE. The prognosis is generally good if antibiotics and surgery are not delayed. The decision on introducing immunoruppressive treatment should be made carefully on a case by case basis when kidney function does not improve appropriately after proper anti-infective therapy.

Key messages

• Infective endocarditis associated RPGN is rare and differentiating it from idiopathic vasculitis can be challenging due to overlap in clinical manifestations, ANCA positivity and occasional absence of typical manifestations of infective endocarditis.
• Kidney function usually responds to antibiotic therapy alone.
• Immunosuppressive therapy may be beneficial in carefully selected patients whose kidney function does not improve with antibiotics alone.

     

Plasmapheresis. Technical aspects and indications

In summary, use of plasmapheresis has changed in recent years given advances in medical technology that have allowed a wider clinical application in the critical care setting. Membrane filtration technology has provided an alternative to centrifugation that can be easily applied in intensive care units. Use of plasmapheresis has also changed in recent years reflecting the availability of evidence largely obtained from controlled prospective studies. However, the clinical efficacy of plasmapheresis for many acute renal conditions is still controversial. Plasmapheresis appears to be a useful adjunct to conventional therapy in the treatment of anti-GBM nephritis, severe dialysis-dependent forms of pauciimmune RPGN, cryoglobulinemia, and HUS-TTP. Reported data also suggest a possible benefit of plasmapheresis in patients with myeloma cast nephropathy, sepsis, and poisoning/overdose, but the case for plasmapheresis in these disorders is largely unproven and the reported evidence insufficient to recommend its use outside research settings. In contrast, data from controlled trials do not support a role for plasmapheresis in immune complex-mediated RPGN, such as lupus nephritis, and acute allograft rejection. The more widespread application of prospective, randomized, controlled clinical trials should help to better define the value of plasmapheresis for treatment of acute renal diseases.     

Plasmapheresis in the treatment of rapidly progressive glomerulonephritis.

The present study was carried out to examine the efficacy of plasma exchange in patients with rapidly progressive glomerulonephritis (RPGN). Seventeen patients with RPGN were treated with plasmapheresis as adjunct to immunosuppressive therapy. Of these, 4 had antiglomerular basement membrane (GBM) antibody-mediated glomerulonephritis (GN), 8 had immune-complex GN (5 SLE, 2 HSP, 1 cryoblobulinemia), 5 had pauci-immune GN (3 peripheral antineutrophil cytoplasmic antibody [P-ANCA], 1 cytoplasmic antineutrophil cytoplasmic antibody [C-ANCA], 1 other). Treatment of 10 of these patients with plasmapheresis within the first month of disease onset resulted in a stable renal function for a period extending from 1 to 3 years, except in 2 patients who had high baseline levels of serum creatinine. In the remaining patients, 2 were treated with hemodialysis 6 years later at the end of follow-up. We conclude that plasmapheresis, when used in combination with immunosuppressive drugs, is beneficial, leading to improved renal function.     

Rapidly progressive glomerulonephritis in ANCA-associated vasculitis: a course, treatment efficacy, prognosis

AIM: To study efficacy of ANCA-RPGN treatment with corticosteroids and cyclophosphamide or mycophenolic acid drugs. MATERIAL AND METHODS: We treated 28 patients (17 males and 11 females aged 19-71 years) with morphologically verified ANCA-associated crescentic RPGN (crescentic median 79 (63:88)%. The patients received corticosteroids and cytostatics. RESULTS: The response to the treatment was registered in 22 (78%) patients in 8-16 weeks: a complete remission was achieved in 8 patients, a partial one--in 14 patients. In partial remission renal functions recovered incompletely (median Pcr 200 (180;255) mcmol/l) in persistence of moderate proteinuria (median 0.7 (0.6;1.3)g/day) and absence of microhematuria. Probability of the treatment success depended on severity of glomerulosclerosis and weakly depended on activity of extracapillary reaction. Severe renal failure was not an absolute predictor of treatment failure. CONCLUSION: In the absence of advanced nephrosclerosis early treatment with corticosteroid in combination with cytostatics can produce a positive effect in 70-80% patients with ANCA associated RPGN.     

Recent advances of clinical and basic research in rapidly progressive glomerulonephritis

Rapidly progressive glomerulonephritis(RPGN) is the most severe form of glomerulonephritis. Recently, a variety of highly specific therapeutic methods has been considered due to advances in our understandings of molecular and cellular mechanisms of glomerular crescentic formation. Although the prognosis of the patients with RPGN has improved these days, treatment related opportunistic infection and relapse are still matters of concern. Especially, in Japan, the prevalence of older patients with MPO-ANCA related RPGN is extremely high compared to other countries. To improve the prognosis of Japanese patients with RPGN, a prospective randomized trial with specific protocol for older patients with MPO-ANCA related RPGN should be conducted.