{"title":"保济免疫新月体肾小球肾炎的预后及其预后因素;单中心病例系列。","authors":"Neda Najafi, Sharzad Ossareh, Mitra Mehrazma, Mohsen Vahedi","doi":"","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Pauci-immune crescentic glomerulonephritis (GN) is the most common cause of rapidly progressive GN in adults. The aim of this study was to determine the outcome of patients with pauci-immune crescentic GN and risk factors of the development of end-stage kidney disease (ESKD) in these patients.</p><p><strong>Methods: </strong>This case series study was carried on 120 patients with pauci-immune crescentic GN biopsied in our center betwen 1998 and 2016. Inclusion criteria were age > 16 years, at least one crescentic glomerulus, maximally 1+ deposition of immunoglobulins and complement components at fluorescent microscopy, and at least 6 months follow-up. The main outcomes were ESKD and death.</p><p><strong>Results: </strong>The study population included 120 patients with pauciimmune crescentic GN (mean age was 47 ± 17 years and 49.1% male). There was no significant difference in outcome between patients with diffuse or focal crescentic GN. Seventy-two patients (60%) developed ESKD and 31 patients (25.8%) died. The need for dialysis at admission, lower baseline hemoglobin and GFR and GFR at four months and high percentage of glomerulosclerosis and interstitial fibrosis had a significant relationship with low kidney survival (P < .05). The rate of ESKD was higher in patients who did not receive cyclophosphamide therapy, due to focal crescentic GN or high chronicity, compared to patients who received it (70.7 vs. 28.5%, P < .001).</p><p><strong>Conclusion: </strong>In our study, a high percentage of patients with pauciimmune crescentic GN developed ESKD. Low first GFR and high chronicity in biopsy were associated with lower kidney survival. Failure to administer cyclophosphamide in seemingly limited or advanced cases, together with late referral may have led to poor prognosis. DOI: 10.52547/ijkd.7545.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"1 1","pages":"9-17"},"PeriodicalIF":0.8000,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"The Outcome of Pauci-immune Crescentic Glomerulonephritis and Its Prognostic Factors; A single Center Case Series.\",\"authors\":\"Neda Najafi, Sharzad Ossareh, Mitra Mehrazma, Mohsen Vahedi\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>Pauci-immune crescentic glomerulonephritis (GN) is the most common cause of rapidly progressive GN in adults. The aim of this study was to determine the outcome of patients with pauci-immune crescentic GN and risk factors of the development of end-stage kidney disease (ESKD) in these patients.</p><p><strong>Methods: </strong>This case series study was carried on 120 patients with pauci-immune crescentic GN biopsied in our center betwen 1998 and 2016. Inclusion criteria were age > 16 years, at least one crescentic glomerulus, maximally 1+ deposition of immunoglobulins and complement components at fluorescent microscopy, and at least 6 months follow-up. The main outcomes were ESKD and death.</p><p><strong>Results: </strong>The study population included 120 patients with pauciimmune crescentic GN (mean age was 47 ± 17 years and 49.1% male). There was no significant difference in outcome between patients with diffuse or focal crescentic GN. Seventy-two patients (60%) developed ESKD and 31 patients (25.8%) died. The need for dialysis at admission, lower baseline hemoglobin and GFR and GFR at four months and high percentage of glomerulosclerosis and interstitial fibrosis had a significant relationship with low kidney survival (P < .05). The rate of ESKD was higher in patients who did not receive cyclophosphamide therapy, due to focal crescentic GN or high chronicity, compared to patients who received it (70.7 vs. 28.5%, P < .001).</p><p><strong>Conclusion: </strong>In our study, a high percentage of patients with pauciimmune crescentic GN developed ESKD. Low first GFR and high chronicity in biopsy were associated with lower kidney survival. Failure to administer cyclophosphamide in seemingly limited or advanced cases, together with late referral may have led to poor prognosis. DOI: 10.52547/ijkd.7545.</p>\",\"PeriodicalId\":14610,\"journal\":{\"name\":\"Iranian journal of kidney diseases\",\"volume\":\"1 1\",\"pages\":\"9-17\"},\"PeriodicalIF\":0.8000,\"publicationDate\":\"2024-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Iranian journal of kidney diseases\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"UROLOGY & NEPHROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Iranian journal of kidney diseases","FirstCategoryId":"3","ListUrlMain":"","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
引用次数: 0
摘要
导言:贫免疫性新月体肾小球肾炎(GN)是导致成人快速进展性肾小球肾炎的最常见原因。本研究旨在确定贫免疫性新月体肾小球肾炎患者的预后以及这些患者发展为终末期肾病(ESKD)的风险因素:本病例系列研究的对象是1998年至2016年间在本中心进行活检的120例贫免疫新月体GN患者。纳入标准为年龄大于16岁,至少有一个新月体肾小球,荧光显微镜下免疫球蛋白和补体成分沉积最大值为1+,随访至少6个月。主要结果为ESKD和死亡:研究对象包括120名低免疫新月体GN患者(平均年龄为47±17岁,49.1%为男性)。弥漫性或局灶性新月体 GN 患者的预后无明显差异。72名患者(60%)发展为ESKD,31名患者(25.8%)死亡。入院时需要透析、基线血红蛋白和 GFR 较低、4 个月时 GFR 较低、肾小球硬化和间质纤维化比例高与肾脏存活率低有显著关系(P < .05)。与接受环磷酰胺治疗的患者相比,因局灶性新月体 GN 或高度慢性化而未接受环磷酰胺治疗的患者 ESKD 发生率更高(70.7% 对 28.5%,P < .001):结论:在我们的研究中,高比例的低免疫新月体 GN 患者发展为 ESKD。首次肾小球滤过率低和活检慢性化程度高与肾脏存活率较低有关。在看似局限性或晚期病例中未使用环磷酰胺,加上转诊较晚,可能导致预后不良。 DOI: 10.52547/ijkd.7545.
The Outcome of Pauci-immune Crescentic Glomerulonephritis and Its Prognostic Factors; A single Center Case Series.
Introduction: Pauci-immune crescentic glomerulonephritis (GN) is the most common cause of rapidly progressive GN in adults. The aim of this study was to determine the outcome of patients with pauci-immune crescentic GN and risk factors of the development of end-stage kidney disease (ESKD) in these patients.
Methods: This case series study was carried on 120 patients with pauci-immune crescentic GN biopsied in our center betwen 1998 and 2016. Inclusion criteria were age > 16 years, at least one crescentic glomerulus, maximally 1+ deposition of immunoglobulins and complement components at fluorescent microscopy, and at least 6 months follow-up. The main outcomes were ESKD and death.
Results: The study population included 120 patients with pauciimmune crescentic GN (mean age was 47 ± 17 years and 49.1% male). There was no significant difference in outcome between patients with diffuse or focal crescentic GN. Seventy-two patients (60%) developed ESKD and 31 patients (25.8%) died. The need for dialysis at admission, lower baseline hemoglobin and GFR and GFR at four months and high percentage of glomerulosclerosis and interstitial fibrosis had a significant relationship with low kidney survival (P < .05). The rate of ESKD was higher in patients who did not receive cyclophosphamide therapy, due to focal crescentic GN or high chronicity, compared to patients who received it (70.7 vs. 28.5%, P < .001).
Conclusion: In our study, a high percentage of patients with pauciimmune crescentic GN developed ESKD. Low first GFR and high chronicity in biopsy were associated with lower kidney survival. Failure to administer cyclophosphamide in seemingly limited or advanced cases, together with late referral may have led to poor prognosis. DOI: 10.52547/ijkd.7545.
期刊介绍:
The Iranian Journal of Kidney Diseases (IJKD), a peer-reviewed journal in English, is the official publication of the Iranian Society of Nephrology. The aim of the IJKD is the worldwide reflection of the knowledge produced by the scientists and clinicians in nephrology. Published quarterly, the IJKD provides a new platform for advancement of the field. The journal’s objective is to serve as a focal point for debates and exchange of knowledge and experience among researchers in a global context. Original papers, case reports, and invited reviews on all aspects of the kidney diseases, hypertension, dialysis, and transplantation will be covered by the IJKD. Research on the basic science, clinical practice, and socio-economics of renal health are all welcomed by the editors of the journal.