Pallav Gupta, Vinant Bhargava, Manish Malik, Anurag Gupta, Anil K Bhalla, Ashwini Gupta, Vaibhav Tiwari, Devinder S Rana, Ramesh L Sapra
{"title":"ANCA阴性和ANCA阳性免疫性新月体肾小球肾炎的临床病理特征和预后比较：单中心研究。","authors":"Pallav Gupta, Vinant Bhargava, Manish Malik, Anurag Gupta, Anil K Bhalla, Ashwini Gupta, Vaibhav Tiwari, Devinder S Rana, Ramesh L Sapra","doi":"10.4103/ijpm.ijpm_604_22","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Pauci-immune crescentic glomerulonephritis (PICN) is an important cause of rapidly progressive renal failure. 10-40% of PICN cases have ANCA (antineutrophil cytoplasmic antibody) negative serology. The present study compared clinico-pathologic features, Brix's renal risk score, Berden's histopathological classes and differences in outcome between ANCAnegative vs ANCA positive PICN patients.</p><p><strong>Materials and methods: </strong>Sixty-one patients of biopsy-proven PICN were studied. Biochemical findings and ANCA serology were recorded. Renal biopsy slides were reviewed along with direct immunofluorescence. Clinical and histological features were compared between ANCA negative and positive PICN using the Man Whitney U test and Chi-square test. Patients were compared for distribution in Berden's histological classes and Brix's renal risk categories. Patient and renal survival were compared using Kaplan-Meier survival analysis.</p><p><strong>Results: </strong>ANCA negative PICN patients were younger (44.9 ± 16.5 years vs 53.6 ± 15.1 years, P = 0.049). Nasal (0 vs 18%, P = 0.035) and pulmonary involvement (9% vs 38%, P = 0.014) were lower in ANCA negative group. Both ANCA groups had similar renal biochemical profiles, percentage normal glomeruli, 16.3 ± 18.2 vs 21.7 ± 20.4 and percentage glomeruli with crescents, 64.5 ± 28.1 vs 64.3 ± 27.1. Twenty-seven per cent of ANCA negative cases fell in the sclerotic class in Berden's classification vs just 2.5% in ANCA positive group (p = 0.037) without significant difference in Brix's renal risk categories (p = 0.329). Thirteen per cent of ANCA negative patients achieved complete remission on treatment compared to 33% in ANCA positive patients. Patient survival and overall probability of progressing to ESRD were similar in the two groups.</p><p><strong>Conclusion: </strong>ANCA negative PICN cases present at younger ages. Nasal and pulmonary involvement is uncommon in these patients. Patient survival and progression to ESRD are similar in both ANCA groups.</p>","PeriodicalId":13488,"journal":{"name":"Indian Journal of Pathology and Microbiology","volume":"1 1","pages":"86-91"},"PeriodicalIF":0.8000,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Comparison of Clinico-pathologic features and outcomes of ANCA negative and ANCA positive pauci immune crescentic glomerulonephritis: A single centre study.\",\"authors\":\"Pallav Gupta, Vinant Bhargava, Manish Malik, Anurag Gupta, Anil K Bhalla, Ashwini Gupta, Vaibhav Tiwari, Devinder S Rana, Ramesh L Sapra\",\"doi\":\"10.4103/ijpm.ijpm_604_22\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>Pauci-immune crescentic glomerulonephritis (PICN) is an important cause of rapidly progressive renal failure. 10-40% of PICN cases have ANCA (antineutrophil cytoplasmic antibody) negative serology. The present study compared clinico-pathologic features, Brix's renal risk score, Berden's histopathological classes and differences in outcome between ANCAnegative vs ANCA positive PICN patients.</p><p><strong>Materials and methods: </strong>Sixty-one patients of biopsy-proven PICN were studied. Biochemical findings and ANCA serology were recorded. Renal biopsy slides were reviewed along with direct immunofluorescence. Clinical and histological features were compared between ANCA negative and positive PICN using the Man Whitney U test and Chi-square test. Patients were compared for distribution in Berden's histological classes and Brix's renal risk categories. Patient and renal survival were compared using Kaplan-Meier survival analysis.</p><p><strong>Results: </strong>ANCA negative PICN patients were younger (44.9 ± 16.5 years vs 53.6 ± 15.1 years, P = 0.049). Nasal (0 vs 18%, P = 0.035) and pulmonary involvement (9% vs 38%, P = 0.014) were lower in ANCA negative group. Both ANCA groups had similar renal biochemical profiles, percentage normal glomeruli, 16.3 ± 18.2 vs 21.7 ± 20.4 and percentage glomeruli with crescents, 64.5 ± 28.1 vs 64.3 ± 27.1. Twenty-seven per cent of ANCA negative cases fell in the sclerotic class in Berden's classification vs just 2.5% in ANCA positive group (p = 0.037) without significant difference in Brix's renal risk categories (p = 0.329). Thirteen per cent of ANCA negative patients achieved complete remission on treatment compared to 33% in ANCA positive patients. Patient survival and overall probability of progressing to ESRD were similar in the two groups.</p><p><strong>Conclusion: </strong>ANCA negative PICN cases present at younger ages. Nasal and pulmonary involvement is uncommon in these patients. 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引用次数: 0
摘要
简介保济免疫性新月体肾小球肾炎（PICN）是导致快速进展性肾功能衰竭的一个重要原因。10%-40%的 PICN 病例血清 ANCA（抗中性粒细胞胞浆抗体）阴性。本研究比较了 ANCA 阴性与 ANCA 阳性 PICN 患者的临床病理特征、Brix 肾脏风险评分、Berden 组织病理学分级和预后差异：研究对象为61例经活检证实的PICN患者。记录生化检查结果和 ANCA 血清学检查结果。对肾活检切片进行了直接免疫荧光检查。使用 Man Whitney U 检验和 Chi-square 检验比较了 ANCA 阴性和阳性 PICN 的临床和组织学特征。比较患者在 Berden 组织学分级和 Brix 肾脏风险分级中的分布情况。采用 Kaplan-Meier 存活率分析比较患者和肾脏的存活率：结果：ANCA阴性的PICN患者更年轻（44.9 ± 16.5岁 vs 53.6 ± 15.1岁，P = 0.049）。ANCA阴性组鼻腔受累（0 vs 18%，P = 0.035）和肺部受累（9% vs 38%，P = 0.014）的比例较低。两组ANCA患者的肾脏生化指标相似，正常肾小球（16.3 ± 18.2 vs 21.7 ± 20.4）和有新月体的肾小球（64.5 ± 28.1 vs 64.3 ± 27.1）的比例均为16.3 ± 18.2 vs 21.7 ± 20.4。27%的ANCA阴性病例属于伯登分级中的硬化级，而ANCA阳性组仅为2.5%（p = 0.037），在布里克斯肾脏风险分级中无显著差异（p = 0.329）。13%的ANCA阴性患者在治疗后获得完全缓解，而ANCA阳性患者的这一比例为33%。两组患者的存活率和进展为 ESRD 的总概率相似：结论：ANCA阴性的PICN病例发病年龄较小。结论：ANCA 阴性的 PICN 患者年龄较轻，鼻部和肺部受累在这些患者中并不常见。两组 ANCA 患者的存活率和进展为 ESRD 的概率相似。
Comparison of Clinico-pathologic features and outcomes of ANCA negative and ANCA positive pauci immune crescentic glomerulonephritis: A single centre study.
Introduction: Pauci-immune crescentic glomerulonephritis (PICN) is an important cause of rapidly progressive renal failure. 10-40% of PICN cases have ANCA (antineutrophil cytoplasmic antibody) negative serology. The present study compared clinico-pathologic features, Brix's renal risk score, Berden's histopathological classes and differences in outcome between ANCAnegative vs ANCA positive PICN patients.
Materials and methods: Sixty-one patients of biopsy-proven PICN were studied. Biochemical findings and ANCA serology were recorded. Renal biopsy slides were reviewed along with direct immunofluorescence. Clinical and histological features were compared between ANCA negative and positive PICN using the Man Whitney U test and Chi-square test. Patients were compared for distribution in Berden's histological classes and Brix's renal risk categories. Patient and renal survival were compared using Kaplan-Meier survival analysis.
Results: ANCA negative PICN patients were younger (44.9 ± 16.5 years vs 53.6 ± 15.1 years, P = 0.049). Nasal (0 vs 18%, P = 0.035) and pulmonary involvement (9% vs 38%, P = 0.014) were lower in ANCA negative group. Both ANCA groups had similar renal biochemical profiles, percentage normal glomeruli, 16.3 ± 18.2 vs 21.7 ± 20.4 and percentage glomeruli with crescents, 64.5 ± 28.1 vs 64.3 ± 27.1. Twenty-seven per cent of ANCA negative cases fell in the sclerotic class in Berden's classification vs just 2.5% in ANCA positive group (p = 0.037) without significant difference in Brix's renal risk categories (p = 0.329). Thirteen per cent of ANCA negative patients achieved complete remission on treatment compared to 33% in ANCA positive patients. Patient survival and overall probability of progressing to ESRD were similar in the two groups.
Conclusion: ANCA negative PICN cases present at younger ages. Nasal and pulmonary involvement is uncommon in these patients. Patient survival and progression to ESRD are similar in both ANCA groups.
期刊介绍：
The journal will cover studies related to pathology including morbid anatomy, surgical pathology, clinical pathology, diagnostic cytopathology including gynecologic cytology and aspiration cytology, hematology including immuno-hematology and medical microbiology. The journal gives preference to clinically oriented studies over experimental and animal studies. The Journal would publish peer-reviewed original research papers, case reports, systematic reviews, meta-analysis, letters to the editor and brief communications. Review articles on current topics usually are invited by the editor.
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