Choudhury Tauhidul Alam, Singh Rana Gopal, Singh Shivendra, Singh Takhellambam Brojen, Rathore Surendra Singh
Department of Nephrology, Dialysis and Transplant Unit, SS Hospital, Institute of Medical Sciences, Varanasi, Uttar Pradesh, India.
Saudi J Kidney Dis Transpl. 2014 May;25(3):689-96. doi: 10.4103/1319-2442.132241.
Recent data regarding the clinical and histopathologic spectrum of crescentic glomerulonephritis (CSGN) among the Indian adult population is unknown. Our aim is to study the clinicopathological features and outcome of CSGN. It is a retrospective observational study from a tertiary care hospital in India over 3.5 years. Biopsy-proven cases of CSGN (i.e., >50% crescents in glomeruli) were included in the study. Cases with insufficient data were excluded. There were 34 cases of CSGN, accounting for an incidence of 5.5% among kidney biopsies. The mean age was 32.2 ± 16.09 years, with male to female ratio of 12:22. Clinical presentations of CSGN include rapidly progressive glomerulonephritis in 23 (67.7%), chronic renal failure (CRF) in seven (20.5%), nephrotic syndrome in two (5.8%) and acute nephritic syndrome in two (5.8%) patients. The immunological profile of CSGN showed MPO-ANCA in nine (26.4%), PR3-ANCA in one (2.9%), both PR3 and MPO-ANCA in one (2.9%), anti-GBM antibody in five (14.7%) and lupus nephritis in six (17.6%) patients. All the three antibodies were present in one patient. The percentage of glomeruli showing crescents were 100% in nine (26.4%) and ≥80% in seven (20.5%) patients. Type of crescents seen were cellular in 11 (32.3%) and fibrocellular in 22 (64.7%) patients and fibrous in one (2.9%) patient. Interstitial fibrosis was found in seven (20.5%) patients. Dialysis dependency was seen in 11 (32.3%) patients. After 3 months of follow-up, mortality was seen in three (8.8%), remission in eight (23.5%), CRF in 15 (44.1%) and ESRD in five (14.7%) patients. CSGN carries a poor prognosis. The disorder may have an insidious onset and a slowly progressive course. ANCA, anti-GBM-antibody and anti-dsDNA can coexist in CSGN.
目前尚不清楚印度成年人群中新月体性肾小球肾炎(CSGN)的临床和组织病理学谱的最新数据。我们的目的是研究CSGN的临床病理特征和预后。这是一项对印度一家三级护理医院超过3.5年的回顾性观察研究。经活检证实的CSGN病例(即肾小球中新月体>50%)纳入研究。数据不足的病例被排除。共有34例CSGN病例,占肾活检病例的5.5%。平均年龄为32.2±16.09岁,男女比例为12:22。CSGN的临床表现包括23例(67.7%)快速进展性肾小球肾炎、7例(20.5%)慢性肾衰竭(CRF)、2例(5.8%)肾病综合征和2例(5.8%)急性肾炎综合征患者。CSGN的免疫谱显示9例(26.4%)为MPO-ANCA、1例(2.9%)为PR3-ANCA、1例(2.9%)同时存在PR3和MPO-ANCA、5例(14.7%)为抗GBM抗体、6例(17.6%)为狼疮性肾炎患者。一名患者同时存在这三种抗体。9例(26.4%)患者肾小球中新月体的比例为100%,7例(20.5%)患者≥80%。观察到的新月体类型为细胞性11例(32.3%)、纤维细胞性22例(64.7%)和纤维性1例(2.9%)患者。7例(20.5%)患者存在间质纤维化。11例(32.3%)患者出现透析依赖。随访3个月后,3例(8.8%)患者死亡、8例(23.5%)缓解、15例(44.1%)为CRF、5例(14.7%)为终末期肾病(ESRD)患者。CSGN预后较差。该疾病可能起病隐匿且病程进展缓慢。ANCA、抗GBM抗体和抗dsDNA可在CSGN中共存。