Prakash Jai, Patel Prem Shankar, Prakash Suraj, Iqbal Mohd, Sharma Shiv Shankar, Singh Shivendra, Singh Usha
Professor , Corresponding Author.
Senior Resident.
J Assoc Physicians India. 2019 Nov;67(11):18-21.
Crescentic Glomerulonephritis (CGN) is most aggressive structural phenotype and accounts for 2%-7% of renal biopsy in most series. The aim of study was to assess the clinical feature and outcome of CGN at our centre.
The renal biopsy performed during the period of January 2015 to January 2018 was studied and patients showing crescentic glomerulonephritis on histology were selected for this study. The clinical presentation, immunological assay, biochemical and haematological investigations, treatment protocol and final outcome at three month of these patients were analysed in the present study.
Of 380 biopsy, 26 (male=17, female=9) patients had histological evidence of CGN (6.8%). The age of patients ranged between 13-75 (mean=43) years. Fibro cellular and cellular crescent was noted in 84.61% and 15.38% of patients respectively. Small vessels vasculitis and granuloma was observed in 5 (19.23%) cases. Based on immunohistopathology, we observed type I (n=3), type II (n=8), type III (n=5), type IV (n=3), and type V (n=7) crescentic GN in 11.53%, 30.76%, 19.23%, 11.53% and 26.92% of patients respectively. Haemodialysis was given to 22(84.61%) and 4(15.38%) patients were treated with immunosuppressive therapy. Plasmapheresis was used in two double positive (ANCA + Anti GBM Ab) patients. Remaining 21(80.76%) has progressed to ESRD over a period of 2-3 months.
Type II (immune complex) CGN was most common type followed by type V (immune negative) and type III (pauci-immune) CGN. The crescentic GN had worse prognosis with >80% of patients progressed to ESRD within 3 month of time from onset of illness. Early diagnosis and treatment is associated with favourable outcome.
新月体性肾小球肾炎(CGN)是最具侵袭性的结构表型,在大多数系列研究中占肾活检的2%-7%。本研究的目的是评估我院CGN的临床特征及预后。
对2015年1月至2018年1月期间进行的肾活检进行研究,选取组织学表现为新月体性肾小球肾炎的患者纳入本研究。分析这些患者的临床表现、免疫测定、生化及血液学检查、治疗方案以及三个月时的最终结局。
在380例活检中,26例(男性17例,女性9例)有CGN的组织学证据(6.8%)。患者年龄在13-75岁之间(平均43岁)。分别有84.61%和15.38%的患者出现纤维细胞性新月体和细胞性新月体。5例(19.23%)观察到小血管血管炎和肉芽肿。基于免疫组织病理学,我们分别在11.53%、30.76%、19.23%、11.53%和26.92%的患者中观察到I型(n=3)、II型(n=8)、III型(n=5)、IV型(n=3)和V型(n=7)新月体性肾小球肾炎。22例(84.61%)患者接受血液透析,4例(15.38%)患者接受免疫抑制治疗。两名双阳性(抗中性粒细胞胞浆抗体+抗肾小球基底膜抗体)患者接受了血浆置换。其余21例(80.76%)在2-3个月内进展为终末期肾病。
II型(免疫复合物型)CGN是最常见的类型,其次是V型(免疫阴性型)和III型(寡免疫型)CGN。新月体性肾小球肾炎预后较差,超过80%的患者在发病后3个月内进展为终末期肾病。早期诊断和治疗与良好结局相关。