Elahi Tabassum, Ahmed Saima, Mubarak Muhammed
Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi 74200, Pakistan.
Department of Histopathology, Sindh Institute of Urology and Transplantation, Karachi 74200, Pakistan.
World J Nephrol. 2024 Dec 25;13(4):98969. doi: 10.5527/wjn.v13.i4.98969.
Primary immunoglobulin (Ig)-associated mesangiocapillary glomerulonephritis (Ig-MCGN) is an immune complex glomerulonephritis of unknown etiology. It is a common cause of chronic kidney disease in developing countries. There is limited data available on renal and patient outcomes of this disease from developing countries.
To determine the short-term renal and patient outcomes of adults with a tissue-confirmed diagnosis of primary Ig-MCGN at a single center in Pakistan.
A retrospective cohort study of adult patients was conducted on biopsy-proven Ig-MCGN cases diagnosed between 1998 and 2019 at the Sindh Institute of Urology and Transplantation, Karachi, Pakistan. Secondary causes were excluded. The primary endpoint was renal survival without end-stage kidney disease (ESKD) or mortality. The secondary endpoint was the rate of remission during the 2-year follow-up period. Survival curves were made with the use of Kaplan-Meier estimates.
A total of 163 patients were included in the study and their mean follow-up duration was 29.45 months ± 21.28 months. Among baseline characteristics, young age, lower estimated glomerular filtration rate, requirement of kidney replacement therapy, presence of crescents, and severity of interstitial fibrosis and tubular atrophy were found to have a significant association with renal outcomes. The renal outcomes were negatively correlated with the presence of hypertension, level of complements, and degree of proteinuria. In all, 63 (37.4%) patients were treated with steroids and 21 (13%) received combination therapy (cyclophosphamide with steroids). At 2 years, 124 (76.07%) patients were in complete remission or partial remission [56 (34.3%) and 68 (41.71%), respectively], while 32 (19.63%) patients progressed to ESKD and 7 (4.29%) patients died.
The outcomes of primary Ig-MCGN are guarded in Pakistan and require further prospective studies to improve our understanding of this relatively common disease so that more personalized treatment approaches can be developed.
原发性免疫球蛋白(Ig)相关的系膜毛细血管性肾小球肾炎(Ig-MCGN)是一种病因不明的免疫复合物性肾小球肾炎。它是发展中国家慢性肾脏病的常见病因。来自发展中国家的关于该疾病肾脏及患者预后的数据有限。
确定在巴基斯坦一个中心经组织学确诊的原发性Ig-MCGN成年患者的短期肾脏及患者预后。
对1998年至2019年在巴基斯坦卡拉奇信德泌尿与移植研究所经活检证实为Ig-MCGN的成年患者进行回顾性队列研究。排除继发原因。主要终点是无终末期肾病(ESKD)或死亡的肾脏存活率。次要终点是2年随访期内的缓解率。采用Kaplan-Meier估计法绘制生存曲线。
共163例患者纳入研究,平均随访时间为29.45个月±21.28个月。在基线特征中,发现年轻、较低的估计肾小球滤过率、肾脏替代治疗需求、新月体的存在以及间质纤维化和肾小管萎缩的严重程度与肾脏预后显著相关。肾脏预后与高血压的存在、补体水平及蛋白尿程度呈负相关。共有63例(37.4%)患者接受了类固醇治疗,21例(13%)接受了联合治疗(环磷酰胺与类固醇)。2年时,124例(76.07%)患者完全缓解或部分缓解[分别为56例(34.3%)和68例(41.71%)],而32例(19.63%)患者进展为ESKD,7例(4.29%)患者死亡。
在巴基斯坦,原发性Ig-MCGN的预后不容乐观,需要进一步的前瞻性研究来增进我们对这种相对常见疾病的了解,以便制定更个性化的治疗方法。