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作 者:Tabassum Elahi Saima Ahmed Muhammed Mubarak
机构地区:[1]Department of Nephrology,Sindh Institute of Urology and Transplantation,Karachi 74200,Pakistan [2]Department of Histopathology,Sindh Institute of Urology and Transplantation,Karachi 74200,Pakistan
出 处:《World Journal of Nephrology》2024年第4期62-72,共11页世界肾病学杂志(英文)
摘 要:BACKGROUND 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.AIM To determine the short-term renal and patient outcomes of adults with a tissueconfirmed diagnosis of primary Ig-MCGN at a single center in Pakistan.METHODS 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.RESULTS 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.CONCLUSION 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.
关 键 词:ADULTS Immunoglobulin-associated mesangiocapillary glomerulonephritis End-stage kidney disease Kidney replacement therapy Outcomes
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