Sp181comparison of Various Features and Outcomes in Adult Patients With Immune Complex Membranoproliferative Glomerulonephritis and C3 Glomerulopathy

Aksoy, Elif; Mirioglu, Safak; Uludag, Omer; Demir, Erol; Çalışkan, Yaşar; Yazıcı, Halil; Kiliçaslan, İşın; Türkmen, Aydın; Sever, Mehmet Şükrü

doi:10.1093/ndt/gfz103.sp181

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“…Revised diagnosis is based on revisions of renal biopsy reports by the 2 study pathologists based on the new C3G consensus criteria. 1 , 17 C3G, C3 glomerulopathy; IC-MPGN, immune-complex membranoproliferative glomerulonephritis; PIGN, postinfectious glomerulonephritis. …”

Section: Resultsmentioning

confidence: 99%

“…In a retrospective study of 20 adult patients with IC-MPGN and 15 with C3G followed for a median duration of 68 months, no significant differences were noted in renal survival or treatment response between patients in either disease group. 17 Within the multiple small cohort studies of children with IC-MPGN/C3G conducted postreclassification, some have demonstrated worse renal prognoses in C3G, 3 , 18 whereas others showed no significant differences between the groups. 19 These studies have all been limited by small sample sizes.…”

Section: Discussionmentioning

confidence: 99%

“… Staining for C3 is seen in the glomeruli Immunofluorescence for C3 is at least 2 orders of magnitude more than any other immune reactant a Based on consensus criteria. 1 , 17 …”

Section: Methodsmentioning

confidence: 99%

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Long-Term Outcomes of C3 Glomerulopathy and Immune-Complex Membranoproliferative Glomerulonephritis in Children

Kirpalani

Jawa

Smoyer

et al. 2020

Kidney International Reports

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Introduction: The reclassification of membranoproliferative glomerulonephritis (MPGN) into immunecomplex MPGN (IC-MPGN) and C3 glomerulopathy (C3G) has provided insights into 2 distinct diseases. Although outcomes in adults are poor in both diseases, the pediatric literature is scarce and limited to small, single-center cohorts. Methods: We conducted a retrospective analysis of 165 pediatric patients across 17 hospitals to compare outcomes between children with IC-MPGN and C3G. Results: Forty-two percent of patients initially diagnosed with MPGN were reclassified as C3G after a review of renal biopsy reports. There was a trend toward higher serum creatinine levels in patients with C3G compared with IC-MPGN both at diagnosis (mean 168.9 [range 45.4-292.4] vs. 93.7 [range 70.7-116.6] mmol/l, P ¼ 0.25) and after a mean follow-up time of 4 years (mean 145.0 (range À8.1 to 298.1) vs 99.1 (range 46.3-151.9) mmol/l, P ¼ 0.47), although the estimated glomerular filtration rate (eGFR) was not significantly different. Steroid treatment was associated with a significant improvement in eGFR versus no steroids in C3G (mean þ43.0 (range 12.9-73.0) vs. À3.0 (range À23.1 to 17.2) ml/min per 1.73 m 2 , P ¼ 0.02) but not in IC-MPGN. Overall kidney function was preserved in both groups although hypertension remained prevalent in 42.5% of the cohort at the last follow-up, and the urine protein/creatinine ratio remained elevated (mean 253.8 [range 91.9-415.7] mg/mmol). Conclusion: This large pediatric IC-MPGN/C3G cohort revealed nearly half of the patients were misclassified, and there may be a trend toward worse renal prognosis in C3G although they may have greater steroid responsiveness. The overall prognosis appears to be more favorable than in adults; however, persistent hypertension and proteinuria suggest suboptimal disease control.

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Section: Resultsmentioning

confidence: 99%