Primary immune complex membranoproliferative glomerulonephritis and C3 glomerulonephritis: experience of two rare diseases from a single centre

Tonmay Barai; Koushik Bhatttacharjee; Keya Basu; Sanjay Dasgupta

doi:10.18203/2320-6012.ijrms20252031

Authors

Tonmay Barai Department of Nephrology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
Koushik Bhatttacharjee Department of Nephrology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
Keya Basu Department of Pathology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
Sanjay Dasgupta Department of Nephrology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

DOI:

https://doi.org/10.18203/2320-6012.ijrms20252031

Keywords:

C3 GN, MPGN, Primary immune complex MPGN

Abstract

Background: This study has been conducted to evaluate and compare the clinicopathological profile and treatment outcome of primary immune complex membranoproliferative glomerulonephritis (IC-MPGN) and C3 glomerulonephritis (C3 GN), two rare glomerular diseases and to find any predictive factors for renal and patient outcome.

Methods: A retrospective observational cross-sectional study, conducted at a tertiary care hospital. Patients with biopsy-proven MPGN were included after satisfying the inclusion and exclusion criteria. Detailed history, clinical examinations, laboratory investigations and kidney biopsy were noted. Outcome was recorded at months 3, 6, 12 and at the last available follow-up.

Results: Of the 24 subjects enrolled, 11 (45.8%) had primary IC-MPGN and 13 (54.2%) had C3 GN. Nephrotic syndrome is the prevalent presentation. No statistically significant difference was found in respect to clinical and biochemical parameters between two groups. Complement mediated group had more crescent and features of chronicity in renal biopsy. Renal and patient outcome was significantly better in immune mediated group compared to complement mediated group (log rank p=0.015); including complete remission (p=0.003), proteinuria reduction at 3 months (p=0.009), at the last known follow-up (p=0.005). During follow up, hematuria, higher serum creatinine and low median serum albumin were significantly more common in complement mediated group. No significant predictors were found for renal outcome and outcome was not influenced by treatment modality.

Conclusions: IC-MPGN and C3 GN are rare glomerular diseases. Although phenotypically similar, C3 GN showed poor outcome. Further multicentric study may help to better understand these diseases to find cost effective treatment.

Metrics

Metrics Loading ...

References

Yadla M. Membranoproliferative glomerulonephritis-current understanding. Clin Queries Nephrol. 2013;2(3):126-30. DOI: https://doi.org/10.1016/j.cqn.2013.07.001

Briganti EM, Dowling J, Finlay M, Hill PA, Jones CL, Kincaid-Smith PS, et al. The incidence of biopsy-proven glomerulonephritis in Australia. Nephrol Dial Transplant Off Publ Eur Dial Transpl Assoc - Eur Ren Assoc. 2001;16(7):1364-7.

Pickering MC, D’Agati VD, Nester CM, Smith RJ, Haas M, Appel GB, et al. C3 glomerulopathy: consensus report. Kidney Int. 2013;84(6):1079-89. DOI: https://doi.org/10.1038/ki.2013.377

Cook HT, Pickering MC. Histopathology of MPGN and C3 glomerulopathies. Nat Rev Nephrol. 2015;11:14-22. DOI: https://doi.org/10.1038/nrneph.2014.217

Caravaca-Fontán F, Lucientes L, Cavero T, Praga M. Update on C3 glomerulopathy: a complement-mediated disease. Nephron. 2020;144:272-80. DOI: https://doi.org/10.1159/000507254

Alchi B, Jayne D. Membranoproliferative glomerulonephritis. Pediatr Nephrol. 2010;25:1409-18. DOI: https://doi.org/10.1007/s00467-009-1322-7

Salvadori M, Rosso G, Bertoni E. Complement involvement in kidney diseases: From physiopathology to therapeutical targeting. World J Nephrol. 2015;4(2):169-84. DOI: https://doi.org/10.5527/wjn.v4.i2.169

Cansick JC, Lennon R, Cummins CL, Howie AJ, McGraw ME, Saleem MA, et al. Prognosis, treatment and outcome of childhood mesangiocapillary (membranoproliferative) glomerulonephritis. Nephrol Dial Transplant Off Publ Eur Dial Transpl Assoc - Eur Ren Assoc. 2004;19(11):2769-77. DOI: https://doi.org/10.1093/ndt/gfh484

Lu DF, Moon M, Lanning LD, McCarthy AM, Smith RJH. Clinical features and outcomes of 98 children and adults with dense deposit disease. Pediatr Nephrol Berl Ger. 2012;27(5):773-81. DOI: https://doi.org/10.1007/s00467-011-2059-7

Kirpalani A, Jawa N, Smoyer WE, Licht C, Midwest Pediatric Nephrology Consortium. Long-Term Outcomes of C3 Glomerulopathy and Immune-Complex Membranoproliferative Glomerulonephritis in Children. Kidney Int Rep. 2020;5(12):2313-24. DOI: https://doi.org/10.1016/j.ekir.2020.09.019

Briganti EM, Dowling J, Finlay M, Hill PA, Jones CL, Kincaid-Smith PS, et al. The incidence of biopsy-proven glomerulonephritis in Australia. Nephrol Dial Transplant Off Publ Eur Dial Transpl Assoc - Eur Ren Assoc. 2001;16(7):1364-7. DOI: https://doi.org/10.1093/ndt/16.7.1364

Bomback AS, Santoriello D, Avasare RS, Regunathan-Shenk R, Canetta PA, Ahn W, et al. C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy. Kidney Int. 2018;93(4):977-85. DOI: https://doi.org/10.1016/j.kint.2017.10.022

Nakano M, Karasawa K, Moriyama T, Uchida K, Nitta K. Characteristics of membranoproliferative glomerulonephritis based on a new classification at a single center. Clin Exp Nephrol. 2019;23(6):852-8. DOI: https://doi.org/10.1007/s10157-019-01716-7

Kawasaki Y, Kanno S, Ono A, Suzuki Y, Ohara S, Sato M, et al. Differences in clinical findings, pathology, and outcomes between C3 glomerulonephritis and membranoproliferative glomerulonephritis. Pediatr Nephrol. 2016;31(7):1091-9. DOI: https://doi.org/10.1007/s00467-015-3307-z

Okuda Y, Ishikura K, Hamada R, Harada R, Sakai T, Hamasaki Y, et al. Membranoproliferative glomerulonephritis and C3 glomerulonephritis: frequency, clinical features, and outcome in children. Nephrol Carlton Vic. 2015;20(4):286-92. DOI: https://doi.org/10.1111/nep.12382

Woo SA, Young Ju H, Hyo Kwon S, Lee JH, Jeong Choi S, Cheol Han D, et al. Reanalysis of membranoproliferative glomerulonephritis patients according to the new classification: a multicenter study. Kidney Res Clin Pract. 2014;33(4):187-91. DOI: https://doi.org/10.1016/j.krcp.2014.07.006

Iatropoulos P, Noris M, Mele C, Piras R, Valoti E, Bresin E, et al. Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome. Mol Immunol. 2016;71:131-42. DOI: https://doi.org/10.1016/j.molimm.2016.01.010

Nakagawa N, Mizuno M, Kato S, Maruyama S, Sato H, Nakaya I, et al. Demographic, clinical characteristics and treatment outcomes of immune-complex membranoproliferative glomerulonephritis and C3 glomerulonephritis in Japan: A retrospective analysis of data from the Japan Renal Biopsy Registry. PloS One. 2021;16(9):e0257397. DOI: https://doi.org/10.1371/journal.pone.0257397

Aksoy E, Mirioglu S, Uludag O, Ozluk Y, Demir E, Caliskan Y, et al. Comparison of various features and outcomes in adult patients with immune complex membranoproliferative glomerulonephritis and C3 glomerulopathy. Nephrol Dial Transplant. 2019;34(Supplement_1):gfz103.SP181. DOI: https://doi.org/10.1093/ndt/gfz103.SP181

Meena P, Bhargava V, Mnams DNB, Gupta V. Clinico-pathological features and predictors of End-Stage Renal Disease (ESRD) in C3 glomerulopathy: A retrospective study. Clin Nephrol Res. 2019;3(1).