Background: Idiopathic form of mesangioproliferative glomerulopathy (MesPGP) was excluded by the standardized classification and reporting of glomerulonephritis (Mayo clinic consensus) in 2016 aiming to limit such group only to IgA nephropathy, IgA vasculitis, infection-related GP, lupus nephritis, and fibrillary GP with polyclonal Ig deposits in an attempt to aid in treatment. The case: A 32-year-old man presented with severe hypertension that was associated with progressive renal failure (serum creatinine at 358 umol/L), proteinuria and hematuria. Clinical assessment and laboratory testing did not show evidence of infection and autoimmune disease. Kidney biopsy showed MesPGP with 39% glomerulosclerosis and 60% interstitial fibrosis. Immunohistochemical studies showed 2(+) IgG and IgA deposits over the peripheral capillary loops not mesangium. C3 and C1q were negative. Electron microscopy scanning showed subepithelial, subendothelial and mesangial electron dense deposits. Hence, diagnosis of idiopathic MesPGP was established. He improved after intravenous Solumedrol followed by a tapering dose of Prednisone with Mycophenolate mofetil for 3 months. Subsequently, he remained in remission up to 1 year with Rituximab alone and will be using it for a minimum of 2 years. Conclusion: Acute idiopathic immune complex MesPGP should be reincluded in classification of glomerulopathy and was amenable to immunosuppressive therapy.
References
[1]
Floege, J., Barbour, S.J., Cattran, D.C., Hogan, J.J., Nachman, P.H., Tang, S.C.W., et al. (2019) Management and Treatment of Glomerular Diseases (Part 1): Conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. KidneyInternational, 95, 268-280. https://doi.org/10.1016/j.kint.2018.10.018
[2]
Saran, R., Robinson, B., Abbott, K.C., Agodoa, L.Y.C., Bragg-Gresham, J., et al. (2019) US Renal Data System 2018 Annual Data Report: Epidemiology of Kidney Disease in the United States. American Journal of Kidney Diseases, 73, A7-A8.
[3]
Wu, M.Y., Chen, Y.C., Chiu, I.J. and Wu, M.S. (2022) Genetic Insight into Primary Glomerulonephritis. Nephrology, 27, 649-657. https://doi.org/10.1111/nep.14074
[4]
McGrogan, A., Franssen, C.F.M. and de Vries, C.S. (2010) The Incidence of Primary Glomerulonephritis Worldwide: A Systematic Review of the Literature. NephrologyDialysisTransplantation, 26, 414-430. https://doi.org/10.1093/ndt/gfq665
[5]
Sethi, S. and Fervenza, F.C. (2018) Standardized Classification and Reporting of Glomerulonephritis. NephrologyDialysisTransplantation, 34, 193-199. https://doi.org/10.1093/ndt/gfy220
[6]
Sethi, S., Haas, M., Markowitz, G.S., D’Agati, V.D., Rennke, H.G., Jennette, J.C., et al. (2015) Mayo Clinic/Renal Pathology Society Consensus Report on Pathologic Classification, Diagnosis, and Reporting of GN. JournaloftheAmericanSocietyofNephrology, 27, 1278-1287. https://doi.org/10.1681/asn.2015060612
[7]
Fervenza, F.C., Sethi, S. and Glassock, R.J. (2012) Idiopathic Membranoproliferative Glomerulonephritis: Does It Exist? NephrologyDialysisTransplantation, 27, 4288-4294. https://doi.org/10.1093/ndt/gfs288
[8]
Sethi, S. and Fervenza, F.C. (2012) Membranoproliferative Glomerulonephritis—A New Look at an Old Entity. NewEnglandJournalofMedicine, 366, 1119-1131. https://doi.org/10.1056/nejmra1108178
[9]
Cattran, D.C., Coppo, R., Cook, H.T., Feehally, J., Roberts, I.S.D., Troyanov, S., et al. (2009) The Oxford Classification of Iga Nephropathy: Rationale, Clinicopathological Correlations, and Classification. KidneyInternational, 76, 534-545. https://doi.org/10.1038/ki.2009.243
[10]
Servais, A., Frémeaux-Bacchi, V., Lequintrec, M., Salomon, R., Blouin, J., Knebelmann, B., et al. (2006) Primary Glomerulonephritis with Isolated C3 Deposits: A New Entity Which Shares Common Genetic Risk Factors with Haemolytic Uraemic Syndrome. JournalofMedicalGenetics, 44, 193-199. https://doi.org/10.1136/jmg.2006.045328
[11]
Nasr, S.H., Radhakrishnan, J. and D’Agati, V.D. (2013) Bacterial Infection-Related Glomerulonephritis in Adults. KidneyInternational, 83, 792-803. https://doi.org/10.1038/ki.2012.407
[12]
Retamozo, S., Quartuccio, L. and Ramos-Casals, M. (2022) Crioglobulinemia. MedicinaClínica, 158, 478-487. https://doi.org/10.1016/j.medcli.2021.11.017
[13]
Gokhale, A., Chancay, J., Shapiro, R., Randhawa, P. and Menon, M.C. (2021) Chronic Transplant Glomerulopathy: New Insights into Pathogenesis. ClinicalTransplantation, 35, e14214. https://doi.org/10.1111/ctr.14214
[14]
Wilson, G.J., Cho, Y., Teixiera-Pinto, A., Isbel, N., Campbell, S., Hawley, C., et al. (2019) Long-Term Outcomes of Patients with End-Stage Kidney Disease Due to Membranoproliferative Glomerulonephritis: An ANZDATA Registry Study. BMCNephrology, 20, Article No. 417. https://doi.org/10.1186/s12882-019-1605-6
[15]
Yuan, M., Zou, J., Zhang, X., Liu, H., Teng, J., Zhong, Y., et al. (2010) Combination Therapy with Mycophenolate Mofetil and Prednisone in Steroid-Resistant Idiopathic Membranoproliferative Glomerulonephritis. ClinicalNephrology, 73, 354-359. https://doi.org/10.5414/cnp73354
[16]
Couser, W.G. and Johnson, R.J. (1994) Mechanisms of Progressive Renal Disease in Glomerulonephritis. AmericanJournalofKidneyDiseases, 23, 193-198. https://doi.org/10.1016/s0272-6386(12)80971-1