Primary Nephritic Syndromes
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Wooin AhnDivision of Nephrology, Columbia University, Vagelos College of Physicians and Surgeons New York, New York

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Jonathan J. HoganDivision of Nephrology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania

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Ali G. GharaviDivision of Nephrology, Columbia University, Vagelos College of Physicians and Surgeons, New York, New York

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  • 1.

    Kiryluk K , Li Y , Sanna-Cherchi S , Rohanizadegan M , Suzuki H , Eitner F , et al.: Geographic differences in genetic susceptibility to IgA nephropathy: GWAS replication study and geospatial risk analysis. PLoS Genet 8: e1002765, 2012 PubMed

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  • 2.

    Yu GZ , Guo L , Dong JF , Shi SF , Liu LJ , Wang JW , et al.: Persistent Hematuria and Kidney Disease Progression in IgA Nephropathy: A Cohort Study. Am J Kidney Dis 76: 9099, 2020 PubMed

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  • 3.

    Rodrigues JC , Haas M , Reich HN : IgA nephropathy. Clin J Am Soc Nephrol 12: 677686, 2017 PubMed

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    Barratt J , Feehally J : Treatment of IgA nephropathy. Kidney Int 69: 19341938, 2006 PubMed

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    Herlitz LC , Bomback AS , Stokes MB , Radhakrishnan J , D’Agati VD , Markowitz GS : IgA nephropathy with minimal change disease. Clin J Am Soc Nephrol 9: 10331039, 2014 PubMed

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  • 6.

    Mariani LH , Bomback AS , Canetta PA , Flessner MF , Helmuth M , Hladunewich MA , et al.; CureGN Consortium: CureGN study rationale, design, and methods: Establishing a large prospective observational study of glomerular disease. Am J Kidney Dis 73: 218229, 2019 PubMed

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  • 7.

    Selewski DT , Ambruzs JM , Appel GB , Bomback AS , Matar RB , Cai Y , et al.; CureGN Consortium: Clinical characteristics and treatment patterns of children and adults with IgA nephropathy or IgA vasculitis: Findings from the CureGN study. Kidney Int Rep 3: 13731384, 2018 PubMed

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  • 8.

    Delbarba E , Marasa M , Canetta PA , Piva SE , Chatterjee D , Kil BH , et al.; CureGN Consortium: Persistent disease activity in patients with long-standing glomerular disease. Kidney Int Rep 5: 860871, 2020 PubMed

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  • 9,

    Chen S , Tang Z , Xiang H , Li X , Chen H , Zhang H , et al.: Etiology and outcome of crescentic glomerulonephritis from a single center in China: A 10-year review. Am J Kidney Dis 67: 376383, 2016 PubMed

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    Shima Y , Nakanishi K , Hama T , Mukaiyama H , Sato M , Tanaka Y , et al.: Crescentic IgA nephropathy in children. Pediatr Nephrol 35: 10051014, 2020 PubMed

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  • 11.

    Bellur SS , Lepeytre F , Vorobyeva O , Troyanov S , Cook HT , Roberts IS ; International IgA Nephropathy Working Group: Evidence from the Oxford classification cohort supports the clinical value of subclassification of focal segmental glomerulosclerosis in IgA nephropathy. Kidney Int 91: 235243, 2017 PubMed

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  • 12.

    Cattran DC , Coppo R , Cook HT , Feehally J , Roberts ISD , Troyanov S , et al.; Working Group of the International IgA Nephropathy Network and the Renal Pathology Society: The Oxford classification of IgA nephropathy: Rationale, clinicopathological correlations, and classification. Kidney Int 76: 534545, 2009 PubMed

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  • 13.

    Trimarchi H , Barratt J , Cattran DC , Cook HT , Coppo R , Haas M , et al.; IgAN Classification Working Group of the International IgA Nephropathy Network and the Renal Pathology Society; Conference Participants: Oxford classification of IgA nephropathy 2016: An update from the IgA Nephropathy Classification Working Group. Kidney Int 91: 10141021, 2017 PubMed

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  • 14.

    Jullien P , Laurent B , Berthoux F , Masson I , Dinic M , Claisse G , et al.: Repeat renal biopsy improves the Oxford classification-based prediction of immunoglobulin A nephropathy outcome. Nephrol Dial Transplant 35: 11791186, 2020 PubMed.

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    Cai Q , Shi S , Wang S , Ren Y , Hou W , Liu L , et al.: Microangiopathic lesions in IgA nephropathy: A cohort study. Am J Kidney Dis 74: 629639, 2019 PubMed

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    Satoskar AA , Suleiman S , Ayoub I , Hemminger J , Parikh S , Brodsky SV , et al.: Staphylococcus infection-associated GN: Spectrum of IgA staining and prevalence of ANCA in a single-center cohort. Clin J Am Soc Nephrol 12: 3949, 2017 PubMed

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    Satoskar AA , Molenda M , Scipio P , Shim R , Zirwas M , Variath RS , et al.: Henoch-Schönlein purpura-like presentation in IgA-dominant Staphylococcus infection - associated glomerulonephritis - a diagnostic pitfall. Clin Nephrol 79: 302312, 2013 PubMed

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    Brodsky SV , Nadasdy T , Cassol C , Satoskar A : IgA staining patterns differentiate between IgA nephropathy and IgA-dominant infection-associated glomerulonephritis. Kidney Int Rep 5: 909911, 2020 PubMed

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  • 19.

    Reily C , Stewart TJ , Renfrow MB , Novak J : Glycosylation in health and disease. Nat Rev Nephrol 15: 346366, 2019 PubMed

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    Novak J , Barratt J , Julian BA , Renfrow MB : Aberrant glycosylation of the IgA1 molecule in IgA nephropathy. Semin Nephrol 38: 461476, 2018 PubMed

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  • 21.

    Gharavi AG , Moldoveanu Z , Wyatt RJ , Barker CV , Woodford SY , Lifton RP , et al.: Aberrant IgA1 glycosylation is inherited in familial and sporadic IgA nephropathy. J Am Soc Nephrol 19: 10081014, 2008 PubMed

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  • 22.

    Kiryluk K , Li Y , Moldoveanu Z , Suzuki H , Reily C , Hou P , et al.: GWAS for serum galactose-deficient IgA1 implicates critical genes of the O-glycosylation pathway. PLoS Genet 13: e1006609, 2017 PubMed

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  • 23.

    Gale DP , Molyneux K , Wimbury D , Higgins P , Levine AP , Caplin B , et al.: Galactosylation of IgA1 is associated with common variation in C1GALT1. J Am Soc Nephrol 28: 21582166, 2017 PubMed

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  • 24.

    Kiryluk K , Li Y , Scolari F , Sanna-Cherchi S , Choi M , Verbitsky M , et al.: Discovery of new risk loci for IgA nephropathy implicates genes involved in immunity against intestinal pathogens. Nat Genet 46: 11871196, 2014 PubMed

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    Bhutani G , Nasr SH , Said SM , Sethi S , Fervenza FC , Morice WG , et al.: Hematologic characteristics of proliferative glomerulonephritides with nonorganized monoclonal immunoglobulin deposits. Mayo Clin Proc 90: 587596, 2015 PubMed

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  • 26.

    Thompson A , Carroll K , Inker LA , Floege J , Perkovic V , Boyer-Suavet S , et al.: Proteinuria reduction as a surrogate end point in trials of IgA nephropathy. Clin J Am Soc Nephrol 14: 469481, 2019 PubMed

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  • 27.

    Knoop T , Vikse BE , Mwakimonga A , Leh S , Bjørneklett R : Long-term outcome in 145 patients with assumed benign immunoglobulin A nephropathy. Nephrol Dial Transplant 32: 18411850, 2017 PubMed

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  • 28.

    Bobart SA , Alexander MP , Shawwa K , Vaughan LE , Ghamrawi R , Sethi S , et al.: The association of microhematuria with mesangial hypercellularity, endocapillary hypercellularity, crescent score and renal outcomes in immunoglobulin A nephropathy. Nephrol Dial Transplant: gfz267, 2019 PubMed

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    Sim JJ , Bhandari SK , Batech M , Hever A , Harrison TN , Shu YH , et al.: End-stage renal disease and mortality outcomes across different glomerulonephropathies in a large diverse US population. Mayo Clin Proc 93: 167178, 2018 PubMed

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    Barbour SJ , Coppo R , Zhang H , Liu ZH , Suzuki Y , Matsuzaki K , et al.; International IgA Nephropathy Network: Evaluating a new international risk-prediction tool in IgA nephropathy. JAMA Intern Med 179: 942952, 2019 PubMed

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    Coppo R , D’Arrigo G , Tripepi G , Russo ML , Roberts ISD , Bellur S , et al.; ERA-EDTA Immunonephrology Working Group: Is there long-term value of pathology scoring in immunoglobulin A nephropathy? A validation study of the Oxford classification for IgA nephropathy (VALIGA) update. Nephrol Dial Transplant 35: 10021009, 2020 PubMed

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    Jarrick S , Lundberg S , Welander A , Carrero JJ , Höijer J , Bottai M , et al.: Mortality in IgA nephropathy: A nationwide population-based cohort study. J Am Soc Nephrol 30: 866876, 2019 PubMed

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    Praga M , Gutiérrez E , González E , Morales E , Hernández E : Treatment of IgA nephropathy with ACE inhibitors: A randomized and controlled trial. J Am Soc Nephrol 14: 15781583, 2003 PubMed

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  • 34.

    Hirano K , Kawamura T , Tsuboi N , Okonogi H , Miyazaki Y , Ikeda M , et al.: The predictive value of attenuated proteinuria at 1 year after steroid therapy for renal survival in patients with IgA nephropathy. Clin Exp Nephrol 17: 555562, 2013 PubMed

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  • 35.

    Russo E , Verzola D , Salvidio G , Bonino B , Picciotto D , Drovandi S , et al.: Long-term blood pressure behavior and progression to end-stage renal disease in patients with immunoglobulin A nephropathy: A single-center observational study in Italy. J Hypertens 38: 925935, 2020 PubMed

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    Rauen T , Eitner F , Fitzner C , Sommerer C , Zeier M , Otte B , et al.; STOP-IgAN Investigators: Intensive supportive care plus immunosuppression in IgA nephropathy. N Engl J Med 373: 22252236, 2015 PubMed

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    Rauen T , Wied S , Fitzner C , Eitner F , Sommerer C , Zeier M , et al.; STOP-IgAN Investigators: After ten years of follow-up, no difference between supportive care plus immunosuppression and supportive care alone in IgA nephropathy. Kidney Int 98: 10441052, 2020 , 2020 PubMed

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  • 39.

    Lv J , Zhang H , Wong MG , Jardine MJ , Hladunewich M , Jha V , et al.; TESTING Study Group: Effect of oral methylprednisolone on clinical outcomes in patients with IgA nephropathy: The TESTING randomized clinical trial. JAMA 318: 432442, 2017 PubMed

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  • 40.

    Fellström BC , Barratt J , Cook H , Coppo R , Feehally J , de Fijter JW , et al.; NEFIGAN Trial Investigators: Targeted-release budesonide versus placebo in patients with IgA nephropathy (NEFIGAN): A double-blind, randomised, placebo-controlled phase 2b trial. Lancet 389: 21172127, 2017 PubMed

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    Zand L , Canetta P , Lafayette R , Aslam N , Jan N , Sethi S , et al.: An open-label pilot study of adrenocorticotrophic hormone in the treatment of IgA nephropathy at high risk of progression. kidney int rep 5: 5865, 2019 PubMed

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    Xiaowei L , Bo W , Li L , Peng Z : Comparison of the effects of valsartan plus activated vitamin D versus valsartan alone in IgA nephropathy with moderate proteinuria. Int Urol Nephrol 52: 129136, 2020 PubMed

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    Allen PJ , Chadban SJ , Craig JC , Lim WH , Allen RDM , Clayton PA , et al.: Recurrent glomerulonephritis after kidney transplantation: Risk factors and allograft outcomes. Kidney Int 92: 461469, 2017 PubMed

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    O’Shaughnessy MM , Liu S , Montez-Rath ME , Lenihan CR , Lafayette RA , Winkelmayer WC : Kidney transplantation outcomes across GN subtypes in the United States. J Am Soc Nephrol 28: 632644, 2017 PubMed

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  • 45.

    Kousios A , Duncan N , Charif R , Tam FWK , Levy J , Cook HT , et al.: Autologous stem cell transplant for the treatment of type I crystal cryoglobulinemic glomerulonephritis caused by monoclonal gammopathy of renal significance (MGRS). Kidney Int Rep 4: 13421348, 2019 PubMed

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  • 46.

    Smith RJH , Appel GB , Blom AM , Cook HT , D’Agati VD , Fakhouri F , et al.: C3 glomerulopathy: Understanding a rare complement-driven renal disease. Nat Rev Nephrol 15: 129143, 2019 PubMed

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    Avasare RS , Canetta PA , Bomback AS , Marasa M , Caliskan Y , Ozluk Y , et al.: Mycophenolate mofetil in combination with steroids for treatment of C3 glomerulopathy: A case series. Clin J Am Soc Nephrol 13: 406413, 2018 PubMed

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    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 93: 977985, 2018 PubMed

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    Medjeral-Thomas N , Malik TH , Patel MP , Toth T , Cook HT , Tomson C , et al.: A novel CFHR5 fusion protein causes C3 glomerulopathy in a family without Cypriot ancestry. Kidney Int 85: 933937, 2014 PubMed

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    Ravindran A , Fervenza FC , Smith RJH , Sethi S : C3 glomerulonephritis with a severe crescentic phenotype. Pediatr Nephrol 32: 16251633, 2017 PubMed

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    Andeen NK , Yang HY , Dai DF , MacCoss MJ , Smith KD : DnaJ homolog subfamily B member 9 is a putative autoantigen in fibrillary GN. J Am Soc Nephrol 29: 231239, 2018 PubMed

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    Hogan JJ , Alexander MP , Leung N : Dysproteinemia and the kidney: Core curriculum 2019. Am J Kidney Dis 74: 822836, 2019 PubMed

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    Ravindran A , Fervenza FC , Smith RJH , Sethi S : C3 glomerulopathy associated with monoclonal Ig is a distinct subtype. Kidney Int 94: 178186, 2018 PubMed

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    Levine AP , Chan MMY , Sadeghi-Alavijeh O , Wong EKS , Cook HT , Ashford S , et al.; MPGN/DDD/C3 Glomerulopathy Rare Disease Group; NIHR BioResource: Large-scale whole-genome sequencing reveals the genetic architecture of primary membranoproliferative GN and C3 glomerulopathy. J Am Soc Nephrol 31: 365373, 2020 PubMed

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    Le Quintrec M , Lapeyraque AL , Lionet A , Sellier-Leclerc AL , Delmas Y , Baudouin V , et al.: Patterns of clinical response to eculizumab in patients with C3 glomerulopathy. Am J Kidney Dis 72: 8492, 2018 PubMed

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