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IgA nephropathy, susceptibility to, 1(IGAN1)

MedGen UID:
463619
•Concept ID:
C3160719
•
Finding
Synonym: IGAN1
 
Monarch Initiative: MONDO:0008072
OMIM®: 161950

Clinical features

From HPO
Arthralgia
MedGen UID:
13917
•Concept ID:
C0003862
•
Sign or Symptom
Joint pain.
See: Feature record | Search on this feature
Hematuria
MedGen UID:
5488
•Concept ID:
C0018965
•
Disease or Syndrome
The presence of blood in the urine. Hematuria may be gross hematuria (visible to the naked eye) or microscopic hematuria (detected by dipstick or microscopic examination of the urine).
See: Feature record | Search on this feature
Proteinuria
MedGen UID:
10976
•Concept ID:
C0033687
•
Finding
Increased levels of protein in the urine.
See: Feature record | Search on this feature
Stage 5 chronic kidney disease
MedGen UID:
384526
•Concept ID:
C2316810
•
Disease or Syndrome
A degree of kidney failure severe enough to require dialysis or kidney transplantation for survival characterized by a severe reduction in glomerular filtration rate (less than 15 ml/min/1.73 m2) and other manifestations including increased serum creatinine.
See: Feature record | Search on this feature
IgA deposition in the glomerulus
MedGen UID:
892365
•Concept ID:
C4025827
•
Finding
The presence of immunoglobulin A deposits in the glomerulus.
See: Feature record | Search on this feature
Hypertensive disorder
MedGen UID:
6969
•Concept ID:
C0020538
•
Disease or Syndrome
The presence of chronic increased pressure in the systemic arterial system.
See: Feature record | Search on this feature
Nephritis
MedGen UID:
14328
•Concept ID:
C0027697
•
Disease or Syndrome
The presence of inflammation affecting the kidney.
See: Feature record | Search on this feature
Purpura
MedGen UID:
19584
•Concept ID:
C0034150
•
Disease or Syndrome
Purpura (from Latin
See: Feature record | Search on this feature
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Professional guidelines

PubMed

Pathogenesis and treatment of glomerulonephritis-an update.
Couser WG
J Bras Nefrol 2016 Mar;38(1):107-22. doi: 10.5935/0101-2800.20160016. PMID: 27049372
An update on the pathogenesis and treatment of IgA nephropathy.
Boyd JK, Cheung CK, Molyneux K, Feehally J, Barratt J
Kidney Int 2012 May;81(9):833-43. Epub 2012 Feb 8 doi: 10.1038/ki.2011.501. PMID: 22318424
Epidemiology, major outcomes, risk factors, prevention and management of chronic kidney disease in China.
Xie Y, Chen X
Am J Nephrol 2008;28(1):1-7. Epub 2007 Sep 20 doi: 10.1159/000108755. PMID: 17890852

Recent clinical studies

Etiology

Celiac Disease-Related Conditions: Who to Test?
Zingone F, Bai JC, Cellier C, Ludvigsson JF
Gastroenterology 2024 Jun;167(1):64-78. Epub 2024 Mar 7 doi: 10.1053/j.gastro.2024.02.044. PMID: 38460606
Factor H-Related Protein 1 Drives Disease Susceptibility and Prognosis in C3 Glomerulopathy.
Márquez-Tirado B, Gutiérrez-Tenorio J, Tortajada A, Lucientes Continente L, Caravaca-Fontán F, Malik TH, Roldán Montero R, Elías S, Saiz Gonzalez A, Fernández-Juarez G, Sánchez-Corral P, Pickering MC, Praga M, Rodríguez de Córdoba S, Goicoechea de Jorge E
J Am Soc Nephrol 2022 Jun;33(6):1137-1153. Epub 2022 May 11 doi: 10.1681/ASN.2021101318. PMID: 35545301Free PMC Article
IgA Nephropathy Susceptibility Loci and Disease Progression.
Shi M, Ouyang Y, Yang M, Yang M, Zhang X, Huang W, Wang W, Wang Z, Zhang W, Chen X, Pan X, Ren H, Chen N, Xie J
Clin J Am Soc Nephrol 2018 Sep 7;13(9):1330-1338. Epub 2018 Jul 24 doi: 10.2215/CJN.13701217. PMID: 30042224Free PMC Article
Pathogenesis and treatment of glomerulonephritis-an update.
Couser WG
J Bras Nefrol 2016 Mar;38(1):107-22. doi: 10.5935/0101-2800.20160016. PMID: 27049372
The pathophysiology of IgA nephropathy.
Suzuki H, Kiryluk K, Novak J, Moldoveanu Z, Herr AB, Renfrow MB, Wyatt RJ, Scolari F, Mestecky J, Gharavi AG, Julian BA
J Am Soc Nephrol 2011 Oct;22(10):1795-803. Epub 2011 Sep 23 doi: 10.1681/ASN.2011050464. PMID: 21949093Free PMC Article

Diagnosis

Celiac Disease-Related Conditions: Who to Test?
Zingone F, Bai JC, Cellier C, Ludvigsson JF
Gastroenterology 2024 Jun;167(1):64-78. Epub 2024 Mar 7 doi: 10.1053/j.gastro.2024.02.044. PMID: 38460606
Mutations in the alternative complement pathway in multiple myeloma patients with carfilzomib-induced thrombotic microangiopathy.
Moscvin M, Liacos CI, Chen T, Theodorakakou F, Fotiou D, Hossain S, Rowell S, Leblebjian H, Regan E, Czarnecki P, Bagnoli F, Bolli N, Richardson P, Rennke HG, Dimopoulos MA, Kastritis E, Bianchi G
Blood Cancer J 2023 Feb 27;13(1):31. doi: 10.1038/s41408-023-00802-0. PMID: 36849497Free PMC Article
Association of HLA-DRB1 and -DQB1 Alleles with Susceptibility to IgA Nephropathy in Korean Patients.
In JW, Jung K, Shin S, Park KU, Lee H, Song EY
Ann Lab Med 2022 Jan 1;42(1):54-62. doi: 10.3343/alm.2022.42.1.54. PMID: 34374349Free PMC Article
A New Vision of IgA Nephropathy: The Missing Link.
Sallustio F, Curci C, Di Leo V, Gallone A, Pesce F, Gesualdo L
Int J Mol Sci 2019 Dec 26;21(1) doi: 10.3390/ijms21010189. PMID: 31888082Free PMC Article
Current Understanding of the Role of Complement in IgA Nephropathy.
Maillard N, Wyatt RJ, Julian BA, Kiryluk K, Gharavi A, Fremeaux-Bacchi V, Novak J
J Am Soc Nephrol 2015 Jul;26(7):1503-12. Epub 2015 Feb 18 doi: 10.1681/ASN.2014101000. PMID: 25694468Free PMC Article

Therapy

Interleukin Variants Are Associated with the Development and Progression of IgA Nephropathy: A Candidate-Gene Association Study and Meta-Analysis.
Chronopoulou I, Tziastoudi M, Pissas G, Dardiotis E, Dardioti M, Golfinopoulos S, Filippidis G, Mertens PR, Tsironi EE, Liakopoulos V, Eleftheriadis T, Stefanidis I
Int J Mol Sci 2023 Nov 15;24(22) doi: 10.3390/ijms242216347. PMID: 38003536Free PMC Article
Genetic regulation of serum IgA levels and susceptibility to common immune, infectious, kidney, and cardio-metabolic traits.
Liu L, Khan A, Sanchez-Rodriguez E, Zanoni F, Li Y, Steers N, Balderes O, Zhang J, Krithivasan P, LeDesma RA, Fischman C, Hebbring SJ, Harley JB, Moncrieffe H, Kottyan LC, Namjou-Khales B, Walunas TL, Knevel R, Raychaudhuri S, Karlson EW, Denny JC, Stanaway IB, Crosslin D, Rauen T, Floege J, Eitner F, Moldoveanu Z, Reily C, Knoppova B, Hall S, Sheff JT, Julian BA, Wyatt RJ, Suzuki H, Xie J, Chen N, Zhou X, Zhang H, Hammarström L, Viktorin A, Magnusson PKE, Shang N, Hripcsak G, Weng C, Rundek T, Elkind MSV, Oelsner EC, Barr RG, Ionita-Laza I, Novak J, Gharavi AG, Kiryluk K
Nat Commun 2022 Nov 11;13(1):6859. doi: 10.1038/s41467-022-34456-6. PMID: 36369178Free PMC Article
Association of AGT M235T gene polymorphism with HSP/HSPN risk.
Mao S, Huang S
Ren Fail 2015 Feb;37(1):16-21. Epub 2014 Oct 28 doi: 10.3109/0886022X.2014.977142. PMID: 25350836
Association between transforming growth factor-β1gene-509C/T polymorphism and susceptibility of IgA nephropathy: a meta-analysis.
Li Z, Li Y, Chen L
Ren Fail 2014 Nov;36(10):1473-80. Epub 2014 Aug 12 doi: 10.3109/0886022X.2014.947517. PMID: 25112155
An update on the pathogenesis and treatment of IgA nephropathy.
Boyd JK, Cheung CK, Molyneux K, Feehally J, Barratt J
Kidney Int 2012 May;81(9):833-43. Epub 2012 Feb 8 doi: 10.1038/ki.2011.501. PMID: 22318424

Prognosis

Mesangial C3 Deposition, Complement-Associated Variant, and Disease Progression in IgA Nephropathy.
Kang Y, Xu B, Shi S, Zhou X, Chen P, Liu L, Li Y, Leng Y, Lv J, Zhu L, Zhang H
Clin J Am Soc Nephrol 2023 Dec 1;18(12):1583-1591. Epub 2023 Aug 31 doi: 10.2215/CJN.0000000000000290. PMID: 37651123Free PMC Article
Factor H-Related Protein 1 Drives Disease Susceptibility and Prognosis in C3 Glomerulopathy.
Márquez-Tirado B, Gutiérrez-Tenorio J, Tortajada A, Lucientes Continente L, Caravaca-Fontán F, Malik TH, Roldán Montero R, Elías S, Saiz Gonzalez A, Fernández-Juarez G, Sánchez-Corral P, Pickering MC, Praga M, Rodríguez de Córdoba S, Goicoechea de Jorge E
J Am Soc Nephrol 2022 Jun;33(6):1137-1153. Epub 2022 May 11 doi: 10.1681/ASN.2021101318. PMID: 35545301Free PMC Article
IgA Nephropathy Susceptibility Loci and Disease Progression.
Shi M, Ouyang Y, Yang M, Yang M, Zhang X, Huang W, Wang W, Wang Z, Zhang W, Chen X, Pan X, Ren H, Chen N, Xie J
Clin J Am Soc Nephrol 2018 Sep 7;13(9):1330-1338. Epub 2018 Jul 24 doi: 10.2215/CJN.13701217. PMID: 30042224Free PMC Article
Current Understanding of the Role of Complement in IgA Nephropathy.
Maillard N, Wyatt RJ, Julian BA, Kiryluk K, Gharavi A, Fremeaux-Bacchi V, Novak J
J Am Soc Nephrol 2015 Jul;26(7):1503-12. Epub 2015 Feb 18 doi: 10.1681/ASN.2014101000. PMID: 25694468Free PMC Article
The genetic contribution to recurrent autoimmune nephritis.
Brenchley PE, Poulton K, Morton M, Picton ML
Transplant Rev (Orlando) 2014 Jul;28(3):140-4. Epub 2014 Jan 27 doi: 10.1016/j.trre.2014.01.004. PMID: 24630158

Clinical prediction guides

Mesangial C3 Deposition, Complement-Associated Variant, and Disease Progression in IgA Nephropathy.
Kang Y, Xu B, Shi S, Zhou X, Chen P, Liu L, Li Y, Leng Y, Lv J, Zhu L, Zhang H
Clin J Am Soc Nephrol 2023 Dec 1;18(12):1583-1591. Epub 2023 Aug 31 doi: 10.2215/CJN.0000000000000290. PMID: 37651123Free PMC Article
Genetic regulation of serum IgA levels and susceptibility to common immune, infectious, kidney, and cardio-metabolic traits.
Liu L, Khan A, Sanchez-Rodriguez E, Zanoni F, Li Y, Steers N, Balderes O, Zhang J, Krithivasan P, LeDesma RA, Fischman C, Hebbring SJ, Harley JB, Moncrieffe H, Kottyan LC, Namjou-Khales B, Walunas TL, Knevel R, Raychaudhuri S, Karlson EW, Denny JC, Stanaway IB, Crosslin D, Rauen T, Floege J, Eitner F, Moldoveanu Z, Reily C, Knoppova B, Hall S, Sheff JT, Julian BA, Wyatt RJ, Suzuki H, Xie J, Chen N, Zhou X, Zhang H, Hammarström L, Viktorin A, Magnusson PKE, Shang N, Hripcsak G, Weng C, Rundek T, Elkind MSV, Oelsner EC, Barr RG, Ionita-Laza I, Novak J, Gharavi AG, Kiryluk K
Nat Commun 2022 Nov 11;13(1):6859. doi: 10.1038/s41467-022-34456-6. PMID: 36369178Free PMC Article
IgA Nephropathy Susceptibility Loci and Disease Progression.
Shi M, Ouyang Y, Yang M, Yang M, Zhang X, Huang W, Wang W, Wang Z, Zhang W, Chen X, Pan X, Ren H, Chen N, Xie J
Clin J Am Soc Nephrol 2018 Sep 7;13(9):1330-1338. Epub 2018 Jul 24 doi: 10.2215/CJN.13701217. PMID: 30042224Free PMC Article
Lessons from CKD-Related Genetic Association Studies-Moving Forward.
Limou S, Vince N, Parsa A
Clin J Am Soc Nephrol 2018 Jan 6;13(1):140-152. Epub 2017 Dec 14 doi: 10.2215/CJN.09030817. PMID: 29242368Free PMC Article
The genetic contribution to recurrent autoimmune nephritis.
Brenchley PE, Poulton K, Morton M, Picton ML
Transplant Rev (Orlando) 2014 Jul;28(3):140-4. Epub 2014 Jan 27 doi: 10.1016/j.trre.2014.01.004. PMID: 24630158

Recent systematic reviews

Association of Immune and Inflammatory Gene Polymorphism With the Risk of IgA Nephropathy: A Systematic Review and Meta-Analysis of 45 Studies.
Ding X, Mei Y, Mao Z, Long L, Han Q, You Y, Zhu H
Front Immunol 2021;12:683913. Epub 2021 Jun 30 doi: 10.3389/fimmu.2021.683913. PMID: 34354705Free PMC Article

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