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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

Endothelin receptor antagonists for the treatment of diabetic and nondiabetic chronic kidney disease.
Smeijer JD, Kohan DE, Webb DJ, Dhaun N, Heerspink HJL
Curr Opin Nephrol Hypertens 2021 Jul 1;30(4):456-465. doi: 10.1097/MNH.0000000000000716. PMID: 33990507
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

Recent clinical studies

Etiology

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
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
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

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
Interaction between GALNT12 and C1GALT1 Associates with Galactose-Deficient IgA1 and IgA Nephropathy.
Wang YN, Zhou XJ, Chen P, Yu GZ, Zhang X, Hou P, Liu LJ, Shi SF, Lv JC, Zhang H
J Am Soc Nephrol 2021 Mar;32(3):545-552. Epub 2021 Feb 16 doi: 10.1681/ASN.2020060823. PMID: 33593824Free 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
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
Pathogenesis of IgA nephropathy.
Wada J, Sugiyama H, Makino H
Semin Nephrol 2003 Nov;23(6):556-63. doi: 10.1053/s0270-9295(03)00134-7. PMID: 14631563

Therapy

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
Cigarette smoking may accelerate the progression of IgA nephropathy.
Wang S, Qin A, Pei G, Jiang Z, Dong L, Tan J, Tan L, Tang Y, Qin W
BMC Nephrol 2021 Jun 29;22(1):239. doi: 10.1186/s12882-021-02453-4. PMID: 34187402Free PMC Article
Endothelin receptor antagonists for the treatment of diabetic and nondiabetic chronic kidney disease.
Smeijer JD, Kohan DE, Webb DJ, Dhaun N, Heerspink HJL
Curr Opin Nephrol Hypertens 2021 Jul 1;30(4):456-465. doi: 10.1097/MNH.0000000000000716. PMID: 33990507
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
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
Cigarette smoking may accelerate the progression of IgA nephropathy.
Wang S, Qin A, Pei G, Jiang Z, Dong L, Tan J, Tan L, Tang Y, Qin W
BMC Nephrol 2021 Jun 29;22(1):239. doi: 10.1186/s12882-021-02453-4. PMID: 34187402Free 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

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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