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Membranous nephropathy(MGN)

MedGen UID:
42231
Concept ID:
C0017665
Disease or Syndrome
Synonyms: Extramembranous glomerulonephritis; Idiopathic membranous nephropathy; Membranous glomerulonephritis; Membranous GN; MGN
SNOMED CT: MGN - Membranous glomerulonephritis (77182004); Chronic nephritic syndrome, diffuse membranous glomerulonephritis (77182004); Membranous glomerulonephritis (77182004)
 
HPO: HP:0012578
Monarch Initiative: MONDO:0005376

Definition

A type of glomerulonephropathy characterized by thickening of the basement membrane and deposition of immune complexes in the subepithelial space. [from HPO]

Conditions with this feature

Autoimmune lymphoproliferative syndrome, type III caused by mutation in PRKCD
MedGen UID:
816258
Concept ID:
C3809928
Disease or Syndrome
Autoimmune lymphoproliferative syndrome type III is an autosomal recessive disorder of immune dysregulation. The phenotype is variable, but most patients have significant lymphadenopathy associated with variable autoimmune manifestations. Some patients may have recurrent infections. Lymphocyte accumulation results from a combination of impaired apoptosis and excessive proliferation (summary by Oliveira, 2013). For a general description and a discussion of genetic heterogeneity of ALPS, see 601859.
Autoinflammation, immune dysregulation, and eosinophilia
MedGen UID:
1750270
Concept ID:
C5436572
Disease or Syndrome
Autoinflammation, immune dysregulation, and eosinophilia (AIIDE) is an autosomal dominant disorder characterized by onset of severe atopic dermatitis and chronic gastrointestinal inflammation, mainly involving the colon, in infancy or early childhood. Affected individuals tend to have asthma and food or environmental allergies, as well as poor overall growth with short stature. Severe liver involvement has also been reported (Takeichi et al., 2021). Laboratory studies show increased eosinophils with normal or increased IgE levels, as well as evidence of a hyperactive immune state, including increased erythrocyte sedimentation rate and C-reactive protein. Treatment with JAK inhibitors, such as ruxolitinib and tofacitinib, results in dramatic clinical improvement (summary by Gruber et al., 2020).

Professional guidelines

PubMed

Rovin BH, Adler SG, Barratt J, Bridoux F, Burdge KA, Chan TM, Cook HT, Fervenza FC, Gibson KL, Glassock RJ, Jayne DRW, Jha V, Liew A, Liu ZH, Mejía-Vilet JM, Nester CM, Radhakrishnan J, Rave EM, Reich HN, Ronco P, Sanders JF, Sethi S, Suzuki Y, Tang SCW, Tesar V, Vivarelli M, Wetzels JFM, Lytvyn L, Craig JC, Tunnicliffe DJ, Howell M, Tonelli MA, Cheung M, Earley A, Floege J
Kidney Int 2021 Oct;100(4):753-779. doi: 10.1016/j.kint.2021.05.015. PMID: 34556300
Couser WG
Clin J Am Soc Nephrol 2017 Jun 7;12(6):983-997. Epub 2017 May 26 doi: 10.2215/CJN.11761116. PMID: 28550082Free PMC Article
Kodner C
Am Fam Physician 2016 Mar 15;93(6):479-85. PMID: 26977832

Recent clinical studies

Etiology

Sethi S, Fervenza FC
Nephrol Dial Transplant 2024 Mar 27;39(4):600-606. doi: 10.1093/ndt/gfad227. PMID: 37863839
Peritore L, Labbozzetta V, Maressa V, Casuscelli C, Conti G, Gembillo G, Santoro D
Medicina (Kaunas) 2023 Nov 14;59(11) doi: 10.3390/medicina59111997. PMID: 38004046Free PMC Article
Teisseyre M, Cremoni M, Boyer-Suavet S, Ruetsch C, Graça D, Esnault VLM, Brglez V, Seitz-Polski B
Front Immunol 2022;13:859419. Epub 2022 May 4 doi: 10.3389/fimmu.2022.859419. PMID: 35603210Free PMC Article
Caravaca-Fontán F, Fernández-Juárez GM, Floege J, Goumenos D, Kronbichler A, Turkmen K, van Kooten C, Frangou E, Stevens KI, Segelmark M, Tesar V, Anders HJ, Bruchfeld A
Nephrol Dial Transplant 2022 May 25;37(6):1033-1042. doi: 10.1093/ndt/gfab316. PMID: 34748001
Couser WG
Clin J Am Soc Nephrol 2017 Jun 7;12(6):983-997. Epub 2017 May 26 doi: 10.2215/CJN.11761116. PMID: 28550082Free PMC Article

Diagnosis

Dantas M, Silva LBB, Pontes BTM, Dos Reis MA, de Lima PSN, Moysés Neto M
J Bras Nefrol 2023 Apr-Jun;45(2):229-243. doi: 10.1590/2175-8239-JBN-2023-0046en. PMID: 37527529Free PMC Article
Liu Q, Liu J, Lin B, Zhang Y, Ma M, Yang M, Qin X
Front Immunol 2022;13:845767. Epub 2022 Apr 22 doi: 10.3389/fimmu.2022.845767. PMID: 35529848Free PMC Article
Hoxha E, Reinhard L, Stahl RAK
Nat Rev Nephrol 2022 Jul;18(7):466-478. Epub 2022 Apr 28 doi: 10.1038/s41581-022-00564-1. PMID: 35484394
Gu Y, Xu H, Tang D
Biomolecules 2021 Mar 30;11(4) doi: 10.3390/biom11040513. PMID: 33808418Free PMC Article
Sethi S
J Am Soc Nephrol 2021 Feb;32(2):268-278. Epub 2020 Dec 30 doi: 10.1681/ASN.2020071082. PMID: 33380523Free PMC Article

Therapy

Radhakrishnan Y, Zand L, Sethi S, Fervenza FC
Nephrol Dial Transplant 2024 Feb 28;39(3):403-413. doi: 10.1093/ndt/gfad225. PMID: 37934599
Dantas M, Silva LBB, Pontes BTM, Dos Reis MA, de Lima PSN, Moysés Neto M
J Bras Nefrol 2023 Apr-Jun;45(2):229-243. doi: 10.1590/2175-8239-JBN-2023-0046en. PMID: 37527529Free PMC Article
Chung EYM, Wang YM, Keung K, Hu M, McCarthy H, Wong G, Kairaitis L, Bose B, Harris DCH, Alexander SI
Front Immunol 2022;13:1036249. Epub 2022 Nov 2 doi: 10.3389/fimmu.2022.1036249. PMID: 36405681Free PMC Article
Alsharhan L, Beck LH Jr
Am J Kidney Dis 2021 Mar;77(3):440-453. Epub 2021 Jan 21 doi: 10.1053/j.ajkd.2020.10.009. PMID: 33487481
Fervenza FC, Appel GB, Barbour SJ, Rovin BH, Lafayette RA, Aslam N, Jefferson JA, Gipson PE, Rizk DV, Sedor JR, Simon JF, McCarthy ET, Brenchley P, Sethi S, Avila-Casado C, Beanlands H, Lieske JC, Philibert D, Li T, Thomas LF, Green DF, Juncos LA, Beara-Lasic L, Blumenthal SS, Sussman AN, Erickson SB, Hladunewich M, Canetta PA, Hebert LA, Leung N, Radhakrishnan J, Reich HN, Parikh SV, Gipson DS, Lee DK, da Costa BR, Jüni P, Cattran DC; MENTOR Investigators
N Engl J Med 2019 Jul 4;381(1):36-46. doi: 10.1056/NEJMoa1814427. PMID: 31269364

Prognosis

Sethi S, Fervenza FC
Nephrol Dial Transplant 2024 Mar 27;39(4):600-606. doi: 10.1093/ndt/gfad227. PMID: 37863839
Dantas M, Silva LBB, Pontes BTM, Dos Reis MA, de Lima PSN, Moysés Neto M
J Bras Nefrol 2023 Apr-Jun;45(2):229-243. doi: 10.1590/2175-8239-JBN-2023-0046en. PMID: 37527529Free PMC Article
Liu Q, Liu J, Lin B, Zhang Y, Ma M, Yang M, Qin X
Front Immunol 2022;13:845767. Epub 2022 Apr 22 doi: 10.3389/fimmu.2022.845767. PMID: 35529848Free PMC Article
Sethi S, Madden BJ, Debiec H, Charlesworth MC, Gross L, Ravindran A, Hummel AM, Specks U, Fervenza FC, Ronco P
J Am Soc Nephrol 2019 Jun;30(6):1123-1136. Epub 2019 May 6 doi: 10.1681/ASN.2018080852. PMID: 31061139Free PMC Article
Keri KC, Blumenthal S, Kulkarni V, Beck L, Chongkrairatanakul T
Postgrad Med J 2019 Jan;95(1119):23-31. Epub 2019 Jan 25 doi: 10.1136/postgradmedj-2018-135729. PMID: 30683678

Clinical prediction guides

Sealfon R, Mariani L, Avila-Casado C, Nair V, Menon R, Funk J, Wong A, Lerner G, Hayashi N, Troyanskaya O, Kretzler M, Beck LH Jr
J Am Soc Nephrol 2022 Jun;33(6):1208-1221. Epub 2022 Apr 27 doi: 10.1681/ASN.2021060784. PMID: 35477557Free PMC Article
Alsharhan L, Beck LH Jr
Am J Kidney Dis 2021 Mar;77(3):440-453. Epub 2021 Jan 21 doi: 10.1053/j.ajkd.2020.10.009. PMID: 33487481
Dubin RF, Rhee EP
Clin J Am Soc Nephrol 2020 Mar 6;15(3):404-411. Epub 2019 Oct 21 doi: 10.2215/CJN.07420619. PMID: 31636087Free PMC Article
McAdoo SP, Pusey CD
Clin J Am Soc Nephrol 2017 Jul 7;12(7):1162-1172. Epub 2017 May 17 doi: 10.2215/CJN.01380217. PMID: 28515156Free PMC Article
Dahan K, Debiec H, Plaisier E, Cachanado M, Rousseau A, Wakselman L, Michel PA, Mihout F, Dussol B, Matignon M, Mousson C, Simon T, Ronco P; GEMRITUX Study Group
J Am Soc Nephrol 2017 Jan;28(1):348-358. Epub 2016 Jun 27 doi: 10.1681/ASN.2016040449. PMID: 27352623Free PMC Article

Recent systematic reviews

Bose B, Chung EYM, Hong R, Strippoli GFM, Johnson DW, Yang WL, Badve SV, Palmer SC
J Nephrol 2022 May;35(4):1159-1170. Epub 2022 Feb 23 doi: 10.1007/s40620-022-01268-2. PMID: 35199314Free PMC Article
Kaegi C, Wuest B, Crowley C, Boyman O
Front Immunol 2021;12:788830. Epub 2022 Feb 2 doi: 10.3389/fimmu.2021.788830. PMID: 35185862Free PMC Article
von Groote TC, Williams G, Au EH, Chen Y, Mathew AT, Hodson EM, Tunnicliffe DJ
Cochrane Database Syst Rev 2021 Nov 15;11(11):CD004293. doi: 10.1002/14651858.CD004293.pub4. PMID: 34778952Free PMC Article
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article
McGrogan A, Franssen CF, de Vries CS
Nephrol Dial Transplant 2011 Feb;26(2):414-30. Epub 2010 Nov 10 doi: 10.1093/ndt/gfq665. PMID: 21068142

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