IgG4-related kidney disease

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O.B. Iaremenko
D.I. Koliadenko


IgG4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disease characterized by the formation of sclerotic tumor-like masses with dense lymphoplasmacytic infiltrates containing a significant number of IgG4-plasma cells. IgG4-RD is characterized by a wide range of clinical manifestations since the disease can involve almost any organ. Renal da­mage is observed in about 15 % of patients with IgG4-RD. The article presents a review of data about clinical manifestations of IgG4-related kidney disease (IgG4-RKD), specific features of diagnosis, and modern treatment approaches. The review of scientific publications was conducted in the international electronic scientometric database PubMed using keywords “IgG4-related kidney disease”, “IgG4-RKD radiographic findings”, “IgG4-related tubulointerstitial nephritis”, “IgG4-related membranous glomerulonephritis” over the period 2012–2020. The most common renal lesions in IgG4-RD are tubulointerstitial nephritis and membranous glomerulonephritis. They occur predominantly in older males. At the time of diagnosis, patients mostly already have concomitant extrarenal manifestations of IgG4-RD (sialadenitis, pancreatitis, lymphadenopathy). The clinical picture is usually vague, although nephrotic syndrome and chronic renal failure may occur. Typical manifestations of IgG4-RKD on CT are multiple low-dense foci, thickening of the renal pelvis, diffuse kidney enlargement. Renal involvement in IgG4-RD is often accompanied by low levels of complement. Early detection and treatment of IgG4-RKD are extremely important due to the increase in morbidity and mortality due to the development of chronic kidney disease. Glucocorticoids (GC) are first-line drugs for IgG4-RD. Despite a good response to GC, even a slight delay in treatment can lead to the development of renal tissue atrophy. The use of immunosuppressive drugs is advisable in cases when the dose of GC cannot be reduced due to the constantly high activity of the di­sease. Rituximab (a CD20+ B-lymphocyte inhibitor) may be used in patients with a recurrent course of IgG4-RKD.

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How to Cite
Iaremenko, O., and D. Koliadenko. “IgG4-Related Kidney Disease”. KIDNEYS, vol. 10, no. 2, July 2021, pp. 114-23, doi:10.22141/2307-1257.10.2.2021.234332.


Wallace ZS, Naden RP, Chari S, et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis. 2020 Jan;79(1):77-87. doi:10.1136/annrheumdis-2019-216561.

Stone JH, Khosroshahi A, Deshpande V, et al. Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum. 2012 Oct;64(10):3061-3067. doi:10.1002/art.34593.

Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012 Feb;22(1):21-30. doi:10.1007/s10165-011-0571-z.

Khosroshahi A, Wallace ZS, Crowe JL, et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015 Jul;67(7):1688-1699. doi:10.1002/art.39132.

Uchida K, Masamune A, Shimosegawa T, Okazaki K. Prevalence of IgG4-Related Disease in Japan Based on Nationwide Survey in 2009. Int J Rheumatol. 2012;2012:358371. doi:10.1155/2012/358371.

Yaremenko OB, Kolyadenko DI, Petelytska LB. IgG4-related disease: current status of the problem and description of the case report. Ukrainian Journal of Rheumatology. 2019;(75):10-19. (in Ukrainian).

Sebastian A, Sebastian M, Misterska-Skóra M, et al. The variety of clinical presentations in IgG4-related disease in Rheumatology. Rheumatol Int. 2018 Feb;38(2):303-309. doi:10.1007/s00296-017-3807-1.

Sedhom R, Sedhom D, Strair R. IgG4-related disease: a mini-review. J Rare Dis Res Treat. 2017;2(2):18-23. doi:10.29245/2572-9411/2017/2.1089.

Weindorf SC, Frederiksen JK. IgG4-Related Disease: A Reminder for Practicing Pathologists. Arch Pathol Lab Med. 2017 Nov;141(11):1476-1483. doi:10.5858/arpa.2017-0257-RA.

Cortazar FB, Stone JH. IgG4-related disease and the kidney. Nat Rev Nephrol. 2015 Oct;11(10):599-609. doi:10.1038/nrneph.2015.95.

Saeki T, Kawano M. IgG4-related kidney disease. Kidney Int. 2014 Feb;85(2):251-257. doi:10.1038/ki.2013.393.

Kawano M, Yamada K. IgG4-Related Kidney Disease and IgG4-Related Retroperitoneal Fibrosis. Semin Liver Dis. 2016 Aug;36(3):283-290. doi:10.1055/s-0036-1584316.

Kawano M, Saeki T. IgG4-related kidney disease--an update. Curr Opin Nephrol Hypertens. 2015 Mar;24(2):193-201. doi:10.1097/MNH.0000000000000102.

Oh JW, Rha SE, Choi MH, Oh SN, Youn SY, Choi JI. Immunoglobulin G4-related Disease of the Genitourinary System: Spectrum of Imaging Findings and Clinical-Pathologic Features. Radiographics. 2020 Sep-Oct;40(5):1265-1283. doi:10.1148/rg.2020200043.

Salvadori M, Tsalouchos A. Immunoglobulin G4-related kidney diseases: An updated review. World J Nephrol. 2018 Jan 6;7(1):29-40. doi:10.5527/wjn.v7.i1.29.

Zhang NN, Wang YY, Kong LX, Zou WZ, Dong B. IgG4-related kidney disease (IgG4-RKD) with membranous nephropathy as its initial manifestation: report of one case and literature review. BMC Nephrol. 2019 Jul 16;20(1):263. doi:10.1186/s12882-019-1419-6.

Fujimori N, Ito T, Igarashi H, et al. Retroperitoneal fibrosis associated with immunoglobulin G4-related disease. World J Gastroenterol. 2013 Jan 7;19(1):35-41. doi:10.3748/wjg.v19.i1.35.

Eroglu E, Sipahioglu MH, Senel S, et al. Successful treatment of tubulointerstitial nephritis in immunoglobulin G4-related disease with rituximab: A case report. World J Clin Cases. 2019 Aug 26;7(16):2309-2315. doi:10.12998/wjcc.v7.i16.2309.

Nada R, Ramachandran R, Kumar A, et al. IgG4-related tubulointerstitial nephritis: A prospective analysis. Int J Rheum Dis. 2016 Jul;19(7):721-729. doi:10.1111/1756-185X.12675.

Pradhan D, Pattnaik N, Silowash R, Mohanty SK. IgG4-related kidney disease--A review. Pathol Res Pract. 2015 Oct;211(10):707-711. doi:10.1016/j.prp.2015.03.004.

Boffa JJ, Esteve E, Buob D. Renal involvement in IgG4-related disease. Presse Med. 2020 Apr;49(1):104017. doi:10.1016/j.lpm.2020.104017.

Mann S, Seidman MA, Barbour SJ, Levin A, Carruthers M, Chen LY. Recognizing IgG4-related tubulointerstitial nephritis. Can J Kidney Health Dis. 2016 Jul 17;3:34. doi:10.1186/s40697-016-0126-5.

Teng F, Lu H, Zheng K, et al. Urinary System Manifestation of IgG4-Related Disease: Clinical, Laboratory, Radiological, and Pathological Spectra of a Chinese Single-Centre Study. J Immunol Res. 2020 Jul 3;2020:5851842. doi:10.1155/2020/5851842.

Ebbo M, Grados A, Samson M, et al. Long-term efficacy and safety of rituximab in IgG4-related disease: Data from a French nationwide study of thirty-three patients. PLoS One. 2017 Sep 15;12(9):e0183844. doi:10.1371/journal.pone.0183844.