An enigmatic case of IgG4-related nephropathy and an updated review of the literature.
IgG4
IgG4-related disease
IgG4-related nephropathy
Retroperitoneal fibrosis
Rituximab
Storiform fibrosis
Journal
Clinical and experimental medicine
ISSN: 1591-9528
Titre abrégé: Clin Exp Med
Pays: Italy
ID NLM: 100973405
Informations de publication
Date de publication:
Aug 2021
Aug 2021
Historique:
received:
29
10
2020
accepted:
12
02
2021
pubmed:
9
3
2021
medline:
1
1
2022
entrez:
8
3
2021
Statut:
ppublish
Résumé
IgG4-related disease (IgG4-RD) is still an underestimated disorder which affects multiple organs, and its recognition as a distinct clinical disease has been only proved in the recent decades. The renal involvement has been documented in approximately 15% of patients with IgG4-RD, and the typical manifestation is a tubulo-interstitial nephritis. The main histological findings in IgG4-RD are typically a dense tissue infiltration of IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis, and frequently elevated IgG4 serum levels. Herein we report our atypical and peculiar clinical presentation of an IgG4-related nephropathy (IgG4-RN) and the remarkable response to rituximab (RTX) treatment at the renal imaging with computerized tomography assessment. The current nephrological evidences support the renal function recovery after steroids or steroids plus RTX therapy, even if the renal imaging data are not always shown. In a complex and enigmatic clinical scenario such as the IgG4-RN, both the renal biopsy and the renal imaging before and after the immunosuppressive therapy become mandatory tools to thoroughly define the diagnosis, the management and the response to the immunological therapy.
Identifiants
pubmed: 33683496
doi: 10.1007/s10238-021-00696-x
pii: 10.1007/s10238-021-00696-x
doi:
Substances chimiques
Rituximab
4F4X42SYQ6
Prednisone
VB0R961HZT
Types de publication
Case Reports
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
493-500Références
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;64(10):3061–7. https://doi.org/10.1002/art.34593 .
doi: 10.1002/art.34593
pubmed: 22736240
pmcid: 5963880
Bledsoe JR, Della-Torre E, Rovati L, Deshpande V. IgG4-related disease: review of the histopathologic features, differential diagnosis, and therapeutic approach. APMIS. 2018;126(6):459–76. https://doi.org/10.1111/apm.12845 .
doi: 10.1111/apm.12845
pubmed: 29924455
Deshpande V. IgG4-related disease. Introduction Semin Diagn Pathol. 2012;29(4):175–6. https://doi.org/10.1053/j.semdp.2012.07.006 .
doi: 10.1053/j.semdp.2012.07.006
pubmed: 23068295
Sarles H, Sarles JC, Muratore R, Guien C. Chronic inflammatory sclerosis of the pancreas–an autonomous pancreatic disease? Am J Dig Dis. 1961;6:688–98.
doi: 10.1007/BF02232341
Aoki S, Nakazawa T, Ohara H, et al. Immunohistochemical study of autoimmune pancreatitis using anti-IgG4 antibody and patients’ sera. Histopathology. 2005;47(2):147–58.
doi: 10.1111/j.1365-2559.2005.02204.x
Deshpande V, Mino-Kenudson M, Brugge W, Lauwers GY. Autoimmune pancreatitis: more than just a pancreatic disease? A contemporary review of its pathology. Arch Pathol Lab Med. 2005;129(9):1148–54.
doi: 10.5858/2005-129-1148-APMTJA
Igarashi H, Ito T, Oono T, Nakamura T, et al. Relationship between pancreatic and/or extrapancreatic lesions and serum IgG and IgG4 levels in IgG4-related diseases. J Dig Dis. 2012;13(5):274–9. https://doi.org/10.1111/j.1751-2980.2012.00583.x .
doi: 10.1111/j.1751-2980.2012.00583.x
pubmed: 22500790
Raissian Y, Nasr SH, Larsen CP, et al. Diagnosis of IgG4-related tubulointerstitial nephritis. J Am Soc Nephrol. 2011;22(7):1343–52. https://doi.org/10.1681/ASN.2011010062 .
doi: 10.1681/ASN.2011010062
pubmed: 21719792
pmcid: 3137582
Saeki T, Nishi S, Imai N, et al. Clinicopathological characteristics of patients with IgG4-related tubulointerstitialnephritis. Kidney Int. 2010;78(10):1016–23. https://doi.org/10.1038/ki.2010.271 .
doi: 10.1038/ki.2010.271
pubmed: 20720530
Cortazar FB, Stone JH. IgG4-related disease and the kidney. Nat Rev Nephrol. 2015;11(10):599–609. https://doi.org/10.1038/nrneph.2015.95 .
doi: 10.1038/nrneph.2015.95
pubmed: 26122730
Pradhan D, Pattnaik N, Silowash R, Mohanty SK. IgG4-related kidney disease–A review. Pathol Res Pract. 2015;211(10):707–11. https://doi.org/10.1016/j.prp.2015.03.004 .
doi: 10.1016/j.prp.2015.03.004
pubmed: 26341570
Zhang P, Cornell LD. IgG4-related tubulointerstitial nephritis. Adv Chronic Kidney Dis. 2017;24(2):94–100. https://doi.org/10.1053/j.ackd.2016.12.001 .
doi: 10.1053/j.ackd.2016.12.001
pubmed: 28284385
Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181–92. https://doi.org/10.1038/modpathol.2012.72 .
doi: 10.1038/modpathol.2012.72
pubmed: 22596100
Salvadori M, Tsalouchos A. Immunoglobulin G4-related kidney diseases: an updated review. Word J Nephrol. 2018;7(19):29–40.
doi: 10.5527/wjn.v7.i1.29
Hara S, Kawano M, Mizushima I, et al. Distribution and components of interstitial inflammation and fibrosis in IgG4-related kidney disease: analysis of autopsy specimens. Hum Pathol. 2016;55:164–73. https://doi.org/10.1016/j.humpath.2016.05.010 .
doi: 10.1016/j.humpath.2016.05.010
pubmed: 27246178
Raissian Y, Nasr SH, Larsen CP, et al. Diagnosis of IgG4-related tubulointerstitial nephritis. J Am Soc Nephrol. 2011. https://doi.org/10.1681/ASN.2011010062 .
doi: 10.1681/ASN.2011010062
pubmed: 21719792
pmcid: 3137582
Kawano M, Saeki T. IgG4-related kidney disease–an update. Curr Op in Nephrol Hypertens. 2015;24(2):193–201. https://doi.org/10.1097/MNH.0000000000000102 .
doi: 10.1097/MNH.0000000000000102
Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet. 2006;367(9506):241–51.
doi: 10.1016/S0140-6736(06)68035-5
Quattrocchio G, Roccatello D. IgG4-related nephropathy. J Nephrol. 2016;29(4):487–93. https://doi.org/10.1007/s40620-016-0279-4 .
doi: 10.1007/s40620-016-0279-4
pubmed: 26972314
Wallace ZS, Deshpande V, Mattoo H, et al. IgG4-related disease: clinical and laboratory features in one hundred twenty-five patients. Arthritis Rheumatol. 2015;67(9):2466–75. https://doi.org/10.1002/art.39205 .
doi: 10.1002/art.39205
pubmed: 25988916
pmcid: 4621270
Kawano M, Saeki T, Nakashima H, et al. Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol. 2011;15(5):615–26. https://doi.org/10.1007/s10157-011-0521-2 .
doi: 10.1007/s10157-011-0521-2
pubmed: 21898030
Kim B, Kim JH, Byun JH, et al. IgG4-related kidney disease: MRI findings with emphasis on the usefulness of diffusion-weighted imaging. Eur J Radiol. 2014;83(7):1057–62. https://doi.org/10.1016/j.ejrad.2014.03.033 .
doi: 10.1016/j.ejrad.2014.03.033
pubmed: 24768583
Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539–51. https://doi.org/10.1056/NEJMra1104650 .
doi: 10.1056/NEJMra1104650
pubmed: 22316447
Kamisawa T, Okazaki K. Diagnosis and Treatment of IgG4-Related Disease. Curr Top Microbiol Immunol. 2017;401:19–33. https://doi.org/10.1007/82_2016_36 .
doi: 10.1007/82_2016_36
pubmed: 28197739
Khosroshahi A, Wallace ZS. Crowe JL et al International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015;67(7):1688–99. https://doi.org/10.1002/art.39132 .
doi: 10.1002/art.39132
pubmed: 25809420
Masaki Y, Shimizu H, Sato Nakamura T, et al. IgG4-related disease: diagnostic methods and therapeutic strategies in Japan. J Clin Exp Hematop. 2014;54(2):95–101.
doi: 10.3960/jslrt.54.95
Kamisawa T, Shimosegawa T, Okazaki K, et al. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58:1504–7.
doi: 10.1136/gut.2008.172908
Ghazale A, Chari ST. Optimising corticosteroid treatment for autoimmune pancreatitis. Gut. 2007;56:1650–2.
doi: 10.1136/gut.2007.129833
Ghazale A, Chari ST, Zhang L, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134:706–15.
doi: 10.1053/j.gastro.2007.12.009
Raina A, Yadav D, Krasinskas AM, et al. Evaluation and management of autoimmune pancreatitis: experience at a large US center. Am J Gastroenterol. 2009;104:2295–306.
doi: 10.1038/ajg.2009.325
Kamisawa T, Okazaki K, Kawa S, et al. Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013. III. Treatment and prognosis of autoimmune pancreatitis. J Gastroenterol. 2014;49:961–70.
doi: 10.1007/s00535-014-0945-z
Mizushima I, Yamada K, Fujii H, et al. Clinical and histological changes associated with corticosteroid therapy in IgG4-related tubulointerstitial nephritis. Mod Rheumatol. 2012;22(6):859–70. https://doi.org/10.1007/s10165-011-0589-2 .
doi: 10.1007/s10165-011-0589-2
pubmed: 22262474
pmcid: 3496477
Mise N, Tomizawa Y, Fujii A, Yamaguchi Y, Sugimoto T. A case of tubulointerstitial nephritis in IgG4-related systemic disease with markedly enlarged kidneys. NDT Plus. 2009;2(3):233–5. https://doi.org/10.1093/ndtplus/sfp023 .
doi: 10.1093/ndtplus/sfp023
pubmed: 25983998
pmcid: 4421185
Carruthers MN, Topazian MD, Khosroshahi A, et al. Rituximab for IgG4-related disease: a prospective, open-label trial. Ann Rheum Dis. 2015;74(6):1171–7. https://doi.org/10.1136/annrheumdis-2014-206605 .
doi: 10.1136/annrheumdis-2014-206605
pubmed: 25667206
Yamamoto M, Awakawa T, Takahashi H. Is rituximab effective for IgG4-related disease in the long term? Experience of cases treated with rituximab for 4 years. Ann Rheum Dis. 2015;74(8):e46.
doi: 10.1136/annrheumdis-2015-207625
Khosroshahi A, Carruthers MN, Deshpande V, Unizony S, Bloch DB, Stone JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore). 2012;91(1):57–66. https://doi.org/10.1097/MD.0b013e3182431ef6 .
doi: 10.1097/MD.0b013e3182431ef6
Roccatello D, Sciascia S, Rossi D, Alpa M, Naretto C, Baldovino S. Intensive short-term treatment with rituximab, cyclophosphamide and methylprednisolone pulses induces remission in severe cases of SLE with nephritis and avoids further immunosuppressive maintenance therapy. Nephrol Dial Transplant. 2011;26(12):3987–92. https://doi.org/10.1093/ndt/gfr109 .
doi: 10.1093/ndt/gfr109
pubmed: 21385860
Hart PA, Topazian MD, Witzig TE, et al. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience. Gut. 2013;62:1607–15.
doi: 10.1136/gutjnl-2012-302886
Khosroshahi A, Carruthers MN, Deshpande V, et al. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients with IgG4-related disease. Medicine (Baltimore). 2012;91:57–66.
doi: 10.1097/MD.0b013e3182431ef6
Soh SB, Pham A, O’Hehir RE, et al. Novel use of rituximab in a case of Riedel’s thyroiditis refractory to glucocorticoids and tamoxifen. J Clin Endocrinol Metab. 2013;98:3543–9.
doi: 10.1210/jc.2012-4050