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Review

IgG4-related disease: advances in pathophysiology and treatment

ORCID Icon, ORCID Icon, ORCID Icon, ORCID Icon & ORCID Icon
Pages 537-547 | Received 14 Feb 2023, Accepted 22 Mar 2023, Published online: 06 Apr 2023

References

  • Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539–551.
  • Perugino CA, Stone JH. IgG4-related disease: an update on pathophysiology and implications for clinical care. Nat Rev Rheumatol. 2020;16:702–714.
  • Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25:1181–1192.
  • Bledsoe JR, Della-Torre E, Rovati L, et al. IgG4-related disease: review of the histopathologic features, differential diagnosis, and therapeutic approach. APMIS. 2018;126:459–476.
  • Corradi D, Maestri R, Palmisano A, et al. Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis. Kidney Int. 2007;72:742–753.
  • Carruthers MN, Khosroshahi A, Augustin T, et al. The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis. 2015;74:14–18.
  • Wallace ZS, Deshpande V, Mattoo H, et al. IgG4-related disease: clinical and laboratory features in one hundred twenty-five patients. Arthritis Rheumatol. 2015;67:2466–2475.
  • Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–984.
  • Kamisawa T, Nakajima H, Egawa N, et al. IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology. 2006;6:132–137.
  • Khosroshahi A, Stone JH. A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol. 2011;23:57–66.
  • Wallace ZS, Zhang Y, Perugino CA, et al. Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts. Ann Rheum Dis. 2019;78:406–412.
  • Geyer JT, Ferry JA, Harris NL, et al. Chronic sclerosing sialadenitis (Küttner tumor) is an IgG4-associated disease. Am J Surg Pathol. 2010;34:202–210.
  • Yamamoto M, Takahashi H, Ohara M, et al. A new conceptualization for Mikulicz’s disease as an IgG4-related plasmacytic disease. Mod Rheumatol. 2006;16:335–340.
  • Dahlgren M, Khosroshahi A, Nielsen GP, et al. Riedel’s thyroiditis and multifocal fibrosclerosis are part of the IgG4-related systemic disease spectrum. Arthritis Care Res (Hoboken). 2010;62:1312–1318.
  • Zhang X, Wu G, Wang M, et al. Novel advances in the study of IgG4-related disease in the eye and ocular adnexa. Ophthalmic Res. 2022;65:605–614.
  • Kasashima F, Kawakami K, Matsumoto Y, et al. IgG4-related arterial disease. Ann Vasc Dis. 2018;11:72–77.
  • Khosroshahi A, Carruthers MN, Stone JH, et al. Rethinking Ormond’s disease: “idiopathic” retroperitoneal fibrosis in the era of IgG4-related disease. Medicine (Baltimore). 2013;92:82–91.
  • Vaglio A, Palmisano A, Alberici F, et al. Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial. Lancet. 2011;378:338–346.
  • Maritati F, Rocco R, Accorsi Buttini E, et al. Clinical and prognostic significance of serum IgG4 in chronic periaortitis. an analysis of 113 patients. Front Immunol. 2019;10:693.
  • Saeki T, Nishi S, Imai N, et al. Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis. Kidney Int. 2010;78:1016–1023.
  • Kawano M, Saeki T, Nakashima H, et al. Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol. 2011;15:615–626.
  • Takahashi N, Kawashima A, Fletcher JG, et al. Renal involvement in patients with autoimmune pancreatitis: cT and MR imaging findings. Radiology. 2007;242:791–801.
  • Maritati F, Peyronel F, Vaglio A. IgG4-related disease: a clinical perspective. Rheumatology (Oxford). 2020;59:iii123–31.
  • Ishikawa Y, Terao C. Genetic analysis of IgG4-related disease. Mod Rheumatol. 2020;30:17–23.
  • Watanabe T, Maruyama M, Ito T, et al. Two siblings with type 1 autoimmune pancreatitis. Intern Med. 2013;52:895–899.
  • Kawa S, Ota M, Yoshizawa K, et al. HLA DRB10405-DQB10401 haplotype is associated with autoimmune pancreatitis in the Japanese population. Gastroenterology. 2002;122:1264–1269.
  • Buechter M, Manka P, Heinemann FM, et al. Outcome and genetic factors in IgG4-associated autoimmune pancreatitis and cholangitis: a single center experience. Gastroenterol Res Pract. 2017;2017:6126707.
  • Martorana D, Márquez A, Carmona FD, et al. A large-scale genetic analysis reveals an autoimmune origin of idiopathic retroperitoneal fibrosis. J Allergy Clin Immunol. 2018;142:1662–1665.
  • Umemura T, Ota M, Hamano H, et al. Association of autoimmune pancreatitis with cytotoxic T-lymphocyte antigen 4 gene polymorphisms in Japanese patients. Am J Gastroenterol. 2008;103:588–594.
  • Chang MC, Chang YT, Tien YW, et al. T-cell regulatory gene CTLA-4 polymorphism/haplotype association with autoimmune pancreatitis. Clin Chem. 2007;53:1700–1705.
  • Alberici F, Bonatti F, Maritati F, et al. Association of a polymorphism of the Fcγ-receptor 2A (FCGR2A) gene with chronic periaortitis. Clin Exp Rheumatol. 2018;37:222–226.
  • Umemura T, Ota M, Hamano H, et al. Genetic association of Fc receptor-like 3 polymorphisms with autoimmune pancreatitis in Japanese patients. Gut. 2006;55:1367–1368.
  • Ota M, Ito T, Umemura T, et al. Polymorphism in the KCNA3 gene is associated with susceptibility to autoimmune pancreatitis in the Japanese population. Dis Markers. 2011;31:223–229.
  • Umemura T, Fujinaga Y, Ashihara N, et al. IL1R1 gene variants associate with disease susceptibility to IgG4-related periaortitis/periarteritis in IgG4-related disease. Gene. 2022;820:146212.
  • Chang MC, Jan IS, Liang PC, et al. Human cationic trypsinogen but not serine peptidase inhibitor, Kazal type 1 variants increase the risk of type 1 autoimmune pancreatitis. J Gastroenterol Hepatol. 2014;29:2038–2042.
  • Kino-Ohsaki J, Nishimori I, Morita M, et al. Serum antibodies to carbonic anhydrase I and II in patients with idiopathic chronic pancreatitis and Sjögren’s syndrome. Gastroenterology. 1996;110:1579–1586.
  • Okazaki K, Uchida K, Ohana M, et al. Autoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response. Gastroenterology. 2000;118:573–581.
  • Asada M, Nishio A, Uchida K, et al. Identification of a novel autoantibody against pancreatic secretory trypsin inhibitor in patients with autoimmune pancreatitis. Pancreas. 2006;33:20–26.
  • Frulloni L, Lunardi C, Simone R, et al. Identification of a novel antibody associated with autoimmune pancreatitis. N Engl J Med. 2009;361:2135–2142.
  • Endo T, Takizawa S, Tanaka S, et al. Amylase alpha-2A autoantibodies: novel marker of autoimmune pancreatitis and fulminant type 1 diabetes. Diabetes. 2009;58:732–737.
  • Hubers LM, Vos H, Schuurman AR, et al. Annexin A11 is targeted by IgG4 and IgG1 autoantibodies in IgG4-related disease. Gut. 2018;67:728–735.
  • Shiokawa M, Kodama Y, Sekiguchi K, et al. Laminin 511 is a target antigen in autoimmune pancreatitis. Sci Transl Med. 2018;10(453):eaaq0997.
  • Du H, Shi L, Chen P, et al. Prohibitin is Involved in Patients with IgG4 related disease. PLoS ONE. 2015;10:e0125331.
  • Perugino CA, AlSalem SB, Mattoo H, et al. Identification of galectin-3 as an autoantigen in patients with IgG(4)-related disease. J Allergy Clin Immunol. 2019;143:736–45.e6.
  • Liu H, Perugino CA, Ghebremichael M, et al. Disease severity linked to increase in autoantibody diversity in IgG4-related disease. Arthritis Rheumatol. 2020;72:687–693.
  • Maritati F, Corradi D, Versari A, et al. Rituximab therapy for chronic periaortitis. Ann Rheum Dis. 2012;71:1262–1264.
  • Maillette de Buy Wenniger LJ, Doorenspleet ME, Klarenbeek PL, et al. Immunoglobulin G4+ clones identified by next-generation sequencing dominate the B cell receptor repertoire in immunoglobulin G4 associated cholangitis. Hepatology. 2013;57:2390–2398.
  • Mattoo H, Mahajan VS, Della-Torre E, et al. De Novo oligoclonal expansions of circulating plasmablasts in active and relapsing IgG4-related disease. J Allergy Clin Immunol. 2014;134:679–687.
  • Cho H, Lee JJ, Kim M, et al. Laboratory characteristics of IgG4-related disease: a retrospective study from a single tertiary medical center. Medicine (Baltimore). 2022;101:e30387.
  • Shiokawa M, Kodama Y, Kuriyama K, et al. Pathogenicity of IgG in patients with IgG4-related disease. Gut. 2016;65:1322–1332.
  • Wallace ZS, Mattoo H, Carruthers M, et al. Plasmablasts as a biomarker for IgG4-related disease, independent of serum IgG4 concentrations. Ann Rheum Dis. 2015;74:190–195.
  • Lin W, Zhang P, Chen H, et al. Circulating plasmablasts/plasma cells: a potential biomarker for IgG4-related disease. Arthritis Res Ther. 2017;19:25.
  • Li J, Liu Z, Zhang P, et al. Peripheral B-Cell immunophenotyping identifies heterogeneity in IgG4-related disease. Front Immunol. 2021;12:747076.
  • Lanzillotta M, Della-Torre E, Milani R, et al. Increase of circulating memory B cells after glucocorticoid-induced remission identifies patients at risk of IgG4-related disease relapse. Arthritis Res Ther. 2018;20:222.
  • Della-Torre E, Rigamonti E, Perugino C, et al. B lymphocytes directly contribute to tissue fibrosis in patients with IgG(4)-related disease. J Allergy Clin Immunol. 2020;145:968–81.e14.
  • Maehara T, Mattoo H, Mahajan VS, et al. The expansion in lymphoid organs of IL-4(+) BATF(+) T follicular helper cells is linked to IgG4 class switching in vivo. Life Sci Alliance. 2018;1(1):e201800050.
  • Akiyama M, Suzuki K, Yamaoka K, et al. Number of circulating follicular helper 2 T cells correlates with IgG4 and interleukin-4 levels and plasmablast numbers in IgG4-related disease. Arthritis Rheumatol. 2015;67:2476–2481.
  • Morita R, Schmitt N, Bentebibel SE, et al. Human blood CXCR5(+)CD4(+) T cells are counterparts of T follicular cells and contain specific subsets that differentially support antibody secretion. Immunity. 2011;34:108–121.
  • Jeannin P, Lecoanet S, Delneste Y, et al. IgE versus IgG4 production can be differentially regulated by IL-10. J Immunol. 1998;160:3555–3561.
  • Akiyama M, Suzuki K, Yoshimoto K, et al. Peripheral TIGIT+ T follicular helper cells that produce high levels of interleukin-21 via OX40 represent disease activity in IgG4-related disease. Front Immunol. 2021;12:651357.
  • Mancuso G, Jofra T, Lanzillotta M, et al. Persistence of circulating T-follicular helper cells after rituximab is associated with relapse of IgG4-related disease. Rheumatology (Oxford). 2021;60:3947–3949.
  • Ito F, Kamekura R, Yamamoto M, et al. IL-10(+) T follicular regulatory cells are associated with the pathogenesis of IgG4-related disease. Immunol Lett. 2019;207:56–63.
  • Mattoo H, Mahajan VS, Maehara T, et al. Clonal expansion of CD4(+) cytotoxic T lymphocytes in patients with IgG4-related disease. J Allergy Clin Immunol. 2016;138:825–838.
  • Della-Torre E, Bozzalla-Cassione E, Sciorati C, et al. A CD8α-subset of CD4+SLAMF7+ cytotoxic T cells is expanded in patients with IgG4-related disease and decreases following glucocorticoid treatment. Arthritis Rheumatol. 2018;70:1133–1143.
  • Maehara T, Mattoo H, Ohta M, et al. Lesional CD4+ IFN-γ+ cytotoxic T lymphocytes in IgG4-related dacryoadenitis and sialoadenitis. Ann Rheum Dis. 2017;76:377–385.
  • Perugino CA, Kaneko N, Maehara T, et al. CD4(+) and CD8(+) cytotoxic T lymphocytes may induce mesenchymal cell apoptosis in IgG(4)-related disease. J Allergy Clin Immunol. 2021;147:368–382.
  • Pillai S, Perugino C, Kaneko N. Immune mechanisms of fibrosis and inflammation in IgG4-related disease. Curr Opin Rheumatol. 2020;32:146–151.
  • Nicastro M, Vescovini R, Maritati F, et al. Fibrocytes in chronic periaortitis: a novel mechanism linking inflammation and fibrosis. Arthritis Rheumatol. 2019;71:1913–1922.
  • Vaglio A, Catanoso MG, Spaggiari L, et al. Interleukin-6 as an inflammatory mediator and target of therapy in chronic periaortitis. Arthritis Rheum. 2013;65:2469–2475.
  • Furukawa S, Moriyama M, Tanaka A, et al. Preferential M2 macrophages contribute to fibrosis in IgG4-related dacryoadenitis and sialoadenitis, so-called Mikulicz’s disease. Clin Immunol. 2015;156:9–18.
  • Furukawa S, Moriyama M, Miyake K, et al. Interleukin-33 produced by M2 macrophages and other immune cells contributes to Th2 immune reaction of IgG4-related disease. Sci Rep. 2017;7:42413.
  • Chinju A, Moriyama M, Kakizoe-Ishiguro N, et al. CD163+ M2 macrophages promote fibrosis in IgG4-related disease via toll-like receptor 7/interleukin-1 receptor-associated kinase 4/NF-κB signaling. Arthritis Rheumatol. 2022;74:892–901.
  • Schils M, Betrains A, Vanderschueren S, et al. How specific are elevated IgG4 levels for IgG4-related disease? Eur J Intern Med. 2021;87:115–118.
  • Peuraharju E, Hagström J, Tarkkanen J, et al. IgG4-positive plasma cells in nonspecific sialadenitis and sialolithiasis. Mod Pathol. 2022;35:1423–1430.
  • Trivioli G, Bond M, Emmi G, et al. IgG4-related disease: not just a matter of IgG4. Rheumatology (Oxford). 2021;60:iii35–8.
  • Kawashiri SY, Origuchi T, Umeda M, et al. Association of serum levels of fibrosis-related biomarkers with disease activity in patients with IgG4-related disease. Arthritis Res Ther. 2018;20:277.
  • Aalberse RC, Stapel SO, Schuurman J, et al. Immunoglobulin G4: an odd antibody. Clin Exp Allergy. 2009;39:469–477.
  • Maslinska M, Dmowska-Chalaba J, Jakubaszek M. The role of IgG4 in autoimmunity and rheumatic diseases. Front Immunol. 2021;12:787422.
  • Dekkers G, Bentlage AEH, Stegmann TC, et al. Affinity of human IgG subclasses to mouse Fc gamma receptors. MAbs. 2017;9:767–773.
  • Gon Y, Kandou T, Tsuruyama T, et al. Increased number of T cells and exacerbated inflammatory pathophysiology in a human IgG4 knock-in MRL/lpr mouse model. PLoS ONE. 2023;18:e0279389.
  • Sasaki T, Akiyama M, Kaneko Y, et al. Risk factors of relapse following glucocorticoid tapering in IgG4-related disease. Clin Exp Rheumatol. 2018;36(112):186–189.
  • Wallace ZS, Mattoo H, Mahajan VS, et al. Predictors of disease relapse in IgG4-related disease following rituximab. Rheumatology (Oxford). 2016;55:1000–1008.
  • Zhang P, Liu Z, Li J, et al. The association of different serum IgG4 levels with distinct clinical characteristics and treatment efficacy in patients with IgG4-related disease. Clin Exp Rheumatol. 2021;39:727–735.
  • Urban ML, Maritati F, Palmisano A, et al. Rituximab for chronic periaortitis without evidence of IgG4-related disease: a long-term follow-up study of 20 patients. Ann Rheum Dis. 2020;79:433–434.
  • Nirula A, Glaser SM, Kalled SL, et al. What is IgG4? A review of the biology of a unique immunoglobulin subtype. Curr Opin Rheumatol. 2011;23:119–124.
  • Muraki T, Hamano H, Ochi Y, et al. Autoimmune pancreatitis and complement activation system. Pancreas. 2006;32:16–21.
  • Sugimoto M, Watanabe H, Asano T, et al. Possible participation of IgG4 in the activation of complement in IgG4-related disease with hypocomplementemia. Mod Rheumatol. 2016;26:251–258.
  • Wang R, He D, Zhao L, et al. Role of complement system in patients with biopsy-proven immunoglobulin G4-related kidney disease. Hum Pathol. 2018;81:220–228.
  • Peng L, Lu H, Zhou J, et al. Clinical characteristics and outcome of IgG4-related disease with hypocomplementemia: a prospective cohort study. Arthritis Res Ther. 2021;23:102.
  • Kawa S. The Immunobiology of immunoglobulin G4 and complement activation pathways in IgG4-related disease. Curr Top Microbiol Immunol. 2017;401:61–73.
  • Khosroshahi A, Wallace ZS, Crowe JL, et al. International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol. 2015;67:1688–1699.
  • Akiyama M, Yasuoka H, Yamaoka K, et al. Enhanced IgG4 production by follicular helper 2 T cells and the involvement of follicular helper 1 T cells in the pathogenesis of IgG4-related disease. Arthritis Res Ther. 2016;18:167.
  • Wu Q, Chang J, Chen H, et al. Efficacy between high and medium doses of glucocorticoid therapy in remission induction of IgG4-related diseases: a preliminary randomized controlled trial. Int J Rheum Dis. 2017;20:639–646.
  • Masaki Y, Matsui S, Saeki T, et al. A multicenter phase II prospective clinical trial of glucocorticoid for patients with untreated IgG4-related disease. Mod Rheumatol. 2017;27:849–854.
  • Kubota K, Kamisawa T, Okazaki K, et al. Low-dose maintenance steroid treatment could reduce the relapse rate in patients with type 1 autoimmune pancreatitis: a long-term Japanese multicenter analysis of 510 patients. J Gastroenterol. 2017;52:955–964.
  • Masamune A, Nishimori I, Kikuta K, et al. Randomised controlled trial of long-term maintenance corticosteroid therapy in patients with autoimmune pancreatitis. Gut. 2017;66:487–494.
  • de Pretis N, Amodio A, Bernardoni L, et al. Azathioprine maintenance therapy to prevent relapses in autoimmune pancreatitis. Clin Transl Gastroenterol. 2017;8:e90.
  • Della-Torre E, Campochiaro C, Bozzolo EP, et al. Methotrexate for maintenance of remission in IgG4-related disease. Rheumatology (Oxford). 2015;54:1934–1936.
  • Wang Y, Li K, Gao D, et al. Combination therapy of leflunomide and glucocorticoids for the maintenance of remission in patients with IgG4-related disease: a retrospective study and literature review. Intern Med J. 2017;47:680–689.
  • Luo X, Peng Y, Zhang P, et al. Comparison of the effects of cyclophosphamide and mycophenolate mofetil treatment against immunoglobulin G4-related disease: a retrospective cohort study. Front Med. 2020;7:253.
  • Hart PA, Topazian MD, Witzig TE, et al. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the mayo clinic experience. Gut. 2013;62:1607–1615.
  • Yunyun F, Yu C, Panpan Z, et al. Efficacy of cyclophosphamide treatment for immunoglobulin G4-related disease with addition of glucocorticoids. Sci Rep. 2017;7:6195.
  • Buechter M, Klein CG, Kloeters C, et al. Tacrolimus as a reasonable alternative in a patient with steroid-dependent and thiopurine-refractory autoimmune pancreatitis with IgG4-associated cholangitis. Z Gastroenterol. 2014;52:564–568.
  • Fukui S, Kawaai S, Nakai T, et al. Effectiveness and safety of mizoribine for the treatment of IgG4-related disease: a retrospective cohort study. Rheumatology (Oxford). 2021;60:5697–5704.
  • Alberici F, Palmisano A, Urban ML, et al. Methotrexate plus prednisone in patients with relapsing idiopathic retroperitoneal fibrosis. Ann Rheum Dis. 2013;72:1584–1586.
  • Yunyun F, Yu P, Panpan Z, et al. Efficacy and safety of low dose mycophenolate mofetil treatment for immunoglobulin G4-related disease: a randomized clinical trial. Rheumatology (Oxford). 2019;58:52–60.
  • Carruthers MN, Topazian MD, Khosroshahi A, et al. Rituximab for IgG4-related disease: a prospective, open-label trial. Ann Rheum Dis. 2015;74:1171–1177.
  • Khosroshahi A, Bloch DB, Deshpande V, et al. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum. 2010;62:1755–1762.
  • Khosroshahi A, Carruthers MN, Deshpande V, et al. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore). 2012;91:57–66.
  • 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;12:e0183844.
  • Campochiaro C, Della-Torre E, Lanzillotta M, et al. Long-term efficacy of maintenance therapy with Rituximab for IgG4-related disease. Eur J Intern Med. 2020;74:92–98.
  • Della-Torre E, Feeney E, Deshpande V, et al. B-cell depletion attenuates serological biomarkers of fibrosis and myofibroblast activation in IgG4-related disease. Ann Rheum Dis. 2015;74:2236–2243.
  • Della-Torre E, Lanzillotta M, Campochiaro C, et al. Efficacy and safety of rituximab biosimilar (CT-P10) in IgG4-related disease: an observational prospective open-label cohort study. Eur J Intern Med. 2021;84:63–67.
  • Study to evaluate the effect of XmAb®5871 on disease activity in patients with IgG4-related disease (RD). ClinicalTrials.gov: NCT02725476.
  • A study of inebilizumab efficacy and safety in IgG4- related disease. ClinicalTrials.gov: NCT04540497.
  • Matza MA, Perugino CA, Harvey L, et al. Abatacept in IgG4-related disease: a prospective, open-label, single-arm, single-centre, proof-of-concept study. Lancet Rheumatol. 2022;4:e105–12.
  • Open label two-arm study to evaluate rilzabrutinib in IgG4-related disease patients. ClinicalTrials.gov: NCT04520451.
  • Campbell KS, Cohen AD, Pazina T. Mechanisms of NK cell activation and clinical activity of the therapeutic SLAMF7 antibody, elotuzumab in multiple myeloma. Front Immunol. 2018;9:2551.
  • He B, Santamaria R, Xu W, et al. The transmembrane activator TACI triggers immunoglobulin class switching by activating B cells through the adaptor MyD88. Nat Immunol. 2010;11:836–845.
  • Kiyama K, Kawabata D, Hosono Y, et al. Serum BAFF and APRIL levels in patients with IgG4-related disease and their clinical significance. Arthritis Res Ther. 2012;14:R86.
  • Belimumab treatment for IgG4-related disease. ClinicalTrials.gov: NCT04660565.
  • Nakajima I, Taniguchi Y, Tsuji H, et al. Therapeutic potential of the interleukin-4/interleukin-13 inhibitor dupilumab for treating IgG4-related disease. Rheumatology (Oxford). 2022;61:e151–3.
  • Ebbo M, De Sainte-Marie B, Muller R, et al. Correspondence on: ‘Dupilumab as a novel steroid-sparing treatment for IgG(4)-related disease’ by Simpson et al. Ann Rheum Dis. 2022;81:e26.
  • Zanubrutinib in patients with IgG4-related disease. ClinicalTrials.gov: NCT04602598.
  • Elotuzumab in immunoglobulin G4-related disease (IgG4-RD). ClinicalTrials.gov: NCT 04918147.
  • Yamamoto M, Takahashi H, Takano K, et al. Efficacy of abatacept for IgG4-related disease over 8 months. Ann Rheum Dis. 2016;75:1576–1578.
  • Karim F, Paridaens D, Westenberg LEH, et al. Infliximab for IgG4-related orbital disease. Ophthalmic Plast Reconstr Surg. 2017;33:S162–s5.
  • Jalaj S, Dunbar K, Campbell A, et al. Treatment of pediatric IgG4-related orbital disease with TNF-α inhibitor. Ophthalmic Plast Reconstr Surg. 2018;34:e10–2.
  • Moussiegt A, Müller R, Ebbo M, et al. IgG4-related disease and hypereosinophilic syndrome: overlapping phenotypes. Autoimmun Rev. 2021;20:102889.

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