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Key medical literature

The published sources behind this site. Where possible we link to a version you can read in full; otherwise to the publication record. This is information and does not replace your specialist.

1.Della-Torre E, Talarico R, Ballarin J, et al. Identification of red flags for IgG4-related disease: an international European Reference Network for Rare Connective Tissue Diseases framework. Lancet Rheumatol 2025;7(1):e64-e71
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2.Wallace ZS, Naden RP, Chari S, et al. The 2019 ACR/EULAR classification criteria for IgG4-related disease. Arthritis Rheumatol 2020;72(1):7-19
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3.Wallace ZS, Katz G, Hernandez-Barco YG, Baker MC. Current and future advances in practice: IgG4-related disease. Rheumatol Adv Pract 2024;8(2):rkae020
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4.Iaccarino L, Talarico R, Scire CA, et al. IgG4-related diseases: state of the art on clinical practice guidelines. RMD Open 2019;4(Suppl 1):e000787
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5.Sina S, Bonisoli GL, et al. Clinicopathological pearls and diagnostic pitfalls in IgG4-related disease. Diagnostics 2025;15(18):2299
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6.Maritati F, Peyronel F, Vaglio A. IgG4-related disease: a clinical perspective. Rheumatology 2020;59(Suppl 3):iii123-iii131
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7.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):1688-1699
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8.Stone JH, et al. Inebilizumab for treatment of IgG4-related disease. New England Journal of Medicine, 2025.
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9.Cagnotto G, Backlund RT, Bengtsson A, Bruschettini M. Immunosuppressants for IgG4-related disease (protocol). Cochrane Database Syst Rev 2025;(8):CD016094
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10.Cuéllar MC, et al. Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos. Rev Med Chile 2022;150:705-710.
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11.Lanzillotta M, Campochiaro C, Mancuso G, et al. Clinical phenotypes of IgG4-related disease reflect different prognostic outcomes. Rheumatology (Oxford) 2020;59:2435-2442.
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12.Okazaki K (ed.). IgG4-Related Disease. Current Topics in Microbiology and Immunology, vol. 401. Springer International Publishing, 2017.
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13.Orozco Gálvez O. Enfermedad relacionada con la IgG4: creación de un algoritmo diagnóstico y terapéutico europeo y nuevas técnicas de medicina nuclear. Doctoral thesis, Universitat Autònoma de Barcelona, 2024.
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14.Bateman AC, Culver EL. Challenges and pitfalls in the diagnosis of IgG4-related disease. Semin Diagn Pathol 2024;41(2):45-53
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15.Reisch M, Spindelböck WJ, Hodl I, Pietsch D, Rainer F, Kickinger D, Sutic A, Thiel J, Stradner M. Azathioprine as maintenance therapy for IgG4-related diseases. Rheumatology International 2026;46(3):47.
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16.Hammitzsch A, Ferraro N, Bachmann Q, Heemann U, Moog P. Long-term treatment patterns and outcomes in IgG4-related disease: a retrospective single-center cohort study focusing on rituximab. Rheumatology International 2026;46:33.
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17.Taranto S, Bernardo L, Mauro A, Perrone A, Tamborino A, Giani T. IgG4-Related Disease in Childhood: Clinical Presentation, Management, and Diagnostic Challenges. Children (Basel) 2025;12(7):888.
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18.González-García A, Starita-Fajardo G, Lucena López D, Iranzo Alcolea MP, et al. New Developments in the Treatment of IgG4-Related Disease: A Comprehensive Clinical Approach. J Clin Med 2025;14(19):6774 (literature searched through July 2025).
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19.Peyronel F, Della-Torre E, Maritati F, Urban ML, Bajema I, Schleinitz N, Vaglio A. IgG4-related disease and other fibro-inflammatory conditions. Nat Rev Rheumatol 2025;21(5):275-290.
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20.Stone JH. IgG4-related disease: lessons from the first 20 years. Rheumatology (Oxford) 2025;64(Suppl 1):i24-i27.
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21.Arias-Intriago M, Gomolin T, Jaramillo F, et al. IgG4-Related Disease: Current and Future Insights into Pathological Diagnosis. Int J Mol Sci 2025;26(11):5325.
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22.Khurram SA, Gueiros LAM, Soares CD, Fonseca FP. IgG4-Related Disease. In: Lymphoproliferative and Granulomatous Disorders of the Oral Cavity. Springer, 2025:207-213.
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23.Hao Q, Lian D, Zhou M, et al. Identification of candidate biomarkers for diagnosis, disease activity, and relapse prediction in IgG4-related disease. Arthritis Res Ther 2026;28(1):96
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24.Della-Torre E, Baker MC, Zhang W, et al; INDIGO Trial Investigators. Obexelimab for the Treatment of IgG4-Related Disease. N Engl J Med, published online 2 June 2026.
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25.Stone JH, Carruthers M, Baker MC, et al. A phase 2 trial of rilzabrutinib in IgG4-related disease. Annals of the Rheumatic Diseases, 2026.
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26.Duru N, van der Goes MC, Jacobs JWG, et al. EULAR evidence-based and consensus-based recommendations on the management of medium to high-dose glucocorticoid therapy in rheumatic diseases. Ann Rheum Dis 2013;72(12):1905-1913.
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27.Beuschlein F, Else T, Bancos I, et al. European Society of Endocrinology and Endocrine Society Joint Clinical Guideline: Diagnosis and Therapy of Glucocorticoid-induced Adrenal Insufficiency. J Clin Endocrinol Metab 2024;109(7):1657-1683
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28.Fong AC, Cheung NW. The high incidence of steroid-induced hyperglycaemia in hospital. Diabetes Res Clin Pract 2013;99(3):277-280
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29.Nuthalapati M, Menon AR, Rajendran R, et al. IgG4-Related Disease in Urological Practice: A Case Series of Mistaken Malignancies. Indian J Surg Oncol 2025;16(6):1446-1451.
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30.Tomkins M, Martin-Grace J, Kennedy C, et al. Adrenal insufficiency is common amongst kidney transplant recipients receiving maintenance prednisolone and can be predicted using morning cortisol. Nephrol Dial Transplant 2023;38(1):236-245
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31.Mahajan A, Tinianow A, Katz G. IgG4-Related disease: From diagnosis to remission. Best Practice & Research Clinical Rheumatology 2025;39:102108.
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32.Peng L, Nie Y, Zhou J, et al. Withdrawal of immunosuppressants and low-dose steroids in patients with stable IgG4-RD (WInS IgG4-RD): an investigator-initiated, multicentre, open-label, randomised controlled trial. Ann Rheum Dis 2024;83(5):651-660
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33.2019 update of EULAR recommendations for vaccination in adult patients with autoimmune inflammatory rheumatic diseases, European Alliance of Associations for Rheumatology
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34.Choosing Wisely Canada, Allergy and Clinical Immunology recommendations, Canadian Society of Allergy and Clinical Immunology, updated May 2025
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35.Yang F, Wang T, Liu Y. Risk factors for relapse of IgG4-related disease: a systematic review and meta-analysis. Clin Rheumatol 2025;44:4137-4147
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36.Angarola E, Pasqua A, Castro HM, Cáceres A, Spina JC, Casciato P. Clinical characteristics, disease course, and chronic organ damage in a real-world cohort of IgG4-related disease: insights from an Interdisciplinary Unit. Medicina Clínica 2026;166:107317
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37.Peyronel F, Palmisano A, Maritati F, et al. Methotrexate and low-dose prednisone in idiopathic retroperitoneal fibrosis: a randomised clinical trial. J Autoimmun 2025;157:103487
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38.Koster MJ, Ghaffar U, Duong SQ, et al. Incidence, prevalence and mortality of chronic periaortitis: a population-based study. Clin Exp Rheumatol 2022;40:751-757
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39.Dattani R, Barwick TD, El Wardany G, et al; RaDaR Rare Disease Group. An international patient-centred study of retroperitoneal fibrosis. QJM 2022;115(3):148-154
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40.Tang H, Yang H, Zhang P, et al. Malignancy and IgG4-related disease: the incidence, related factors and prognosis from a prospective cohort study in China. Sci Rep 2020;10:4910
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41.So H, Choi SJ, Park S, et al. Increased cancer risk in patients with IgG4-related disease in a nationwide South Korean cohort, 2012-2021. Sci Rep 2025;15:37273
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42.American College of Rheumatology. IgG4-Related Disease (IgG4-RD). Patient information page, updated February 2025.
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43.Gwozdz-Broczkowska A. Fatigue beyond inflammation in inflammatory rheumatic diseases: a narrative review and treatable-traits framework. Rheumatol Int 2026;46(8):232.
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44.US Food and Drug Administration. RAYOS (prednisone) delayed-release tablets, prescribing information, revision 12/2019.
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45.Liu Q, Zheng Y, Sturmlechner I, et al. IKZF1 and UBR4 gene variants drive autoimmunity and Th2 polarization in IgG4-related disease. J Clin Invest 2024;134(16):e178692.
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46.FAI2R. Maladie associee aux IgG4: les reponses a vos questions.
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47.Russell MD, Dey M, Flint J, et al. British Society for Rheumatology guideline on prescribing drugs in pregnancy and breastfeeding: immunomodulatory anti-rheumatic drugs and corticosteroids. Rheumatology (Oxford) 2023;62(4):e48-e88.
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48.European Medicines Agency. MabThera (rituximab), Summary of Product Characteristics.
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49.European Medicines Agency. Nordimet (methotrexate), Summary of Product Characteristics.
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50.US Food and Drug Administration. IMURAN (azathioprine) 50-mg scored tablets, product information, revision 12/2018.
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51.Yu K, Gao H, Liu S, et al. Perception of recurrence risk in patients with IgG4-related disease: a descriptive phenomenological study. Rheumatol Adv Pract 2025;9(1):rkae148.
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52.Newman JH, Shaver A, Sheehan JH, et al; Undiagnosed Disease Network. IgG4-related disease: Association with a rare gene variant expressed in cytotoxic T cells. Mol Genet Genomic Med 2019;7(6):e686.
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53.Karamya ZA, Kovacs A, Illes D, Czako B, Fazekas A, Farkas N, Hegyi P, Czako L. Prevalence of autoimmune pancreatitis in pancreatic resection for suspected malignancy: a systematic review and meta-analysis. BMC Gastroenterol 2024;24:278.
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54.Dures E, Farisogullari B, Santos EJF, et al. 2023 EULAR recommendations for the management of fatigue in people with inflammatory rheumatic and musculoskeletal diseases. Ann Rheum Dis 2024;83(10):1260-1267.
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55.Motor J, Gajewska A, Cienkowski K, et al. IgG4-related disease: focus on digestive system involvement. Front Immunol 2025;16:1584107.
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56.Orphanet. Enfermedad sistemica relacionada con IgG4 (ORPHA:596448), Spanish version.
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57.Orphanet. IgG4-gerelateerde systemische ziekte (ORPHA:596448), Dutch version.
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58.Orphanet. Maladie systemique liee a la presence d'anticorps IgG4 (ORPHA:596448), French version.
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59.Orphanet. IgG4-related systemic disease (ORPHA:596448), German version.
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60.European Medicines Agency. Uplizna (inebilizumab), European public assessment report.
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61.Erasmus MC. IgG4-gerelateerde ziekte, patient information.
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62.Carruthers MN, Topazian MD, Khosroshahi A, Witzig TE, Wallace ZS, Hart PA, Deshpande V, Smyrk TC, Chari S, Stone JH. Rituximab for IgG4-related disease: a prospective, open-label trial. Ann Rheum Dis 2015;74(6):1171-1177.
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63.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(9):e0183844.
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64.ClinicalTrials.gov registry record NCT04520451. An Open Label, Two-Arm Study to Evaluate the Effect of Rilzabrutinib (PRN1008/SAR444671) on Safety and Disease Activity in Participants With IgG4-Related Disease. Phase 2, completed. Sponsor: Principia Biopharma, a Sanofi Company.
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65.ClinicalTrials.gov registry record NCT07190196. A Randomized, Phase 3, Double-blind, 52-week Study to Evaluate the Efficacy and Safety of Rilzabrutinib (SAR444671) Compared to Placebo in Adult Participants With Active IgG4-related Disease (RILIEF). Recruiting. Sponsor: Sanofi.
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66.Stapel SO, Asero R, Ballmer-Weber BK, Knol EF, Strobel S, Vieths S, Kleine-Tebbe J; EAACI Task Force. Testing for IgG4 against foods is not recommended as a diagnostic tool: EAACI Task Force Report. Allergy 2008;63(7):793-796.
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67.Orphanet. IgG4-related systemic disease (ORPHA:596448).
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68.Agencia Espanola de Medicamentos y Productos Sanitarios (AEMPS). Ficha tecnica prednisona, CIMA.
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69.Protocole National de Diagnostic et de Soins (PNDS), Maladie associee aux IgG4. Haute Autorite de Sante, with FAI2R and CERAINOM-IgG4, November 2025.
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70.Het Oogziekenhuis Rotterdam. IgG4-gerelateerde orbitale ziekte, patient information.
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71.Imperial College Healthcare NHS Trust. RPF and IgG4-RD (retroperitoneal fibrosis and immunoglobulin-4 related disease), specialist nephrology service.
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National clinical guidance

The links below point outside this site, to help you look further on your own. We do not control or endorse them, and we gain nothing from listing them. Unlike our own sources above, these are not checked by us for origin or accuracy. Always confirm anything important with your specialist.

1.Protocole National de Diagnostic et de Soins (PNDS), Maladie associee aux IgG4, Haute Autorite de Sante, FAI2R and CERAINOM-IgG4, November 2025.
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Product information (regulatory)

1.Ficha técnica prednisona, Agencia Española de Medicamentos y Productos Sanitarios (AEMPS), CIMA
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Patient information from specialist centres

1.Het Oogziekenhuis Rotterdam, IgG4-Gerelateerde Orbitale Ziekte (IgG4-related orbital disease), patient information. Recognised Dutch expertise centre for rare orbital conditions.
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2.Imperial College Healthcare NHS Trust, RPF and IgG4-RD service: specialist multidisciplinary service for retroperitoneal fibrosis and IgG4-related disease.
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Useful websites

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From the same authors: Balanced Divinity