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IgG4-RD is a systemic, chronic, and immune-mediated disease with a heterogeneous clinical presentation that is often characterized by
promo-1

Unpredictable flares

promo-two

Inflammation

promo-three

Fibrosis that may progress over time and permanently damage multiple organs

Increased awareness of IgG4-RD has led to a greater understanding of its incidence7

A portrait of the patient with IgG4-RD is emerging7,8

  • The current incidence of IgG4-RD is 1 to 5 people per 100,0009
  • While anyone can develop IgG4-RD, the typical age of onset of IgG4-RD is from 50 to 70 years old7,8
  • While IgG4-RD affects both males and females, it tends to have a male predominance, increasing with age starting at 40 years7,8
  • IgG4-RD presentation can look different in men and women. Women experience more head and neck involvement than other organs; men are more likely to experience organ involvement outside of the head and neck7,8

B cells such as plasmablasts and plasma cells are believed to be drivers of IgG4-RD activity2,10-12
  • Patients with IgG4-RD have elevated counts of plasmablasts and plasma cells which are believed to drive inflammation and fibrosis that may lead to permanent organ damage2,10-12

IgG4-RD, immunoglobulin G4-related disease.

EXPLORE THE PATIENT IMPACT
  • References:

    1. Stone JH, Zen Y, Deshpande V. N Engl J Med. 2012;366:539-551. doi:10.1056/NEJMra1104650 2. Perugino CA, Stone JH. Nature. 2020;16:702-714. doi:10.1038/s41584-020-0500-7 3. Wallace ZS, Deshpande V, Mattoo H, et al. Arthritis Rheumatol. 2015;67(9):2466-2475. doi:10.1002/art.39205 4. Lee HW, Moon S-H, Kim M-H, et al. J Gastroenterol. 2018;53:967-977. doi: 10.1007/s00535-018-1434-6 5. Kalaitzakis E, Webster GJM. Aliment Pharmacol Ther. 2011;33:291-303. 6. Yamada K, Yamamoto M, Saeki T, et al. Arthritis Research & Therapy. 2017;19:262. doi:10.1186/s13075-017-1467-x 7. Floreani A, Okazaki K, Uchida K, et al. J Transl Autoimmun. 2020;19(4):100074. doi:10.1016/j.jtauto.2020.100074 8. Lanzillotta M, Mancuso G, Della-Torre E. BMJ. 2020;369:m1067. doi:10.1136/bmj.m1067 9. Wallace ZS, Miles G, Smolkina E, et al. Ann Rheum Dis. 2023;82:957-962. 10. Lin W, Zhang P, Chen H, et al. Arthritis Res Ther. 2017;19(1):25. doi:10.1186/s13075-017-1231-2 11. Della-Torre E, Rigamonti E, Perugino C, et al. J Allergy Clin Immunol. 2020;145(3):968-981.e14. doi:10.1016/j.jaci.2019.07.004 12. Lanzillotta M, Stone JH, Della-Torre E. Mod Rheumatol. 2023;33(2):258-265. doi:10.1093/mr/roac098