For US Healthcare Professionals

IgG4-RD flares may occur in the originally diagnosed organ and/or in other systems4-6

Within 3 years of any flare4-6

  • Up to 90% of patients with IgG4-RD will experience flare recurrence in any organ
  • 1 in 3 patients will experience flare recurrence in the same organ

Due to the often asymptomatic, silently progressive nature of IgG4-RD, patients may experience permanent structural damage and functional organ impairment. Awareness of how structural damage and organ impairment manifests is critically important to inform the timely diagnosis of IgG4-RD in your patients.1-3

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Predicting the progression of IgG4-RD is an evolving science4,7


  • Several indicators can help predict the potential for severe disease, including visceral organ or multi-organ involvement, organ dysfunction, and a susceptibility for flare over a short period of time8,9
  • While some biomarkers may correlate with IgG4-RD activity, there is no singular biomarker that reliably detects or predicts disease activity in all patients4,7
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There are 2 primary goals when addressing IgG4-RD9


  • Reduce short-term inflammation
  • Maintain remission over the long term
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There are challenges in managing both acute symptoms and disease progression in IgG4-RD9,10


  • Corticosteroids are a common first-line treatment for IgG4-RD, but long-term use raises tolerability concerns9,11
  • Second-line treatment options after IgG4-RD relapse are limited to therapies that lack FDA approval.9,10
  • 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. Lanzillotta M, Mancuso G, Della-Torre E. BMJ. 2020;369:m1067. doi:10.1136/bmj.m1067 5. Lee HW, Moon S-H, Kim M-H, et al. J Gastroenterol. 2018;53:967-977. doi:10.1007/s00535-018-1434-6 6. Kalaitzakis E, Webster GJM. Aliment Pharmacol Ther. 2011;33(3):291-303. doi:10.1111/j.1365-2036.2010.04526.x 7. Abraham M, Khoshroshahi A. Expert Rev Clin Immunol. 2017;13(9):867-875. doi:10.1080/1744666X.2017.1354698 8. Umehara H, Okazaki K, Kawa S, et al. Mod Rheumatol. 2021;31(3):529-533. doi:10.1080/14397595.2020.1859710 9. Khosroshahi A, Wallace ZS, Crowe JL, et al. Arthritis Rheumatol. 2015;67(7):1688-1699. doi:10.1002/art.39132 10. Tanaka Y, Stone JH. Mod Rheumatol. 2023;33(2):229-236. doi:10.1093/mr/roac141 11. Strehl C, Ehlers L, Gaber T, et al. Front Immunol. 2019;10:1744. doi:10.3389/fimmu.2019.01744