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Therapeutic Options in IgG4-Related Disease

  • Vasculitis (L Barra, Section Editor)
  • Published:
Current Treatment Options in Rheumatology Aims and scope Submit manuscript

Abstract

Purpose of review

IgG4-related disease (IgG4-RD) is an autoimmune fibrosing condition which is being increasingly recognized. This condition has been classically managed with glucocorticoids (GC) with variable dosing and frequent relapses. Multiple observational reports studied different GC doses and steroid-sparing agents. Rituximab (RTX) was reported as an effective induction treatment, with less relapses after 1 year of treatment. In the absence of guidelines, an international management consensus was made in 2015.

Recent findings

In the last few years, significant advances have been made to clarify IgG4-RD pathophysiology. The new classification criteria will enable investigators to design new clinical trials with more homogeneous populations. Furthermore, B and T lymphocytes have a central role in the inflammatory cascade. More solid evidence has been published supporting treatment with GC, RTX, and some other steroid-sparing agents like mofetil mycophenolate. The combination of GC and immunosuppressants has been pointed by a meta-analysis as the most effective treatment for IgG4-RD, although the number and quality of studies remains limited.

Summary

The use of GC and steroid-sparing agents is effective in IgG4-RD, although the choice between rituximab and other immunosuppressants can be influenced by many factors. Novel agents will be trialed in the following years.

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Authors and Affiliations

  1. General Internal Medicine department, Systemic Autoimmune Diseases unit, Hospital Universitari Vall d’Hebron, Passeig Vall d’Hebron 119-129, 08035, Barcelona, Spain

    Andreu Fernández-Codina MD, MSc, PhD, Olimpia Orozco-Gálvez MD, PhD(c) & Fernando Martínez-Valle

  2. Rheumatology division, University of Western Ontario, London, ON, Canada

    Andreu Fernández-Codina MD, MSc, PhD

  3. General Internal Medicine department-Windsor campus, University of Western Ontario, Windsor, ON, Canada

    Andreu Fernández-Codina MD, MSc, PhD

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  1. Andreu Fernández-Codina MD, MSc, PhD
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  2. Olimpia Orozco-Gálvez MD, PhD(c)
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Correspondence to Andreu Fernández-Codina MD, MSc, PhD.

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Conflict of Interest

Dr. Fernandez Codina reports grants from Scleroderma Society of Ontario, grants from Saint Joseph’s Health Care London Foundation, personal fees from Actelion, grants from Medicina, other from Catalan-Balearic Society of Internal Medicine, outside the submitted work. The other authors declare no conflicts of interest relevant to this manuscript.

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Fernández-Codina, A., Orozco-Gálvez, O. & Martínez-Valle, F. Therapeutic Options in IgG4-Related Disease. Curr Treat Options in Rheum 6, 191–204 (2020). https://doi.org/10.1007/s40674-020-00147-w

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