Treatment and disease management

In this chapter we cover how to calm IgG4-RD quickly and keep it quiet over time. You’ll see why treatment begins most often with glucocorticoids (steroids) in the interest of achieving disease control quickly. This is then followed by a careful steroid taper and may pivot to other therapies such as a B cell-directed therapy or a “disease-modifying anti-rheumatic drug” (DMARD). Such a pivot is designed to maintain disease control while decreasing steroid exposure. Because IgG4-RD can relapse, we’ll also cover healthy routines, clear follow-up plans, and supportive steps to protect organs over the long term.

In this video, Dr. John Stone, Dr. Arezou Khosroshahi, and Dr. Guy Katz explain how doctors think about treating IgG4-RD today, and why the field is moving toward less long-term steroid use and more targeted immune therapy.

Treatment and disease management

The full care pathway for IgG4-related disease (IgG4-RD) includes not only medication, but also a care plan over time that include lifetime disease management. Monitoring is a key aspect of management over the long-term.

IgG4-RD can cause inflammation and swelling that may improve quickly with steroids, but it can also leave behind fibrosis (damaged, scarred tissue) that takes longer to change and in some cases may not improve significantly. That’s why treatment is often a mix of fast control now and smart prevention later.

Induction and maintenance

Treatment is often thought of in two phases:

• Induction means getting inflammation under control quickly to protect organs. For many people, this starts with glucocorticoids (steroids), followed by a gradual taper to reduce side effects. After a short period at a higher dose, the plan usually shifts to a careful taper, lowering the dose step-by-step to reduce side effects.

• Maintenance means keeping the disease quiet over time and lowering the risk of flares, also called relapses. Because IgG4-RD can relapse, many people need ongoing follow-up and sometimes ongoing medication support, especially if a vital organ is involved or if the disease has flared before.

Ongoing treatment

Most people with IgG4-RD do need treatment, especially when organs are involved, because disease activity can sometimes be “silent” while damage builds. In some milder situations (for example, certain stable head-and-neck gland involvement), careful monitoring (“watchful waiting”) instead of immediate treatment may be reasonable, but only with regular check-ins and a clear plan.1 Watchful waiting means watching, not denying, forgetting, or ignoring!

This chapter introduces the main categories of treatment: steroids, B-cell depletion therapies, B-cell inhibitors, DMARDs, stents and other organ-protecting procedures, and lifestyle and supportive care.

Monitoring and follow-up care

Monitoring helps doctors understand whether the plan is working. That usually includes how you feel, physical examinations, lab tests, and sometimes imaging. A key goal is to tell the difference between active inflammation, which may improve with treatment, and longer-term damage or fibrosis, which may not fully reverse.

What you’ll learn in this chapter

Lesson 1: Overview of disease management
Treatment for IgG4-RD includes calming inflammation, protecting organs, watching for relapse, and adjusting care over time.

Lesson 2: Steroids
First-line therapy and side effects—what steroids do, how we taper, and key cautions to keep you safe.

Lesson 3: B cell depletion
Rituximab and other B-cell–targeted therapy—how it works and its impact on controlling disease.

Lesson 4: B cell inhibition
Newer, experimental therapies that modulate B cells—an introduction to evolving treatments and where they fit.

Lesson 5: DMARDs
Additional immunosuppressive medicines that broaden options and help limit steroid exposure—showing the treatment breadth.

Lesson 6: Stents
Supportive procedures for narrowed bile ducts or ureters—why temporary stents are used and how they protect organs.

Lesson 7: Lifestyle
How diet, exercise, stress care, and wellness habits support treatment and empower self-management.

Quiz: Test your knowledge
A short quiz to check understanding of how IgG4‑RD is treated and disease relapse is managed

Let’s get started.

References

1. IgG4ward! Foundation — Reader’s Guide (PDF)
https://igg4ward.org/wp-content/uploads/2025/06/Readers-Guide_FiresideChat_Treatment.pdf

2. Stone JH, Zen Y, Deshpande V. IgG4-Related Disease. NEJM 2012 (article page) https://www.nejm.org/doi/abs/10.1056/NEJMra1104650

(Alternate PubMed record) https://pubmed.ncbi.nlm.nih.gov/22316447/

3. Stone JH, et al. Inebilizumab for Treatment of IgG4-Related Disease. NEJM (MITIGATE) https://www.nejm.org/doi/abs/10.1056/NEJMoa2409712

(Alternate PubMed record) https://pubmed.ncbi.nlm.nih.gov/39541094/

4. Amgen press release — FDA approval for IgG4-RD indication (Apr 3, 2025) https://www.amgen.com/newsroom/press-releases/2025/04/uplizna-inebilizumabcdon-is-now-the-first-and-only-fdaapproved-treatment-for-igg4related-disease

5. Reuters — FDA approves expanded use of Amgen’s Uplizna for IgG4-RD (Apr 3, 2025) https://www.reuters.com/business/healthcare-pharmaceuticals/us-fda-approves-expanded-use-amgens-rare-disease-drug-2025-04-03/