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Treatment and disease management
Understand how inflammation is managed through steroids, immune therapies, and supportive care—and what to expect at each step.
DMARDs
When steroids or B-cell therapy aren’t enough, DMARDs can help control inflammation and support daily life.
DMARDs: When are existing medicines the right choice?
Sometimes the best course of action in treating IgG4-related disease (IgG4-RD) is not to find a new drug, but to use well-established therapies. Many people with IgG4-RD do well with medicines that rheumatologists have used for years to calm the immune system. These are called DMARDs (disease-modifying anti-rheumatic drugs).
In IgG4-RD, DMARDs are sometimes used as steroid-sparing or remission-maintenance medicines. The full benefit of DMARDs above and beyond steroids is not entirely clear, because few rigorous studies have been conducted with these medications in IgG4-RD (and other diseases). Evidence on just how well the DMARDs work in IgG4-RD is limited because these drugs have been tested specifically in studies of remission induction. A limited bit of information about them exists with regard to remission maintenance. [1–2]
In this lesson, we’ll explain what DMARDs are, why a clinician might suggest one, and how to use them safely alongside other treatments.
What you’ll learn
What DMARDs are and how they differ from steroids and B-cell therapies
The most commonly used DMARDs in IgG4-RD (off-label) and where they fit
Practical pros and cons, including safety labs and red-flag symptoms
How DMARDs can support a long-term plan focused on remission and organ protection
What are DMARDs?
DMARDs are medicines that lower the activity of over-active immune cells. In IgG4-RD, clinicians sometimes use certain DMARDs off-label to:
Help taper steroids more quickly and safely, and
Maintain remission once inflammation is quiet.
DMARDs are different from steroids (which work fast but can cause side effects with long-term use) and different from B-cell therapies (inebilizumab), which directly target B cells. DMARDs act more broadly and usually more slowly. 1
The short list: DMARDs you may hear about
Doses shown are typical rheumatology starting ranges. Your team will individualize based on age, organ function, other medicines, and goals. (Off-label use in IgG4-RD.)
DMARD | How it works | Typical adult dose range* | Pros | Cons & safety checks |
|---|---|---|---|---|
Azathioprine | Calms new immune cell growth | 1–2 mg/kg/day by mouth4 | Widely used; once-daily | Check TPMT/NUDT15 when available; monitor CBC, liver tests; watch for nausea, infections 1,2 |
Mycophenolate mofetil | Blocks lymphocyte building blocks | 500–1,000 mg twice daily 5,6 | Often well-tolerated; adjustable | CBC, liver tests; GI upset common; avoid in pregnancy 1–3 |
Methotrexate | Slows immune cell division | 10–20 mg once weekly + folic acid 7 | Weekly dosing; inexpensive | CBC, liver tests, avoid alcohol excess; not for pregnancy; can cause fatigue, mouth sores 1,2 |
Tacrolimus (selected cases) | T-cell signal blocker | Individualized, often 1–2 mg twice daily with level checks8 | Useful in certain organ patterns | Requires drug-level monitoring; kidney effects; drug interactions1,2 |
Cyclophosphamide (rare, severe cases) | Strong immune suppression | Pulse IV or oral (specialist use)9-10 | For life-threatening organ disease | Significant risks; specialist and strict monitoring required1,2 |
*These are off-label for IgG4-RD. Always follow your clinician’s plan.
Where do DMARDs fit next to B cell therapies?
Consider B cell therapy (such as inebilizumab) when the disease is multi-organ, relapsing, or steroid-dependent. 3
Add a DMARD when:
steroids are hard to taper,
B cell therapy isn’t available or isn’t a good fit, or
your team wants a maintenance “bridge” to keep disease quiet.
Safety first: how we monitor
DMARDs usually work gradually (weeks). Plan follow-ups so you and your team can adjust early. 1,2
Talking with your care team
Your goals: symptom relief, organ protection, and steroid minimization.
Your context: other illnesses, pregnancy plans, vaccines, and travel.
Your preferences: pills vs. infusions, lab-draw frequency, and how you feel about risks.
Shared decision-making keeps the plan aligned with what matters most to you.
Summary
DMARDs are a steady, time-tested part of the toolbox for IgG4-RD. They’re not right for everyone, but they can help reduce steroid exposure and maintain remission when used thoughtfully, with regular monitoring and clear goals.
References
1. Stone JH, Zen Y, Deshpande V. IgG4-Related Disease. N Engl J Med. 2012;366:539-551. doi:10.1056/NEJMra1104650. Available at: https://www.nejm.org/doi/full/10.1056/NEJMra1104650
2. Lang D, et al. IgG4-related disease: current challenges and future prospects. Int J Gen Med. 2016;9:231-246. Available at: https://doi.org/10.2147/TCRM.S99985
3. Peyronel F, et al. IgG4-related disease and other fibro-inflammatory conditions. Nat Rev Rheumatol. 2025. Available at: https://doi.org/10.1038/s41584-025-01240-x
4. U.S. Food and Drug Administration. IMURAN (azathioprine) prescribing information. Revised 2018. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2018/016324s039lbl.pdf
5. NHS Dorset Medicines Advisory Group. Mycophenolate Mofetil Shared Care Protocol (Adults, non-transplant indications). Feb 2025. Available at: https://nhsdorset.nhs.uk/medicines/wp-content/uploads/sites/3/2025/05/Mycophenolate-Shared-Care-Protocol.pdf
6. NHS North Yorkshire & York Formulary. Shared Care Guideline: Mycophenolate Mofetil (Adults). March 2021 (v2 Sept 2021). Available at: https://www.northyorkshireandyorkformulary.nhs.uk/docs/files/Mycophenolate%20Shared%20Care%20Guideline%20March%202021%20V2%20Sep%2021%20APC%20alteration%201.pdf
7. U.S. Food and Drug Administration. Methotrexate Tablets prescribing information. Revised 2020. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/040054s015,s016,s017.pdf
8. Dutta S, Bandyopadhyay S, et al. The efficacy and safety of tacrolimus in rheumatoid arthritis. Ther Adv Musculoskelet Dis. 2011;3(2):69–77. Available at: https://pmc.ncbi.nlm.nih.gov/articles/PMC3383498/
9. Hellmich B, et al. EULAR recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2024;83(1):30–56. Available at: https://ard.bmj.com/content/83/1/30
10. Yates M, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. 2016. Available at: https://www.diva-portal.org/smash/get/diva2%3A1038451/FULLTEXT01.pdf