Key takeaways

  • IgG4-related disease (IgG4-RD) is a chronic inflammatory condition. To treat it, doctors often prescribe corticosteroids to reduce inflammation and lower IgG4 levels. But long-term corticosteroid use may lead to side effects.
  • An FDA-approved monoclonal antibody called Uplizna can treat IgG4-RD by depleting B cells. This medication can help reduce disease flares and may decrease the need for corticosteroid therapy.
  • Other possible treatments for IgG4-RD include disease-modifying antirheumatic drugs, which reduce inflammation, and surgery to remove damaged tissues.

Immunoglobulin G4-related disease (IgG4-RD) is a chronic condition that causes inflammation and immune cell buildup in one or more organs in your body. It may cause swelling, tumor-like growths, tissue scarring, and other organ damage that can lead to potentially serious complications.

Treatment is important to limit organ damage and help prevent possible complications.

Treatment for IgG4-RD may involve one or more of the following methods:

  • corticosteroid therapy
  • monoclonal antibody therapy
  • other immune-modifying medications
  • surgery or other therapies to manage complications

Read on to learn more about treatment options for IgG4-RD.

Corticosteroid therapy has historically been the main treatment for IgG4-RD. Corticosteroids are medications that suppress your immune system and help decrease inflammation and lower your IgG4 levels.

Corticosteroid therapy usually takes 2 to 4 weeks to begin reducing IgG4-RD activity. But long-term treatment may be necessary to keep disease activity low.

Long-term corticosteroid use increases your risk of side effects such as:

  • weight gain
  • rounding of your face
  • easy bruising
  • muscle weakness
  • loss of bone mass
  • stomach inflammation or ulcer
  • changes in sleep, energy, or mood
  • changes in skin color or texture
  • changes in hair growth
  • increased blood sugar
  • increased risk of infection

Your doctor can help you weigh the potential benefits and risks of corticosteroid therapy.

If your doctor recommends corticosteroids, they may prescribe a higher dose at first and gradually reduce it over time. Gradually reducing the dose lowers your risk of side effects.

Your doctor might combine corticosteroid therapy with other medications to help bring the disease into remission and prevent disease flares. You might experience partial remission (reduced disease activity) or complete remission (no signs of disease activity).

Let your doctor know if you think you’re experiencing side effects from corticosteroid therapy. They may adjust your dose or recommend other strategies to manage the side effects.

It’s important to talk with your doctor before you stop taking corticosteroids. Stopping too quickly may cause serious side effects, especially after long-term corticosteroid use.

Monoclonal antibodies are proteins that scientists develop in a lab to help manage certain health conditions, including inflammatory conditions such as IgG4-RD.

In April 2025, the Food and Drug Administration (FDA) approved inebilizumab-cdon (Uplizna) as the first monoclonal antibody therapy for IgG4-RD.

Uplizna depletes B cells, which are immune cells that produce IgG4 and play a role in inflammation.

A phase 3 clinical trial compared Uplizna treatment to placebo treatment for IgG4-RD.

Participants who took Uplizna had fewer IgG4-RD flares and were more likely to experience flare-free complete remission after a year of treatment. The participants were allowed to take corticosteroids to treat disease flares, and those who received Uplizna had less of a need for corticosteroid therapy.

The rate of adverse events (negative side effects) was roughly the same in participants who took Uplizna and those who took a placebo. Among those who took Uplizna, the most common side effects were urinary tract infection and lymphopenia (a low white blood cell count).

Scientists are also studying other monoclonal antibodies that might be useful for treating IgG4-RD, including:

  • obexelimab
  • efgartigimod (Vyvgart)
  • rilzabrutinib (Wayrilz)
  • rituximab (Rituxan)
  • zanubrutinib (Brukinsa)

More research is needed to understand the potential benefits and risks of these medications for treating IgG4-RD.

It’s possible that the FDA might approve some of these treatments for IgG4-RD in the future. You can talk with your doctor to learn about the latest treatment options.

In some cases, your doctor may encourage you to take part in a clinical trial in which you might receive a treatment that scientists are still studying. They can help you understand the possible benefits and risks of participating in a trial.

Doctors sometimes prescribe conventional DMARDs to help treat IgG4-RD. These medications suppress your immune system to reduce inflammation.

Examples of conventional DMARDs include:

  • azathioprine
  • cyclophosphamide
  • iguratimod
  • leflunomide
  • methotrexate
  • mycophenolate mofetil

Combining DMARDs with corticosteroid therapy can help bring IgG4-RD into remission and may allow you to take a lower dose of corticosteroids.

However, conventional DMARDs can increase your risk of infection and may cause other side effects.

Your doctor can help you learn about the possible benefits and risks of a specific DMARD.

IgG4-RD can cause organ damage and lead to potentially serious complications. Early treatment is important for preventing and managing complications.

To treat certain complications, you may need medication, surgery, or other therapies.

For example, IgG4-RD may cause swelling, fibrous scar tissue, or a tumor-like mass that puts pressure on organs such as your kidneys, bile ducts, or urethra.

Surgery may sometimes be necessary to remove damaged tissues or relieve pressure on affected organs. Your doctor may need to place a tube called a stent in an affected organ to allow urine, bile, or other fluids to flow properly.

You can talk with your doctor to learn about your specific condition and treatment needs. They can help you learn how to prevent and manage the possible complications.

Corticosteroids are the primary treatment doctors typically recommend to bring IgG4-RD into remission. These medications can help reduce inflammation and lower your IgG4 levels, but long-term corticosteroid therapy increases your risk of treatment side effects.

Your doctor might also prescribe Uplizna, a monoclonal antibody that helps reduce disease flares and the need for corticosteroid therapy in IgG4-RD.

Conventional DMARDs can help prevent disease flares and may allow you to take a lower dose of corticosteroids.

If you develop certain complications of IgG4-RD, your doctor might prescribe additional treatments, such as surgery.

Scientists are continuing to study and develop new treatments to help manage IgG4-RD.

You can talk with your doctor to learn more about your condition, treatment options, and outlook.