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PubMed This is a summary of 18 peer-reviewed journal articles Updated
Urology

Standard Treatments and Urological Care for IgG4-RPF

At a Glance

Treating IgG4-related retroperitoneal fibrosis (IgG4-RPF) requires a two-pronged approach: taking immunosuppressants like prednisone or Rituximab to stop the inflammatory attack, and using urological procedures like ureteral stenting to protect the kidneys from physical blockages.

Treating IgG4-related retroperitoneal fibrosis (IgG4-RPF) is a two-pronged approach: the first goal is to stop the immune system from attacking your tissue, and the second is to protect your kidneys from the physical pressure caused by the fibrotic mass.

First-Line Therapy: Glucocorticoids

The standard starting point for almost all patients with active IgG4-RPF is a course of glucocorticoids, most commonly prednisone [1][2].

  • How They Work: Steroids work quickly to “cool down” the active inflammation. They cause the inflammatory cells to leave the area, which can lead to a rapid reduction in the size of the retroperitoneal mass [3][4].
  • The Taper: Your doctor will slowly lower (taper) the dose over several months to manage side effects and prevent relapses [5].
  • Important Side Effects: Because you will likely be on steroids for months, you must prepare for side effects. Common issues include blood sugar spikes (especially if you are diabetic), insomnia, mood changes (irritability or anxiety), increased risk of infection, weight gain, and bone density loss [1]. Speak with your doctor about taking calcium and vitamin D to protect your bones, and monitor your diet closely.

Second-Line & Steroid-Sparing Agents

If your disease returns during the steroid taper, or if you cannot tolerate steroids, your doctor may introduce “steroid-sparing” therapies.

  • Rituximab: This is a biologic therapy given by infusion. It works by depleting B-cells, the specific immune cells that eventually become the IgG4-producing plasma cells [6][7]. It is highly effective at inducing and maintaining remission [8].
  • Other Immunosuppressants: Medications like mycophenolate mofetil, azathioprine, or methotrexate are sometimes used to keep the immune system in check [9][10].
  • Infection Warning: All of these medications work by suppressing your immune system. You will be at a much higher risk of contracting infections. You must take precautions, such as avoiding sick contacts, practicing excellent hand hygiene, and discussing safe vaccines (like the flu shot) with your doctor before starting treatment [6].

Urological Interventions: Protecting the Kidneys

If the fibrotic mass is squeezing your ureters and blocking urine flow (obstructive uropathy), medical therapy alone may not be fast enough to prevent kidney damage [11][12].

  • Ureteral Stenting: A urologist may place a stent—a thin, flexible tube—inside the ureter to hold it open [11][13]. Stents are completely internal. You can still shower, exercise, and live normally, though you may experience some bladder irritation, frequent urges to urinate, or mild blood in your urine.
  • Nephrostomy: If a stent cannot be passed, a tube may be placed through the skin of your back directly into the kidney (nephrostomy) to drain the urine into a bag [11][14]. Your care team will teach you exactly how to clean and manage this tube at home.
  • Timing: These procedures are often done immediately at diagnosis to stabilize the kidneys while waiting for the medications to start shrinking the mass [14].

Why Inflammation vs. Fibrosis Matters

It is important to understand that your body’s response to treatment depends on whether the mass is currently inflammatory or fibrotic.

  • Inflammatory Phase: Full of active immune cells. It typically responds very well to steroids and Rituximab, often shrinking significantly in a few weeks [15][16].
  • Fibrotic Phase: Consists of dense, “mature” scar tissue (collagen). Because scar tissue is not made of living immune cells, medications like prednisone cannot “melt” it away as easily [15][17].

If your mass is mostly dense fibrosis, it may stay the same size on a CT scan even if the inflammation is gone. In these cases, the goal of treatment shifts from shrinking the mass to preventing it from growing further [18].

Common questions in this guide

How do steroids treat IgG4-related retroperitoneal fibrosis?
Steroids work quickly to reduce active inflammation in the retroperitoneum. By forcing inflammatory cells to leave the area, medications like prednisone can rapidly shrink the size of the mass and relieve pressure on your organs.
What happens if I cannot tolerate steroids for IgG4-RPF?
If steroids cause severe side effects or if your disease returns during a taper, your doctor may prescribe steroid-sparing agents. Biologic medications like Rituximab or other immunosuppressants can effectively keep your immune system in check to maintain remission.
Why might I need a ureteral stent with IgG4-RPF?
If the fibrotic mass compresses your ureters, it can block urine flow and rapidly damage your kidneys. A urologist may place an internal stent to hold the ureter open and protect your kidney function while waiting for medications to shrink the mass.
Can medications completely dissolve the fibrotic mass?
Medications are very effective at shrinking active inflammation, but they cannot easily dissolve mature, dense scar tissue. If your mass is primarily fibrotic, the main goal of treatment shifts to preventing it from growing further rather than completely shrinking it.
What are the risks of taking immunosuppressants for IgG4-RPF?
Because these treatments suppress your immune system, you face a significantly higher risk of catching infections. You will need to take precautions like practicing excellent hand hygiene, avoiding sick contacts, and discussing safe vaccines with your healthcare team.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Is my mass currently in an 'inflammatory' or 'fibrotic' stage, and how does that affect my chance of a full recovery?
  2. 2.Do I need a ureteral stent right now to protect my kidney function, or can we wait to see how I respond to steroids?
  3. 3.What is the long-term plan for tapering my prednisone, and when should we consider adding a steroid-sparing drug like Rituximab?
  4. 4.What specific precautions should I take to avoid infections while on immunosuppressants?
  5. 5.If my symptoms return during the steroid taper, what is the next step for my treatment?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (18)
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    An Interesting Case of Immunoglobulin G4-Related Retroperitoneal Fibrosis Treated With Rituximab.

    Hamdan A, Moeen Z, Tariq H, et al.

    Cureus 2021; (13(9)):e17940 doi:10.7759/cureus.17940.

    PMID: 34660129
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    IgG4-related Disease from Head to Toe.

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    Radiographics : a review publication of the Radiological Society of North America, Inc 2015; (35(7)):2007-25 doi:10.1148/rg.357150066.

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    Retroperitoneal fibrosis: a rare disease for frontline clinicians.

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    Methotrexate and low-dose prednisone in idiopathic retroperitoneal fibrosis: a randomised clinical trial.

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    Journal of autoimmunity 2025; (157()):103487 doi:10.1016/j.jaut.2025.103487.

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    [IgG4-related disease of the breast].

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    Arkhiv patologii 2022; (84(4)):51-55 doi:10.17116/patol20228404151.

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    Rituximab for retroperitoneal fibrosis due to IgG4-related disease: A case report and literature review.

    Almeqdadi M, Al-Dulaimi M, Perepletchikov A, et al.

    Clinical nephrology. Case studies 2018; (6()):4-10 doi:10.5414/CNCS109321.

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    Management of IgG4-related disease.

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    The Lancet. Rheumatology 2019; (1(1)):e55-e65 doi:10.1016/S2665-9913(19)30017-7.

    PMID: 38229361
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    A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta-a clinical and diagnostic challenge.

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    Journal of vascular surgery cases and innovative techniques 2022; (8(2)):275-280 doi:10.1016/j.jvscit.2022.02.003.

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    IgG4-related retroperitoneal fibrosis: a rare cause of obstructive uropathy.

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    Annals of the Royal College of Surgeons of England 2022; (104(4)):e105-e108 doi:10.1308/rcsann.2021.0159.

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    IgG4-related retroperitoneal fibrosis with acute kidney injury: a case report and literature review.

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    PMID: 37542604
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    Rapidly Progressive Kidney Failure Associated with Perirenal Capsular Lesion Due to IgG4-Related Disease.

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    An Uncommon Expression of Immunoglobulin G4 (IgG4)-Related Disease: Sclerosing Mesenteritis Concomitant With IgG4-Related Autoimmune Pancreatitis.

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    In vitro IL-6/IL-6R Trans-Signaling in Fibroblasts Releases Cytokines That May Be Linked to the Pathogenesis of IgG4-Related Disease.

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This page provides educational information about standard treatments for IgG4-RPF. Always consult your urologist, rheumatologist, or primary care doctor to determine the safest medication plan and necessary procedures for your specific condition.

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