Immunoglobulin G4 Related Disease (IgG4-RD)

IgG4-RD is a chronic inflammatory condition that can affect multiple organs at a time. It is an immune-mediated disease that may lead to fibrosis (scarring of tissues).

Swelling of salivary glands

Lower back pain

Fatigue

Abdominal pain

Eye pain

Weight loss

IgG4-RD can impact people in many different ways. People may have IgG4-RD for months or even years before a diagnosis is confirmed. This can result in organ damage before the individual is even aware. IgG4-RD is diagnosed by different specialties as the condition affects multiple different organs. Therefore, a combination of tests and findings is required for a diagnosis of IgG4-RD.

  • Elevated serum IgG4 levels are seen in some patients with IgG4-RD, however, IgG4 levels alone do not lead to a diagnosis.
  • A diagnosis should be supported by histopathological findings, including an abundance of IgG4-positive plasma cells and lymphocytes, storiform fibrosis (swirling pattern of fibrosis that can be patchy), and obliterative phlebitis (destruction of veins from cell inflammation).
  • The presence of mass forming lesions are a common characteristic of IgG4-RD. These lesions can cause swelling of the lacrimal and salivary glands, as well as a sausage-shaped pancreas due to immune cell infiltration.
  • Biopsies of the affected organs are conducted to confirm a diagnosis, measure condition progression, and rule out any other diseases

Treatments options for IgG4-RD include:

  • Glucocorticoids (steroid hormones) can be effective in disease control in the early stages of the disease. As a result, glucocorticoids are typically the initial course of treatment, but many patients are unable to achieve remission with this alone.
  • B-cell depletion therapy is given through an IV. A common B-cell depletor for IgG4-RD is Rituxan, which has been successful in terms of remission.
  • Other treatments that are sometimes used include non-steroid immunosuppressant medications like cyclophosphamide, mycophenolate mofetil, azathioprine, mercaptopurine, and methotrexate.

Ongoing Research

IgG4-RD is a rare disease that requires more effective treatment options as there are currently no approved therapies specifically for IgG4-RD. In order to find these new treatments for IgG4-RD, further research is necessary.

Current Research Studies

There are over 15 research studies actively recruiting participants with IgG4-RD. Visit ClinicalTrials.gov to find IgG4-RD research studies near you.

Featured Study

Resources

Find an online community of fellow patients, caregivers, and advocates below
as well as some other general resources!

Living with IgG4-Related Disease
A support group for discussion and information about IgG4-RD.
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IGG4
A support group to discuss day to day struggles and share advice.
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IgG4ward!
IgG4ward! is the first and only organization dedicated to advocacy, research, and support for IgG4-RD.
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Alliance for Patient Access - The Lived Experiences of IgG4-RD Patients
The Alliance for Patient Access created this report to highlight IgG4-RD and patients' experiences with the condition.
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IgG4-RD Life Podcast
This podcast was created to promote awareness, build community support, and advocate for enhanced care and research for individuals impacted by IgG4-RD and other rare diseases.
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Center for Chronic Illness
Center for Chronic Illness offers programs to support those living with chronic illnesses as well as support groups specific to rare chronic illnesses.
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Social Security Disability for IgG4-related disease
Learn more about a successful claim for disability benefits for those who live with IgG4-RD.
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A Rarity In Autoimmunity: IgG4-RD is More Common in Males
Read this article to learn about a study that shows men are more than twice as likely to develop IgG4-RD.
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Amgen Announces Positive Results for Phase 3 Registrational Trial Evaluating UPLIZNA® (inebilizumab-cdon) for Treatment of Immunoglobulin G4-Related Disese (IgG4-RD)
Visit this article to learn about the positive results of Amgen's study on IgG4-RD.
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