Immunoglobulin G4 Related Disease (IgG4-RD)

Immune & Autoimmune
Immunoglobulin G4 Related Disease (IgG4-RD)

Overview

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).

At A Glance
Immune & Autoimmune condition Long-term condition Causes inflammation Can involve multiple organs

Common Signs And Symptoms

  • Swelling Of Salivary Glands
  • Lower Back Pain
  • Fatigue
  • Abdominal Pain
  • Eye Pain
  • Weight Loss

People with IgG4-RD may experience a variety of symptoms including:

Diagnosis Journey

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

Treatment Options

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.

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