Symptoms

Symptoms of IgG4-RD can vary depending on which organs of the body are affected. Some IgG4-RD symptoms include:
Swelling of salivary glands
Rash
Fatigue
Abdominal pain
Eye pain
Weight loss

Diagnosis Journey

Learn more about how IgG4-RD can be diagnosed.
When to see your doctor
IgG4-RD can impact people in many different ways. Patients may have IgG4-RD for months or even years before a diagnosis is confirmed. This can result in organ damage before the patient is even aware. Go to your doctor if you are experiencing any signs or symptoms that are consistent with IgG4-RD.
Process for diagnosis
IgG4-RD is diagnosed by different specialities 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 in the blood can indicate the disease.
  • 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.

If you feel that you are not receiving adequate treatment, don't be afraid to explore other physicians and specialists in order to get the care you need.

Specialists

John Smith, MD

Gastroenterologist • Nashville, TN

Link
John Smith, MD

Gastroenterologist • Nashville, TN

Link
John Smith, MD

Gastroenterologist • Nashville, TN

Link
John Smith, MD

Gastroenterologist • Nashville, TN

Link
John Smith, MD

Gastroenterologist • Nashville, TN

Link
John Smith, MD

Gastroenterologist • Nashville, TN

Link

Registered doctors

John Smith, MD

Gastroenterologist • Nashville, TN

Link
John Smith, MD

Gastroenterologist • Nashville, TN

Link
John Smith, MD

Gastroenterologist • Nashville, TN

Link
John Smith, MD

Gastroenterologist • Nashville, TN

Link
John Smith, MD

Gastroenterologist • Nashville, TN

Link
John Smith, MD

Gastroenterologist • Nashville, TN

Link

Ongoing Research and Treatment Options

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

Check out our featured study below. If the INDIGO Study is not right for you, there are over 10 research studies actively recruiting participants. Search this site to find IgG4-RD research studies near you.

Featured Study

INDIGO Study
This is a research study for adults with active IgG4-related disease. The purpose of this study is to learn if a new investigational medicine called obexelimab can safely help prevent flares associated with IgG4-related disease.

Treatments Options

  • 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 to help lower serum IgG4 levels. A common b-cell depletor for IgG4-RD is Rituxan, which has been successful in terms of remission.
  • Other treatments may include biliary stents and chronic therapy. Biliary stents are inserted into the biliary duct to keep it open when it is blocked for symptomatic improvements. Chronic therapy is usually needed even if the disease is not affecting any specific organs yet.

Resources

Find some general resources below!

Living with IgG4-Related Disease
A support group for discussion and information about IgG4-RD.
PSC Support
PSC Support is a patient organization in the UK dedicated to improving the lives of patients and families affected by PSC.
American Liver Foundation
The American Liver Foundation is a non-profit organization that promotes education, advocacy, support services, and research to help prevent, treat, and cure liver diseases.
Children's PSC Foundation
The Children's PSC Foundation is dedicated to helping patients with PSC find treatments that work for them and work on new research for PSC.
RheumMadness Podcast
The “IgG4-RD Classification Criteria” episode of the RheumMadness Podcast highlights IgG4-RD.
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.
Healio Rheuminations
This episode of Healio Rheuminations features John Stone, who leads research in IgG4-related disease. In this episode, he discusses important aspects of what is known about the disease and the future directions of research and treatment.

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.

Social Security Disability for IgG4-related disease
Learn more about a successful claim for disability benefits for those who live with IgG4-RD.

Study Seeks FDA-Approved Therapy for IgG4-RD

Read this article to learn more about how researchers at Massachusetts General Hospital are investigating a new treatment for IgG4-RD.

GI Specialists Seeing More Cases Involving IgG4-Related Disease

Visit this article to learn more about how gastroenterologists are seeing more patients with IgG4-RD.

FAQs

Is IgG4-RD a rare disease?
Yes, IgG4-RD is referred to as a rare disease.
Do patients with IgG4-RD typically have other allergic diseases?
Not all, but many IgG4-RD patients may have aeroallergens (airborne allergens like seasonal allergies) and asthma.
Will IgG4-RD ever go away?
IgG4-RD is almost always a chronic and lifelong disease.
What age group is most affected by IgG4-RD?
IgG4-RD typically affects people between 50-70 years of age.
Where can I find support for my IgG4-RD?
Find different resources for support in the Resources section.
Are males or females more commonly affected by IgG4-RD?
IgG4-RD is more common in males than females.
Are there social limitations for patients with IgG4-RD?
Depending on the disease progression, visible symptoms and discomfort from inflammation can cause mental stress and isolation.
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