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).

Short Bowel Syndrome with Intestinal Failure (SBS-IF)

Short bowel syndrome with intestinal failure is a condition where individuals cannot absorb nutrients properly because they lack part of their intestine due to portions being surgically removed, parts missing since birth, or portions damaged and unable to function properly.

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

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

Registered doctors

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

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.

Visit Clinicaltrials.gov

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.

IGG4

A support group to discuss day to day struggles and share advice.

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

The “IgG4-related Disease with John Stone, MD” episode of the Healio Rheuminations discusses the histology of IgG4-RD, pathophysiology of the disease, and the future directions of 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.

2023 Autoimmune Community Summit | Autoimmune Association

This virtual two-day event for patients and care partners will include educational and empowering sessions presented by autoimmune professionals such as physicians, nurses, policy experts, and patient advocates. You will hear about pressing topics that impact the autoimmune community. This event will take place on October 5^th at 10:00 AM - October 6^th at 5:00 PM ET.

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.

PatientWing gratefully acknowledges Dr. Raymond Phillips for assistance in the review of this condition information. Updated July 2023.

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