IgG4-related disease

IgG4-related disease (also called IgG4-RD) is an uncommon condition in which the immune system causes inflammation and scarring (fibrosis) in one or more organs of the body. The name comes from a specific type of antibody called immunoglobulin G4 (IgG4), which is often found at high levels in the blood and in affected tissues. This disease can affect almost any organ, but the most commonly involved areas include the pancreas, salivary glands, tear glands (lacrimal glands), bile ducts, kidneys, lungs, and the tissue around the aorta (the body's largest blood vessel). When the pancreas is affected, it can be mistaken for pancreatic cancer. When salivary or tear glands swell, it may look like Sjögren syndrome or lymphoma. Symptoms depend on which organs are involved. Common signs include painless swelling of glands, abdominal pain, jaundice (yellowing of the skin), kidney problems, or shortness of breath. Many patients have multiple organs affected at the same time or over the course of the disease. The condition tends to develop slowly, and patients may not notice symptoms until significant damage has occurred. IgG4-related disease is not a cancer and is not an infection. It is considered an immune-mediated condition, meaning the body's own immune system mistakenly attacks healthy tissues. Treatment typically involves medications that suppress the overactive immune response. Corticosteroids like prednisone are the first-line treatment and are very effective in most patients. For patients who relapse or cannot tolerate steroids, other immunosuppressive drugs or the targeted biologic rituximab may be used. Early diagnosis and treatment are important to prevent permanent organ damage from fibrosis.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Bring these to your next appointment

• Q1.Which of my organs are affected by IgG4-related disease, and how much damage has already occurred?,What is my treatment plan, and how long will I need to take steroids?,What are the side effects of my medications, and how can we minimize them?,How often will I need blood tests and imaging to monitor my condition?,What are the signs of a relapse that I should watch for?,Are there clinical trials or newer treatments available for my condition?,Should I see additional specialists based on which organs are involved?

Common questions about IgG4-related disease

Related conditions

Other rare diseases commonly researched alongside IgG4-related disease.

• Autoimmune pancreatitis→
• Primary sclerosing cholangitis→
• Sarcoidosis→