IgG4-Related Disease

Overview

IgG4-related disease is a condition in which the immune system is abnormally regulated and causes inflammation and scarring in one or more organs, commonly the salivary and tear glands, thyroid, lungs, aorta, kidneys, pancreas, and bile ducts, among others. It typically affects middle-aged to older adults and is more common in men, though it can involve more than one organ at the same time and, rarely, occurs in children. Many people have few or no symptoms early on, so organ damage can build silently before the disease is caught, and in some organs it can look enough like cancer to complicate diagnosis.

Questions to Ask Your Doctor

• Which organs show signs of involvement, and how will we monitor them over time?
• Could this be something else, like cancer, and how did you rule that out?
• Why are you starting treatment with steroids, and what happens if the disease comes back after we taper the dose?
• Would rituximab be a better option for me than long-term steroids?
• Since this needs ongoing treatment, how often will I need follow-up visits and testing?

Getting Care

A rheumatologist experienced in IgG4-related disease should coordinate diagnosis and treatment, often together with specialists for whichever organs are involved. Seek care sooner if you notice new swelling, jaundice, shortness of breath, or vision changes, since these can signal that another organ has become involved.

Source: https://rheumatology.org/patients/igg4-related-disease-igg4-rd