IgG4-related disease

From WikiMD's Wellnesspedia

IgG4-related disease (IgG4-RD) is a systemic disease characterized by the infiltration of IgG4-producing plasma cells into various organs, leading to fibrosis and inflammation. The disease can affect multiple organs, including the pancreas, biliary tract, salivary glands, lacrimal glands, kidneys, lungs, lymph nodes, and retroperitoneum.

Etiology[edit | edit source]

The exact cause of IgG4-RD is unknown. However, it is believed to be an autoimmune disease, where the body's immune system mistakenly attacks its own tissues. Some researchers suggest that certain viral infections or genetic predisposition may trigger the disease.

Pathophysiology[edit | edit source]

In IgG4-RD, the body produces an excess of IgG4 antibodies. These antibodies infiltrate various organs, causing inflammation and fibrosis. The disease is characterized by a unique histopathology, with a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis.

Clinical Presentation[edit | edit source]

The clinical presentation of IgG4-RD varies depending on the organs involved. Common symptoms include pain, swelling, and mass effect. In some cases, the disease may be asymptomatic and discovered incidentally during imaging studies for other conditions.

Diagnosis[edit | edit source]

Diagnosis of IgG4-RD is based on a combination of clinical, radiological, and pathological findings. Elevated serum IgG4 levels are suggestive but not diagnostic of the disease. Definitive diagnosis requires histopathological confirmation.

Treatment[edit | edit source]

The mainstay of treatment for IgG4-RD is corticosteroids, which help to reduce inflammation and fibrosis. In refractory cases, immunosuppressive drugs may be used. Regular follow-up is necessary to monitor for disease progression and organ damage.

Prognosis[edit | edit source]

The prognosis of IgG4-RD is variable. Some patients may experience spontaneous remission, while others may have a chronic, relapsing course. Early diagnosis and treatment can help to prevent irreversible organ damage.

File:IgG4-related disease.jpg
Histopathological image of IgG4-related disease showing dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis.

See Also[edit | edit source]

References[edit | edit source]

Resources[edit source]

Latest articles - IgG4-related disease

PubMed
Clinical trials
UpToDate

AI tools[edit source]

Generative AI tools and links to learn more on IgG4-related disease: ChatGPT - Bing Chat - Google Gemini. (caution advised).


Wiki.png

Navigation: Wellness - Encyclopedia - Health topics - Disease Index‏‎ - Drugs - World Directory - Gray's Anatomy - Keto diet - Recipes

Search WikiMD


Ad.Tired of being Overweight? Try W8MD's physician weight loss program.
Semaglutide (Ozempic / Wegovy and Tirzepatide (Mounjaro) available.
Advertise on WikiMD

WikiMD is not a substitute for professional medical advice. See full disclaimer.

Credits:Most images are courtesy of Wikimedia commons, and templates Wikipedia, licensed under CC BY SA or similar.