IgG4-related Disease is a systemic condition characterized by the infiltration of IgG4-positive plasma cells into various organs, leading to organ enlargement, fibrosis, and dysfunction. It often mimics other inflammatory and neoplastic conditions, making accurate diagnosis critical for effective treatment with corticosteroids or immunosuppressive agents.

IgG4-related Disease

Systemic condition

IgG4-positive plasma cells are immune cells that produce the IgG4 subclass of antibodies, often associated with a group of conditions known as IgG4-related diseases. These diseases are characterized by tissue infiltration by IgG4-positive plasma cells, leading to inflammation and fibrosis in various organs.

IgG4-positive Plasma Cells

Organ Enlargement

Fibrosis is a pathological process characterized by the excessive accumulation of extracellular matrix components, primarily collagen, leading to tissue scarring and disruption of normal organ function. It is a common outcome of chronic inflammation and can affect various organs, including the liver, lungs, kidneys, and heart, often resulting in significant morbidity and mortality.

Fibrosis

Organ dysfunction refers to the impaired function of an organ system, where the organ is unable to maintain homeostasis without medical intervention. It is often a result of severe illness or injury and can lead to multi-organ failure if not promptly addressed.

Organ Dysfunction

Inflammatory conditions

Neoplastic conditions

Accurate Diagnosis

Effective Treatment

Corticosteroids are a class of steroid hormones produced in the adrenal cortex, used primarily to reduce inflammation and suppress the immune system in various medical conditions. They play a crucial role in managing conditions like asthma, arthritis, and autoimmune diseases, but their long-term use can lead to significant side effects.

Corticosteroids

Immunosuppressive agents are medications that inhibit or prevent activity of the immune system, primarily used to prevent organ transplant rejection and to treat autoimmune diseases. While they are effective in reducing immune response, their use increases the risk of infections and certain cancers due to decreased immune surveillance.

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