IgG4-related disease

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IgG4-related disease (pronounced as "I-G-four-related disease") is a systemic condition characterized by tumefactive lesions and often elevated serum IgG4 concentrations. It was first described in the early 2000s and has since been recognized in virtually every organ system.

Etymology

The term "IgG4-related disease" was coined in 2010 by an international panel of experts. It refers to the immunoglobulin G4 (IgG4) subclass, which is often elevated in this disease.

Definition

IgG4-related disease is a fibroinflammatory condition that can affect any organ system. Its hallmark is a tumefactive lesion with a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and often, but not always, elevated serum IgG4 concentrations.

Symptoms

The symptoms of IgG4-related disease vary depending on the organ system involved. They can range from no symptoms to severe organ dysfunction. Common symptoms include swelling and inflammation in the affected organs, which can lead to pain, fatigue, and weight loss.

Diagnosis

The diagnosis of IgG4-related disease is based on a combination of clinical, radiological, serological, and pathological findings. The presence of IgG4-positive plasma cells in tissue is a key diagnostic feature.

Treatment

The mainstay of treatment for IgG4-related disease is corticosteroids, which can reduce inflammation and improve symptoms. In some cases, other immunosuppressive drugs may be used.

Related Terms

  • Immunoglobulin G4: A subclass of immunoglobulins that is often elevated in IgG4-related disease.
  • Corticosteroids: A class of drugs used to reduce inflammation in various conditions, including IgG4-related disease.
  • Storiform fibrosis: A pattern of fibrosis often seen in IgG4-related disease.

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