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IgG4-related disease is an immune-mediated condition that can affect multiple organ systems. Common features include IgG4-related autoimmune pancreatitis, swelling of or within an organ system (an inflammatory pseudotumor), salivary gland disease (which can lead to enlargement of the salivary glands), swollen lymph nodes (lymphadenopathy), skin manifestations, and symptoms consistent with allergies or asthma. The cause of IgG4-related disease is unknown. Some researchers believe that it may be an autoimmune or allergic disorder. IgG4-related disease is usually treated with systemic steroids like prednisone. Immunosuppressive medications (azathioprine or mycophenolate mofetil) and biologics (rituximab or bortezomib) may be used in those who don't respond to steroids or for longer-term management. In severe cases, organ replacement therapy may be needed.
Source: GARD Last updated on 05-01-20
Pieringer H, Parzer I, Wohrer A, Peis P, Oppl B, Zwerina J. IgG4-related disease: an orphan with many faces. Orphan Journal of Rare Diseases. 2014; 9:110.
Last updated on 04-27-20
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