IgG4-related disease

摘要: The term "IgG4-related disease" encompasses several disorders described many years ago under various designations depending on the organ or system involved (e.g., Mikulicz syndrome, Riedel's thyroiditis, and retroperitoneal fibrosis). clinical presentation varies widely, as one more organs may be affected, usually in same region of body either synchronously metachronously. main targets are pancreas, bile ducts, salivary glands, lachrymal mediastinal lymph nodes, retroperitoneum. IgG4-related disease is rare, with an estimated incidence 0.2 to 1/100,000 Japan no available data occidental countries. Men older than 50 predominantly affected. Serum IgG4 levels often greater 1.35 g/L. Enlargement organs, which pseudotumoral, due a combination infiltration by T cells IgG4-expressing plasma cells, storiform fibrosis, obliterative thrombophlebitis. Glucocorticoid therapy effective but followed relapses requiring use immunomodulating agents such azathioprine, methotrexate and, recently, rituximab. not autoimmune condition related autoantibodies, neither does it involve immune complexes. Specific joint involvement has been reported very small number patients.