Immunoglobulin G4-related disease is a distinct clinical entity characterised by hyper-IgG4 gammaglobulinemia and IgG4 positive plasma cell infiltration in the tissue. Other characteristic features of this syndrome include mass-forming lesion with fibrosis and prompt and good response to corticosteroids. This disease includes Mikulicz's disease (MD), autoimmune pancreatitis, sclerosing cholangitis, Kuttner's tumour, inflammatory pseudotumor of the lung, liver, and breast, retroperitoneal and mediastinal fibrosis, interstitial nephritis, autoimmune hypophysitis, and many other inflammatory conditions in multiple organs. Glucocorticoids are the mainstay of treatment; recently, rituximab, a select B-cell depleting agent, has been shown to provide an excellent clinical response in select patients.
Category
Immune system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
04 Diseases of the immune system
Nonorgan specific systemic autoimmune disorders
4A43 Overlap or undifferentiated nonorgan specific systemic autoimmune disease
H01761 Immunoglobulin G4-related disease
Masaki Y, Dong L, Kurose N, Kitagawa K, Morikawa Y, Yamamoto M, Takahashi H, Shinomura Y, Imai K, Saeki T, Azumi A, Nakada S, Sugiyama E, Matsui S, Origuchi T, Nishiyama S, Nishimori I, Nojima T, Yamada K, Kawano M, Zen Y, Kaneko M, Miyazaki K, Tsubota K, Eguchi K, Tomoda K, Sawaki T, Kawanami T, Tanaka M, Fukushima T, Sugai S, Umehara H
Title
Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders.
