The early diagnosis and differential diagnosis of IgG4-related diseases / 解放军医学杂志

ABSTRACT

[Abstract] The immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic autoimmune disease characterized by diffuse inflammatory cell infiltration and/or organ enlargement in one or more organ foci and elevated serum IgG4 levels. Typical histopathological changes include lymphocytic cell infiltration, schlieren interstitial fibrosis, and/or occlusive phlebitis. The incidence of IgG4-RD is low, very heterogeneous, and similar to many other diseases in clinical, pathological and laboratory examination, so delayed diagnosis and misdiagnosis are very common. The early diagnosis and differential diagnosis of IgG4-RD have been reviewed in present paper, especially IgG4-related hepatobiliary diseases, IgG4-related skin lesions, Mikulic disease and autoimmune pancreatitis, in order to reduce misdiagnosis.