SUMMARY, EXPLANATION AND LIMITATIONS:
IgG4-related sclerosing disease is a systemic disease characterised by an increased IgG4 level in the serum, sclerosing fibrosis and diffuses lymphoplasmocytic infiltration with many IgG4-positive plasma cells. Since the affected patients usually respond positively to steroid treatment it is important to recognize the disease and to differentiate it from look-alikes (such as lymphoma) which need other treatments. Clinical manifestations are observed in pancreas, bill duct, gall bladder, lachrymal and salivary gland, retroperitoneum, kidney, lung, breast, thyroid, and prostate.
Immunohistochemical analyses of IgG4-associated sclerosing disease not only show significantly more IgG4- positive plasma cells in affected tissues but also significantly increased IgG4/IgG-ratios. Typical values are >30%. The detection of IgG4-positive plasma cells is also considered to be useful for differentiation of auto-immune pancreatitis (AIP) from pancreas carcinoma.
Isotype: IgG1 kappa
Staining pattern: Cytoplasmic.
Positive control: Tissue sample from tonsil.
This antibody is designed for the specific localization of human IgG4 using IHC techniques in formalin-fixed, paraffin-embedded tissue sections.