Systemic IgG4 disease may affect multiple organs in the body, and is characterised by tissue infiltration with immunogglobulin G4 (IgG4)-positive plasma cells. The disease may affect the pancreas, the biliary tract, the lungs, the kidneys and other body organs.
There is little understanding of the natural history of the disease. The extent of the disease is currently unclear, diagnosis is based upon a combination of erological, radiological and histopathological findings. Once diagnosed treatment with corticosteriod results in rapid resolution of symptoms and signs, although relapse may occur on withdrawal.
This study aims to :
1) assess the natural history of IgG4 systemic disease and the response to treatment over time.
2) Investigate the pathogenesis of IgG4 disease – in particular to evaluate the phenotype and function of IgG4 producing B cells and determine immunological factors that drive the expansion of IgG4 subsets.
Participants will be recruited from the John Radcliff hospital, and other sites across the UK.
A cohort and database of patients with systemic IgG4 disease and a control group of patients (those with high serum IgG4 levels only, and diseases that mimic systemic IgG4 disease such as pancreatic cancer and primary sclerosing cholangitis) will be established. These subjects will be assessed annually. Medical details will be recorded and blood samples taken. Healthy volunteers will be recruited for comparative immunological studies and asked to provide a blood sample once.