ReferenceRRK4713

Natural history and pathogenesis of systemic IgG4 disease

Research Summary

Systemic IgG4 disease may affect multiple organs in the body and is characterised by tissue infiltration with immunoglobulin G4 (IgG4)positive plasma cells. The disease may affect the pancreas the biliary tract the lungs the kidneys and other body organs. The disease may mimic cancer leading to distress and unnecessary invasive surgery if misdiagnosed.There is little understanding of the natural history of disease. The extent (prevalence) of disease is currently unclear. Diagnosis is based upon a combination of serological radiological and histopathological findings. Once diagnosed treatment with corticosteroid results in rapid resolution of symptoms and signs although relapse may occur on withdrawal.This study aims to1. assess the natural history of IgG4 systemic disease and the response to treatment over time 2. investigate the pathogenesis of IgG4 diseasein 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 Radcliffe hospital Oxford and UCH London.A cohort and database of patients with systemic IgG4 disease and a control group of patients (those with high serumIgG4 levels only and diseases that mimic systemic IgG4 disease such as pancreatic cancer and primary sclerosingcholangitis) will be established. These subjects will be assessed annually. Medical details will be recorded and a blood sample taken.Healthy volunteers will be recruited for comparative immunological studies and asked to provide a blood sample once.

Research Overview
Sponsor Oxford University
Project Status Open
Proposed End Date 30/06/2018
Study Run through CRF? No
Target number of patients agreed to recruit 2
Recruitment so far 4