BibTex format
@article{Poo:2019:qjmed/hcz149,
author = {Poo, SX and Tham, CSW and Smith, C and Lee, J and Cairns, T and Galliford, J and Hamdulay, S and Jacyna, M and Levy, JB and McAdoo, S and Roufosse, C and Wernig, F and Mason, J and Pusey, C and Tam, F and Tomlinson, J},
doi = {qjmed/hcz149},
journal = {QJM: An International Journal of Medicine},
pages = {763--769},
title = {IgG4-related disease in a multi-ethnic community: Clinical characteristics and association with malignancy},
url = {http://dx.doi.org/10.1093/qjmed/hcz149},
volume = {112},
year = {2019}
}
RIS format (EndNote, RefMan)
TY - JOUR
AB - BackgroundImmunoglobulin-G4-related disease (IgG4-RD) is a recently recognised fibro-inflammatory condition that can affect multiple organs. Despite growing interest in this condition, the natural history and management of IgG4-RD remain poorly understood.AimTo describe the clinical characteristics, treatment and outcomes of IgG4-RD in a multi-ethnic UK cohort, and investigate its possible association with malignancy.DesignRetrospective analysis of case-note and electronic data.MethodsCases were identified from sub-specialty cohorts and a systematic search of an NHS trust histopathology database using ‘IgG4’ or ‘inflammatory pseudotumour’ as search terms. Electronic records, imaging and histopathology reports were reviewed.Results66 identified cases of IgG4-RD showed a similar multi-ethnic spread to the local population of North West London. The median age was 59 years and 71% of patients were male. Presenting symptoms relating to mass effect of a lesion were present in 48% of cases and the mean number of organs involved was 2.4. 10 patients had reported malignancies with 6 of these being haematological. 83% of those treated with steroids had good initial response, however 50% had relapsing-remitting disease. Rituximab was administered in 11 cases and all achieved an initial serological response. Despite this, 7 patients subsequently relapsed after a mean duration of 11 months and 4 progressed despite treatment.ConclusionsWe report a large UK-based cohort of IgG4-RD that shows no clear ethnic predisposition and a wide range of affected organs. We discuss the use of serum IgG4 concentrations as a disease marker in IgG4-RD, the association with malignant disease and outcomes according to differing treatment regimens.
AU - Poo,SX
AU - Tham,CSW
AU - Smith,C
AU - Lee,J
AU - Cairns,T
AU - Galliford,J
AU - Hamdulay,S
AU - Jacyna,M
AU - Levy,JB
AU - McAdoo,S
AU - Roufosse,C
AU - Wernig,F
AU - Mason,J
AU - Pusey,C
AU - Tam,F
AU - Tomlinson,J
DO - qjmed/hcz149
EP - 769
PY - 2019///
SN - 1460-2393
SP - 763
TI - IgG4-related disease in a multi-ethnic community: Clinical characteristics and association with malignancy
T2 - QJM: An International Journal of Medicine
UR - http://dx.doi.org/10.1093/qjmed/hcz149
UR - http://hdl.handle.net/10044/1/70871
VL - 112
ER -
