Imperial College London
Portrait Picture

Emeritus ProfessorCharlesPusey

Faculty of Medicine, Department of Immunology and Inflammation

Emeritus Professor of Medicine
 
 
 
//

Contact

 

+44 (0)20 3313 2308c.pusey

 
 
//

Assistant

 

Miss Anjli Jagpal +44 (0)20 3313 3152

 
//

Location

 

9N4DCommonwealth BuildingHammersmith Campus

//

Summary

 

Publications

Citation

BibTex format

@article{Medjeral-Thomas:2017:10.1016/j.kint.2017.03.043,
author = {Medjeral-Thomas, NR and Lomax-Browne, HJ and Beckwith, H and Willicombe, M and McLean, AG and Brookes, P and Pusey, CD and Falchi, M and Cook, HT and Pickering, MC},
doi = {10.1016/j.kint.2017.03.043},
journal = {Kidney International},
pages = {942--952},
title = {Circulating complement factor H-related proteins 1 and 5 correlate with disease activity in IgA nephropathy},
url = {http://dx.doi.org/10.1016/j.kint.2017.03.043},
volume = {92},
year = {2017}
}
Download

RIS format (EndNote, RefMan)

TY  - JOUR
AB  - IgA nephropathy (IgAN) is a common cause of chronic kidney disease and end-stage renal failure, especially in young people. Due to a wide range of clinical outcomes and difficulty in predicting response to immunosuppression, we need to understand why and identify which patients with IgAN will develop progressive renal impairment. A deletion polymorphism affecting the genes encoding the complement factor H-related protein (FHR)-1 and FHR-3 is robustly associated with protection against IgAN. Some FHR proteins, including FHR-1 and FHR-5, antagonize the ability of complement factor H (fH), the major negative regulator of the complement alternative pathway, to inhibit complement activation on surfaces, a process termed fH deregulation. From a large cohort of patients, we demonstrated that plasma FHR-1 and the FHR-1/fH ratio were elevated in IgAN and associated with progressive disease. Plasma FHR-1 negatively correlated with eGFR but remained elevated in patients with IgAN with normal eGFR. Serum FHR5 was slightly elevated in IgAN but did not correlate with eGFR. Neither FHR5 levels nor the FHR-5/fH ratio was associated with progressive disease. However, higher serum FHR-5 levels were associated with a lack of response to immunosuppression, the presence of endocapillary hypercellularity, and histology scores of disease severity (the Oxford Classification MEST score). Thus, FHR-1 and FHR-5 have a role in IgAN disease progression.
AU  - Medjeral-Thomas,NR
AU  - Lomax-Browne,HJ
AU  - Beckwith,H
AU  - Willicombe,M
AU  - McLean,AG
AU  - Brookes,P
AU  - Pusey,CD
AU  - Falchi,M
AU  - Cook,HT
AU  - Pickering,MC
DO  - 10.1016/j.kint.2017.03.043
EP  - 952
PY  - 2017///
SN  - 0085-2538
SP  - 942
TI  - Circulating complement factor H-related proteins 1 and 5 correlate with disease activity in IgA nephropathy
T2  - Kidney International
UR  - http://dx.doi.org/10.1016/j.kint.2017.03.043
UR  - https://www.sciencedirect.com/science/article/pii/S0085253817302569?via%3Dihub
UR  - http://hdl.handle.net/10044/1/50027
VL  - 92
ER  -
Download