Circulating complement factor H-related proteins 1 and 5 correlate with disease activity in IgA nephropathy

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Title: Circulating complement factor H-related proteins 1 and 5 correlate with disease activity in IgA nephropathy
Authors: Medjeral-Thomas, NR
Lomax-Browne, HJ
Beckwith, H
Willicombe, M
McLean, AG
Brookes, P
Pusey, CD
Falchi, M
Cook, HT
Pickering, MC
Item Type: Journal Article
Abstract: 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.
Issue Date: 30-Jun-2017
Date of Acceptance: 30-Mar-2017
ISSN: 0085-2538
Publisher: Elsevier
Start Page: 942
End Page: 952
Journal / Book Title: Kidney International
Volume: 92
Issue: 4
Copyright Statement: © 2017, International Society of Nephrology. Published by Elsevier Inc. This is an open access article under the CC BY license (
Keywords: IgA nephropathy
glomerular disease
1103 Clinical Sciences
Urology & Nephrology
Publication Status: Published
Appears in Collections:Department of Medicine
Faculty of Medicine

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