Imperial College London
Alternate

PROFESSOR H. TERENCE COOK

Faculty of Medicine, Department of Immunology and Inflammation

Emeritus Professor
 
 
 
//

Contact

 

+44 (0)20 3313 2009t.h.cook

 
 
//

Assistant

 

Miss Claudia Rocchi +44 (0)20 3313 2315

 
//

Location

 

9N9Commonwealth BuildingHammersmith Campus

//

Summary

 

Publications

Citation

BibTex format

@article{Sadeghi-Alavijeh:2018:10.1186/s12882-018-1027-x,
author = {Sadeghi-Alavijeh, O and Henderson, S and Bass, P and Cook, T and DeGroot, K and Salama, AD},
doi = {10.1186/s12882-018-1027-x},
journal = {BMC Nephrology},
title = {Crescentic glomerulonephritis with anti-GBM antibody but no glomerular deposition},
url = {http://dx.doi.org/10.1186/s12882-018-1027-x},
volume = {19},
year = {2018}
}
Download

RIS format (EndNote, RefMan)

TY  - JOUR
AB  - BackgroundAnti-glomerular basement membrane (GBM) antibodies are highly specific for Goodpasture’s or anti-GBM disease, in which they are generally directed against the non-collagenous (NC1) domain of the alpha 3 chain of type IV collagen(α3(IV)), and less commonly, toward the α 4(IV) or α 5(IV) chains, which form a triple helical structure in GBM and alveolar basement membrane (ABM). Alterations in the hexameric structure of the NC1 (α3 (IV)), allows novel epitopes to be exposed and an immune response to develop, with subsequent linear antibody deposition along the GBM, leading to a crescentic glomerulonephritis. Positive anti-GBM antibodies are assumed to be pathogenic and capable of binding GBM in vivo, especially in the context of rapidly progressive glomerulonephritis.We have investigated patients with circulating anti-GBM antibodies, reactive to α3 (IV) and human GBM by immunoassays and Western blotting respectively, with focal necrotising crescentic glomerulonephritis but no linear GBM antibody deposition on immunohistochemistry. Three out of four were also ANCA positive. Despite not binding native GBM, patients’ sera showed linear binding to primate glomeruli by indirect immunofluorescence, in the 2 cases tested. Following treatment, significant improvements in kidney function were found in 3/4 patients.Case presentationWe present four patients with crescentic glomerulonephritis and circulating anti-GBM antibodies, but no glomerular binding.ConclusionsThese novel findings, demonstrate that in some patients anti-GBM antibodies may not bind their own GBM. This has important implications for clinical diagnosis, suggesting that histological confirmation of kidney injury by anti-GBM antibodies should be obtained, as non-binding GBM antibodies may be associated with significant renal recovery.
AU  - Sadeghi-Alavijeh,O
AU  - Henderson,S
AU  - Bass,P
AU  - Cook,T
AU  - DeGroot,K
AU  - Salama,AD
DO  - 10.1186/s12882-018-1027-x
PY  - 2018///
SN  - 1471-2369
TI  - Crescentic glomerulonephritis with anti-GBM antibody but no glomerular deposition
T2  - BMC Nephrology
UR  - http://dx.doi.org/10.1186/s12882-018-1027-x
UR  - http://hdl.handle.net/10044/1/64008
VL  - 19
ER  -
Download