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Anti-glomerular basement membrane nephritis and bullous pemphigoid caused by distinct anti-alpha 3(IV)NC1 and anti-BP180 antibodies in a patient with Crohn's disease

Plaisier, E; Borradori, L; Hellmark, Thomas LU ; Wattiaux, MJ; Flageul, B; Mougenot, B and Ronco, P (2002) In American Journal of Kidney Diseases 40(3). p.649-654
Abstract
Background: Anti-glomerular basement membrane (GBM) nephritis is a rare disease induced by antibodies directed against alpha3(IV)NC1, the Goodpasture antigen. We report a patient with Crohn's disease who developed anti-GBM nephritis and the skin blistering disorder bullous pemphigoid, owing to distinct autoantibodies. Methods: Frozen sections of skin and kidney biopsies were incubated with antisera specific for human IgG, IgA, IgM, fibrin, and C3. Reactivity of the patient's serum with GBM antigens was studied by Western blot using bovine solubilized type IV collagen and by enzyme-linked immunosorbent assays using alpha1(IV), alpha3(IV), and alpha5(IV)NC1 recombinant proteins. Reactivity studies against skin antigens were done by Western... (More)
Background: Anti-glomerular basement membrane (GBM) nephritis is a rare disease induced by antibodies directed against alpha3(IV)NC1, the Goodpasture antigen. We report a patient with Crohn's disease who developed anti-GBM nephritis and the skin blistering disorder bullous pemphigoid, owing to distinct autoantibodies. Methods: Frozen sections of skin and kidney biopsies were incubated with antisera specific for human IgG, IgA, IgM, fibrin, and C3. Reactivity of the patient's serum with GBM antigens was studied by Western blot using bovine solubilized type IV collagen and by enzyme-linked immunosorbent assays using alpha1(IV), alpha3(IV), and alpha5(IV)NC1 recombinant proteins. Reactivity studies against skin antigens were done by Western blot using human keratinocyte and dermal extracts and three recombinant forms of the bullous pemphigoid antigen180 (BP180, also called BPAG2 or type XVII collagen). The patient's serum was affinity fractionated on a (IV)NC1 column, and the bound and unbound fractions were analyzed for their reactivity against GBM and skin antigens. Results: The patient had deposits of IgG along the GBM and the epidermal basement membrane zone. Circulating IgG antibodies against alpha3(IV)NC1 were detected. The patient's autoantibodies immunoblotted the intracellular domain but not the extracellular domain of BP180. Reactivity of the patient's IgG with BP180 was found only in the unbound fraction of the serum. Conclusion: The simultaneous development of a rare renal and skin autoimmune disorder, resulting from non-cross-reactive autoantibodies, suggests that a common triggering event could be responsible for the autoimmune injury. (C) 2002 by the National Kidney Foundation, Inc. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
BP180, crescentic, glomerulonephritis, anti-glomerular basement membrane (GBM) antibodies, Crohn's colitis, goodpasture antigen, bullous pemphigoid (BP)
in
American Journal of Kidney Diseases
volume
40
issue
3
pages
649 - 654
publisher
Elsevier
external identifiers
  • wos:000177796000027
  • pmid:12200819
  • scopus:0036724859
ISSN
1523-6838
DOI
10.1053/ajkd.2002.34929
language
English
LU publication?
yes
id
baa32b3e-88e6-41b2-8e5e-c0f0703e9854 (old id 329919)
date added to LUP
2007-11-09 07:53:53
date last changed
2017-09-03 03:47:24
@article{baa32b3e-88e6-41b2-8e5e-c0f0703e9854,
  abstract     = {Background: Anti-glomerular basement membrane (GBM) nephritis is a rare disease induced by antibodies directed against alpha3(IV)NC1, the Goodpasture antigen. We report a patient with Crohn's disease who developed anti-GBM nephritis and the skin blistering disorder bullous pemphigoid, owing to distinct autoantibodies. Methods: Frozen sections of skin and kidney biopsies were incubated with antisera specific for human IgG, IgA, IgM, fibrin, and C3. Reactivity of the patient's serum with GBM antigens was studied by Western blot using bovine solubilized type IV collagen and by enzyme-linked immunosorbent assays using alpha1(IV), alpha3(IV), and alpha5(IV)NC1 recombinant proteins. Reactivity studies against skin antigens were done by Western blot using human keratinocyte and dermal extracts and three recombinant forms of the bullous pemphigoid antigen180 (BP180, also called BPAG2 or type XVII collagen). The patient's serum was affinity fractionated on a (IV)NC1 column, and the bound and unbound fractions were analyzed for their reactivity against GBM and skin antigens. Results: The patient had deposits of IgG along the GBM and the epidermal basement membrane zone. Circulating IgG antibodies against alpha3(IV)NC1 were detected. The patient's autoantibodies immunoblotted the intracellular domain but not the extracellular domain of BP180. Reactivity of the patient's IgG with BP180 was found only in the unbound fraction of the serum. Conclusion: The simultaneous development of a rare renal and skin autoimmune disorder, resulting from non-cross-reactive autoantibodies, suggests that a common triggering event could be responsible for the autoimmune injury. (C) 2002 by the National Kidney Foundation, Inc.},
  author       = {Plaisier, E and Borradori, L and Hellmark, Thomas and Wattiaux, MJ and Flageul, B and Mougenot, B and Ronco, P},
  issn         = {1523-6838},
  keyword      = {BP180,crescentic,glomerulonephritis,anti-glomerular basement membrane (GBM) antibodies,Crohn's colitis,goodpasture antigen,bullous pemphigoid (BP)},
  language     = {eng},
  number       = {3},
  pages        = {649--654},
  publisher    = {Elsevier},
  series       = {American Journal of Kidney Diseases},
  title        = {Anti-glomerular basement membrane nephritis and bullous pemphigoid caused by distinct anti-alpha 3(IV)NC1 and anti-BP180 antibodies in a patient with Crohn's disease},
  url          = {http://dx.doi.org/10.1053/ajkd.2002.34929},
  volume       = {40},
  year         = {2002},
}