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Contactin-1 links a...
Contactin-1 links autoimmune neuropathy and membranous glomerulonephritis
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- Fehmi, Janev (author)
- University of Oxford
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- Davies, Alexander J. (author)
- University of Oxford
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- Antonelou, Marilina (author)
- University College London
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- Keddie, Stephen (author)
- University College London
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- Pikkupeura, Sonja (author)
- University of Oxford
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- Querol, Luis (author)
- Autonomous University of Barcelona
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- Delmont, Emilien (author)
- La Timone University Hospital
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- Cortese, Andrea (author)
- University of Pavia,University College London
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Franciotta, Diego (author)
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- Persson, Staffan (author)
- Lund University,Lunds universitet,Neurologi, Lund,Sektion IV,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Neurology, Lund,Section IV,Department of Clinical Sciences, Lund,Faculty of Medicine
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Barratt, Jonathan (author)
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- Pepper, Ruth (author)
- University College London
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Farinha, Filipa (author)
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Rahman, Anisur (author)
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Canetti, Diana (author)
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Gilbertson, Janet A. (author)
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Rendell, Nigel B. (author)
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- Radunovic, Aleksandar (author)
- Royal London Hospital
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- Minton, Thomas (author)
- University of Bristol
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Fuller, Geraint (author)
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- Murphy, Sinead M. (author)
- Tallaght University Hospital
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- Carr, Aisling S. (author)
- University College London
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- Reilly, Mary R. (author)
- University College London
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Eftimov, Filip (author)
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Wieske, Luuk (author)
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Teunissen, Charlotte E. (author)
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- Roberts, Ian S.D. (author)
- Oxford University Hospital
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- Ashman, Neil (author)
- Royal London Hospital
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Salama, Alan D. (author)
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Rinaldi, Simon (author)
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(creator_code:org_t)
- 2023-03-09
- 2023
- English.
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In: PLoS ONE. - : Public Library of Science (PLoS). - 1932-6203. ; 18:3 March
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http://dx.doi.org/10... (free)
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https://lup.lub.lu.s...
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Abstract
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- Membranous glomerulonephritis (MGN) is a common cause of nephrotic syndrome in adults, mediated by glomerular antibody deposition to an increasing number of newly recognised antigens. Previous case reports have suggested an association between patients with anti-contactin-1 (CNTN1)-mediated neuropathies and MGN. In an observational study we investigated the pathobiology and extent of this potential cause of MGN by examining the association of antibodies against CNTN1 with the clinical features of a cohort of 468 patients with suspected immune-mediated neuropathies, 295 with idiopathic MGN, and 256 controls. Neuronal and glomerular binding of patient IgG, serum CNTN1 antibody and protein levels, as well as immune-complex deposition were determined. We identified 15 patients with immune-mediated neuropathy and concurrent nephrotic syndrome (biopsy proven MGN in 12/12), and 4 patients with isolated MGN from an idiopathic MGN cohort, all seropositive for IgG4 CNTN1 antibodies. CNTN1-containing immune complexes were found in the renal glomeruli of patients with CNTN1 antibodies, but not in control kidneys. CNTN1 peptides were identified in glomeruli by mass spectroscopy. CNTN1 seropositive patients were largely resistant to first-line neuropathy treatments but achieved a good outcome with escalation therapies. Neurological and renal function improved in parallel with suppressed antibody titres. The reason for isolated MGN without clinical neuropathy is unclear. We show that CNTN1, found in peripheral nerves and kidney glomeruli, is a common target for autoantibody-mediated pathology and may account for between 1 and 2% of idiopathic MGN cases. Greater awareness of this cross-system syndrome should facilitate earlier diagnosis and more timely use of effective treatment.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Neurovetenskaper (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Neurosciences (hsv//eng)
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- By the author/editor
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Fehmi, Janev
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Davies, Alexande ...
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Antonelou, Maril ...
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Keddie, Stephen
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Pikkupeura, Sonj ...
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Querol, Luis
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show more...
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Delmont, Emilien
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Cortese, Andrea
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Franciotta, Dieg ...
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Persson, Staffan
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Barratt, Jonatha ...
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Pepper, Ruth
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Farinha, Filipa
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Rahman, Anisur
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Canetti, Diana
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Gilbertson, Jane ...
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Rendell, Nigel B ...
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Radunovic, Aleks ...
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Minton, Thomas
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Fuller, Geraint
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Murphy, Sinead M ...
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Carr, Aisling S.
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Reilly, Mary R.
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Eftimov, Filip
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Wieske, Luuk
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Teunissen, Charl ...
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Roberts, Ian S.D ...
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Ashman, Neil
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Salama, Alan D.
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Rinaldi, Simon
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Basic Medicine
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and Neurosciences
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PLoS ONE
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Lund University