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C3 glomerulopathy —...
C3 glomerulopathy — understanding a rare complement-driven renal disease
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- Smith, Richard J.H. (author)
- University of Iowa
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- Appel, Gerald B. (author)
- Columbia University
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- Blom, Anna M. (author)
- Lund University,Lunds universitet,Proteinkemi, Malmö,Forskargrupper vid Lunds universitet,Protein Chemistry, Malmö,Lund University Research Groups
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- Cook, H. Terence (author)
- Imperial College London
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- D’Agati, Vivette D. (author)
- Columbia University
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- Fakhouri, Fadi (author)
- Nantes University Hospital
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- Fremeaux-Bacchi, Véronique (author)
- Necker-Enfants Malades Hospital,Paris Descartes University
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- Józsi, Mihály (author)
- Eötvös Loránd University,Semmelweis University
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- Kavanagh, David (author)
- University of Newcastle upon Tyne
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- Lambris, John D. (author)
- University of Pennsylvania
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- Noris, Marina (author)
- Mario Negri Institute for Pharmacological Research
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- Pickering, Matthew C. (author)
- Imperial College London
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- Remuzzi, Giuseppe (author)
- Mario Negri Institute for Pharmacological Research,University of Milan,Azienda Ospedaliera Papa Giovanni XXIII
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- de Córdoba, Santiago Rodriguez (author)
- Biological Research Center (CIB), Madrid,Biomedical Network on Rare Diseases (CIBERER)
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- Sethi, Sanjeev (author)
- Mayo Clinic Minnesota
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- Van der Vlag, Johan (author)
- Radboud University Nijmegen
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- Zipfel, Peter F. (author)
- Friedrich Schiller University Jena,Leibniz Institute for Natural Product Research and Infection Biology
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- Nester, Carla M. (author)
- University of Iowa
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(creator_code:org_t)
- 2019-01-28
- 2019
- English.
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In: Nature Reviews Nephrology. - : Springer Science and Business Media LLC. - 1759-5061 .- 1759-507X.
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Abstract
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- The C3 glomerulopathies are a group of rare kidney diseases characterized by complement dysregulation occurring in the fluid phase and in the glomerular microenvironment, which results in prominent complement C3 deposition in kidney biopsy samples. The two major subgroups of C3 glomerulopathy — dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) — have overlapping clinical and pathological features suggestive of a disease continuum. Dysregulation of the complement alternative pathway is fundamental to the manifestations of C3 glomerulopathy, although terminal pathway dysregulation is also common. Disease is driven by acquired factors in most patients — namely, autoantibodies that target the C3 or C5 convertases. These autoantibodies drive complement dysregulation by increasing the half-life of these vital but normally short-lived enzymes. Genetic variation in complement-related genes is a less frequent cause. No disease-specific treatments are available, although immunosuppressive agents and terminal complement pathway blockers are helpful in some patients. Unfortunately, no treatment is universally effective or curative. In aggregate, the limited data on renal transplantation point to a high risk of disease recurrence (both DDD and C3GN) in allograft recipients. Clinical trials are underway to test the efficacy of several first-generation drugs that target the alternative complement pathway.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Urologi och njurmedicin (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Urology and Nephrology (hsv//eng)
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- By the author/editor
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Smith, Richard J ...
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Appel, Gerald B.
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Blom, Anna M.
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Cook, H. Terence
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D’Agati, Vivette ...
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Fakhouri, Fadi
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show more...
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Fremeaux-Bacchi, ...
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Józsi, Mihály
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Kavanagh, David
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Lambris, John D.
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Noris, Marina
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Pickering, Matth ...
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Remuzzi, Giusepp ...
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de Córdoba, Sant ...
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Sethi, Sanjeev
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Van der Vlag, Jo ...
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Zipfel, Peter F.
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Nester, Carla M.
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show less...
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Urology and Neph ...
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Nature Reviews N ...
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Lund University