Diseases of complement dysregulation-an overview
Open accessAbstract
Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation. Although complement overactivation is common to all, cell surface alternative pathway dysregulation (aHUS), fluid phase alternative pathway dysregulation (C3G), or terminal pathway dysregulation (PNH) predominates resulting in the very different phenotypes seen in these diseases. The mechanism underlying the dysregulation also varies with predominant acquired autoimmune (C3G), somatic mutations (PNH), or inherited germline mutations (aHUS) predisposing to disease. Eculizumab has revolutionized the treatment of PNH and aHUS although has been less successful in C3G. With the next generation of complement therapeutic in late stage development, these archetypal complement diseases will provide the initial targets.
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Patients with genetic abnormalities in the complement system tend to only develop overt disease in one organ (such as AMD, C3 glomerulopathy, or atypical haemolytic uraemic syndrome), even in the presence of rare, pathogenic variants or haploinsufficiency of FH and FI.
“patients with genetic abnormalities in the complement system tend to only develop overt disease in one organ (such as AMD, C3 glomerulopathy, or atypical haemolytic uraemic syndrome), even in the presence of rare, pathogenic variants or haploinsufficiency of FH and FI [81,220,221,222,223].”
From Bruch’s Membrane: A Key Consideration with Complement-Based Therapies for Age-Related Macular Degeneration (2023) · cites this paper as [221]
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References 178 · Cited by 115
- Authors
- Wong, E. K. S., & Kavanagh, D.
- Affiliations
- Royal Victoria Infirmary (GB)
- Corresponding author
- Kavanagh, David
- Venue
- Seminars in Immunopathology
- Published
- 2018-01
- Volume
- 40
- Issue
- 1
- Pages
- 49-64
- ISSN
- 1863-2297 (ISSN-L)
- 1863-2300
- PMC
- PMC5794843
- OpenAlex
- W2784050872
- Cited by
- 115
- References
- 178
- Citations by year
- 2026: 2 · 2025: 7 · 2024: 14 · 2023: 16 · 2022: 15 · 2021: 18 · 2020: 25 · 2019: 10 · 2018: 8
- FWCI
- 1.54
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Locations
- Seminars in Immunopathology (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- Newcastle University ePrints (Newcastle Univesity) (cc-by)
- Funders
- Newcastle University
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Complement system in diseases
- Health Sciences › Medicine › Genetics › Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Health Sciences › Medicine › Nephrology › Renal Diseases and Glomerulopathies
- Keywords
- complement dysregulation, atypical hemolytic uremic syndrome, C3 glomerulopathy, paroxysmal nocturnal hemoglobinuria, alternative complement pathway, terminal complement pathway, eculizumab, complement therapeutics, somatic mutations, germline mutations, autoimmune disease
- MeSH terms
- Animals, Complement System Proteins, Complement Activation, Complement C3, Disease Susceptibility, Glomerulonephritis, Hemoglobinuria, Paroxysmal, Humans, Phenotype, Genetic Predisposition to Disease, Molecular Targeted Therapy, Atypical Hemolytic Uremic Syndrome
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