Assessing Complement Dysregulation in the Thrombotic Microangiopathies and Ultrarare Complement‐Mediated Kidney Disease

Amanda K Slagle; Jill J Hauer; Richard J. H Smith

doi:10.1002/9781683674023.ch11

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Book chapter

Assessing Complement Dysregulation in the Thrombotic Microangiopathies and Ultrarare Complement‐Mediated Kidney Disease

Amanda K Slagle, Jill J Hauer and Richard J. H Smith

Manual of Molecular and Clinical Laboratory Immunology, pp.119-128

John Wiley & Sons, Inc

2024

DOI: 10.1002/9781683674023.ch11

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Abstract

This chapter focuses on the clinical consequence of dysregulation of the alternative pathway (AP) of complement. It provides a review of the AP and illustrates the consequence of its dysregulation by describing two ultrarare diseases: atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G). Regulators of complement activations proteins typically control complement by modulating either the generation or breakdown of the C3 and C5 convertases. This regulation of complement activation can occur both in the circulation (fluid phase) and on cell surfaces and extracellular membranes. Hemolytic uremic syndrome is a rare disease characterized by MAHA, thrombocytopenia, and acute renal failure. The rapid development of complement therapeutics has been truly impactful for persons with aHUS. Transplantation is an option for those who progress to end‐stage kidney disease; however, the risk of recurrence after transplantation is impacted by underlying genetic complement abnormalities, which can be identified in ~30% of C3G patients.

alternative pathway

atypical hemolytic uremic syndrome

C3 glomerulopathy

hemolytic uremic syndrome

kidney disease

Details

Title: Subtitle: Assessing Complement Dysregulation in the Thrombotic Microangiopathies and Ultrarare Complement‐Mediated Kidney Disease
Creators: Amanda K Slagle
Jill J Hauer
Richard J. H Smith
Contributors: John L Schmitz (Editor)
Barbara Detrick (Editor)
Maurice R. G O'Gorman (Editor)
Resource Type: Book chapter
Publication Details: Manual of Molecular and Clinical Laboratory Immunology, pp.119-128
DOI: 10.1002/9781683674023.ch11
Publisher: John Wiley & Sons, Inc; Hoboken, NJ, USA
Number of pages: 10
Language: English
Date published: 2024
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
Record Identifier: 9984795371302771

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