Complement and Renal Thrombotic Microangiopathy Associated With Hypertension and Scleroderma

Jonathan E Zuckerman; Anthony Chang

doi:10.1053/j.ackd.2019.11.002

Complement and Renal Thrombotic Microangiopathy Associated With Hypertension and Scleroderma

Adv Chronic Kidney Dis. 2020 Mar;27(2):149-154. doi: 10.1053/j.ackd.2019.11.002.

Authors

Jonathan E Zuckerman¹, Anthony Chang²

Affiliations

¹ Department of Pathology and Laboratory Medicine, University of California, Los Angeles, Los Angeles, CA.
² Department of Pathology, University of Chicago Medicine, Chicago, IL. Electronic address: anthony.chang@uchospitals.edu.

PMID: 32553247
DOI: 10.1053/j.ackd.2019.11.002

Abstract

Thrombotic microangiopathy is characterized by the presence of thrombocytopenia and microangiopathic hemolytic anemia and can occur in up to 50% of patients with hypertensive emergency and 10-15% with scleroderma. This review discusses the emerging role of complement in these 2 clinical entities. Specifically, we evaluate the evidence linking complement dysregulation with the manifestation of thrombotic microangiopathy and its clinical course in these settings. We also explore the rationale for complement blockade in these complex clinical scenarios that often have poor outcomes.

Keywords: Complement; Hypertension; Kidney; Scleroderma; Thrombotic microangiopathies.

Publication types

Review

MeSH terms

Anemia, Hemolytic* / etiology
Anemia, Hemolytic* / immunology
Complement Activation / immunology*
Disease Management
Humans
Kidney
Thrombocytopenia* / etiology
Thrombocytopenia* / immunology
Thrombotic Microangiopathies* / blood
Thrombotic Microangiopathies* / complications
Thrombotic Microangiopathies* / therapy