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Complement and Renal Thrombotic Microangiopathy Associated With Hypertension and Scleroderma
Jonathan E Zuckerman1, Anthony Chang2
1Department of Pathology and Laboratory Medicine, University of California, Los Angeles, Los Angeles, CA.
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.
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.
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