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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.
Advances in Chronic Kidney Disease
|June 20, 2020
Summary
Thrombotic microangiopathy, a condition involving low platelets and red blood cell destruction, is linked to complement system dysregulation in hypertensive emergency and scleroderma. Complement blockade may offer a new therapeutic approach for these challenging diseases.
Area of Science:
- Nephrology
- Rheumatology
- Immunology
Background:
- Thrombotic microangiopathy (TMA) presents as thrombocytopenia and microangiopathic hemolytic anemia.
- TMA affects up to 50% of patients with hypertensive emergency and 10-15% with scleroderma.
- These conditions often have poor prognoses.
Purpose of the Study:
- To review the emerging role of the complement system in TMA.
- To evaluate the link between complement dysregulation and TMA in hypertensive emergency and scleroderma.
- To explore the potential of complement blockade as a therapeutic strategy.
Main Methods:
- Literature review focusing on complement's role in TMA.
- Analysis of evidence linking complement dysregulation to TMA manifestation and clinical course.
- Evaluation of the rationale for complement inhibition therapies.
Main Results:
- Complement system dysregulation is implicated in the pathogenesis of TMA.
- Evidence suggests a connection between complement activation and TMA development in specific patient populations.
- Poor outcomes in TMA underscore the need for novel treatments.
Conclusions:
- The complement system plays a significant role in TMA associated with hypertensive emergency and scleroderma.
- Targeting the complement system, specifically via complement blockade, presents a promising therapeutic avenue.
- Further research into complement-targeted therapies is warranted for improving patient outcomes.
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