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Diagnostic and risk factors for complement defects in hypertensive emergency and thrombotic microangiopathy

Hypertension Feb 13, 2020

Timmermans SAMEG, et al. - In this study with patients with hypertensive emergency and thrombotic microangiopathy (TMA) on kidney biopsy, researchers tested these individuals for ex vivo C5b9 formation on the endothelium and rare variants in complement genes to recognize complement-mediated TMA. The factors related to defects in complement regulation and poor renal results were also defined. In 18 (69%) of 26 cases, experts detected massive ex vivo C5b9 formation on resting endothelial cells at the presentation, these cases included 9 patients who had at least one rare genetic variant. Progression to end-stage renal disease was reported in 13 (72%, N = 18) and 3 (38%, N = 8) patients with massive and normal ex vivo complement activation, respectively. The classification of patients with hypertensive emergency and TMA into distinct categories with potential therapeutic and prognostic implications may be achieved by evaluating both ex vivo C5b9 formation and screening for rare variants in complement genes. An algorithm was introduced to identify patients at the highest risk for defects in complement regulation.
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