The authors reply

Abstract

We thank El Karoui and colleagues for their comments. 1 El Karoui K. Boudhabhay I. Frémeaux-Bacchi V. Atypical hemolytic uremic syndrome and hypertensive crisis. Kidney Int. 2019; 96: 1239 Abstract Full Text Full Text PDF Scopus (2) Google Scholar We think that both studies, theirs and ours, 2 El Karoui KE, Boudhabhay I, Petitprez F, et al. Impact of hypertensive emergency and complement rare variants on presentation and outcome of atypical hemolytic uremic syndrome [e-pub ahead of print]. Haematologica. https://doi.org/10.3324/haematol.2019.216903. Accessed August 12, 2019. Google Scholar , 3 Cavero T. Arjona E. Soto K. et al. Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome. Kidney Int. 2019; 96: 995-1004 Abstract Full Text Full Text PDF PubMed Scopus (34) Google Scholar show that severe hypertension is a characteristic feature of atypical hemolytic uremic syndrome (aHUS). Even their patients classified as aHUS not associated with hypertensive emergencies had a median blood pressure of 154/90 mm Hg, which would include hypertension grades 1-2, according to the 2018 European Society of Cardiology/European Society of Hypertension guidelines. 4 Williams B. Mancia G. Spiering W. et al. 2018 ESC/ESH guidelines for the management of arterial hypertension. Eur Heart J. 2018; 39: 3021-3104 Crossref PubMed Scopus (4893) Google Scholar Unlike in their study, we found a favorable effect of eculizumab also in patients with severe and malignant hypertension. However, their data show a trend for better 5-year renal survival among their eculizumab-treated (46%) versus not treated (23%) aHUS patients with hypertensive emergencies. It is likely that the low number of treated patients (13) influenced the lack of statistical significance. Severe and malignant hypertension are common in primary atypical hemolytic uremic syndromeKidney InternationalVol. 96Issue 4PreviewMalignant hypertension is listed among the causes of secondary thrombotic microangiopathy, but pathogenic mutations in complement genes have been reported in patients with hypertension-induced thrombotic microangiopathy. Here we investigated the frequency and severity of hypertension in 55 patients with primary atypical hemolytic uremic syndrome (aHUS). A genetic analysis was performed in all patients, and funduscopic examination was performed in all the patients with Grades 2 and 3 hypertension. Full-Text PDF Atypical hemolytic uremic syndrome and hypertensive crisisKidney InternationalVol. 96Issue 5PreviewCavero et al.1 recently reported the frequency and severity of hypertension in a cohort of 55 patients with atypical hemolytic uremic syndrome (aHUS). A major complication of hemolytic uremic syndrome is hypertensive crisis related to juxtaglomerular ischemia and renin angiotensin system activation. However, hypertensive crisis has been also described as a cause of thrombotic microangiopathy because of endothelial shear stress. Despite the persistent incidence of hypertensive crisis in developed and developing countries,2 the pathophysiological mechanisms and treatment of hypertensive crisis–associated hemolytic uremic syndrome remain debated. Full-Text PDF