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Epidemiology, Outcomes, and Complement Gene Variants in Secondary Thrombotic Microangiopathies

医学 血栓性血小板减少性紫癜 非典型溶血尿毒综合征 内科学 病因学 血栓性微血管病 移植 入射(几何) 胃肠病学 补体系统 免疫学 抗体 血小板 疾病 物理 光学
作者
Alexis Wérion,P Storms,Ysaline Zizi,Claire Beguin,J. A. Bernards,J Cambier,Karin Dahan,Daan Dierickx,Nathalie Godefroid,Pascale Hilbert,Catherine Lambert,Elena Levtchenko,Thomas Meyskens,Xavier Poiré,Lambert van den Heuvel,Kathleen Claes,Johann Morelle
出处
期刊:Clinical Journal of The American Society of Nephrology [American Society of Nephrology]
卷期号:18 (7): 881-891 被引量:5
标识
DOI:10.2215/cjn.0000000000000182
摘要

Background The identification of complement defects as major drivers of primary atypical hemolytic uremic syndrome (HUS) has transformed the landscape of thrombotic microangiopathies (TMAs), leading to the development of targeted therapies and better patient outcomes. By contrast, little is known about the presentation, genetics, and outcomes of TMA associated with specific diseases or conditions, also referred to as secondary TMA. Methods In this study, we assessed the relative incidence, clinical and genetic spectra, and long-term outcomes of secondary TMA versus other TMAs in consecutive patients hospitalized with a first episode of TMA from 2009 to 2019 at two European reference centers. Results During the study period, 336 patients were hospitalized with a first episode of TMA. Etiologies included atypical HUS in 49 patients (15%), thrombotic thrombocytopenic purpura (TTP) in 29 (9%), shigatoxin-associated HUS in 70 (21%), and secondary TMA in 188 (56%). The main causes of secondary TMA were hematopoietic stem-cell transplantation ( n =56, 30%), solid-organ transplantation ( n =44, 23%), and malignant hypertension ( n =25, 13%). Rare variants in complement genes were identified in 32 of 49 patients (65%) with atypical HUS and eight of 64 patients (13%) with secondary TMA; pathogenic or likely pathogenic variants were found in 24 of 49 (49%) and two of 64 (3%) of them, respectively ( P < 0.001). After a median follow-up of 1157 days, death or kidney failure occurred in 14 (29%), eight (28%), five (7%), and 121 (64%) patients with atypical HUS, TTP, shigatoxin-associated HUS, and secondary TMA, respectively. Unadjusted and adjusted Cox regressions showed that patients with secondary TMA had the highest risk of death or kidney failure (unadjusted hazard ratio [HR], 3.35; 95% confidence interval [CI], 1.85 to 6.07; P < 0.001; adjusted HR, 4.11; 95% CI, 2.00 to 8.46; P < 0.001; considering atypical HUS as reference). Conclusions Secondary TMAs represent the main cause of TMA and are independently associated with a high risk of death and progression to kidney failure.
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