Assessment of epidemiology and outcomes of adult patients with kidney-limited thrombotic microangiopathies

非典型溶血尿毒综合征 医学 血栓性微血管病 伊库利珠单抗 胃肠病学 肌酐 内科学 补体系统 免疫学 抗体 疾病
作者
Valentin Maisons,Anna Duval,Laurent Mesnard,Marie Frimat,Fádi Fakhouri,Steven Grangé,Aude Servais,C. Cartery,Laurent Fauchier,Paul Coppo,Dimitri Titeca‐Beauport,Nicolas Fage,Yahsou Delmas,Anne-Hélène Querard,Guillaume Séret,Mickaël Bobot,Moglie Le Quintrec,Simon Ville,Florent Von Tokarski,Sophie Chauvet
出处
期刊:Kidney International [Elsevier BV]
卷期号:105 (5): 1100-1112 被引量:8
标识
DOI:10.1016/j.kint.2024.02.014
摘要

Thrombotic microangiopathies (TMA) are usually associated with hematological features (RH-TMA). The epidemiology of TMA limited to kidneys (RL-TMA) is unclear Therefore, patients with TMA and native kidney biopsies were identified during 2009-2022 in 20 French hospitals and results evaluated. RL-TMA was present in 341/757 (45%) patients and associated with lower creatinine levels (median 184 vs 346 μmol/L) than RH-TMA. RL-TMA resulted from virtually all identified causes, more frequently from anti-VEGF treatment and hematological malignancies, but less frequently from shigatoxin-associated hemolytic uremic syndrome (HUS), systemic sclerosis, gemcitabine and bacterial infection, and even less frequently when three or more causes/triggers were combined (RL-TMA: 5%; RH-TMA: 12%). RL-TMA was associated with significantly lower major cardiovascular events (10% vs 20%), kidney replacement therapy (23% vs 43%) and death (12% vs 20%) than RH-TMA during follow-up (median 28 months). Atypical HUS (aHUS) was found in 326 patients (RL-TMA: 43%, RH-TMA: 44%). Among the 69 patients with proven complement-mediated aHUS, eculizumab (anti-C5 therapy) was used in 43 (62%) (RL-TMA: 35%; RH-TMA: 71%). Among the 257 other patients with aHUS, including 51% with RL-TMA, eculizumab was used in 29 but with unclear effects of this treatment. Thus, RL-TMA represent a very high proportion of patients with TMA and results from virtually all known causes of TMA, includes 25% of patients with complement-mediated aHUS. Adverse outcomes of RL-TMA are lower compared to RH-TMA, but remain significant. Anti-C5 therapy was rarely used in RL-TMA, even in proven complement-mediated aHUS, and its effects remain to be assessed.
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