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

Autor:	Maisons V; Service de Néphrologie, CHU de Tours, Tours, France; U1246, INSERM, SPHERE, Université de Tours, Université de Nantes, Tours, Nantes, France., Duval A; Service de Néphrologie, CHU de Strasbourg, Strasbourg, France., Mesnard L; Service de Néphrologie, APHP Hopital Tenon, Paris, France., Frimat M; Service de Néphrologie, CHU de Lille, Lille, France., Fakhouri F; Service de Néphrologie, CHU Vaudois, Lausanne, Switzerland., Grangé S; Service de Néphrologie, CHU de Rouen, Rouen, France., Servais A; Service de Néphrologie, APHP Hopital Necker, Paris, France., Cartery C; Service de Néphrologie, CH de Valenciennes, Valenciennes, France., Fauchier L; Service de Cardiologie, CHU de Tours, Tours, France., Coppo P; Service d'Hématologie, Centre de référence pour les microangiopathies thrombotiques (CNR-MAT), APHP Hopital Saint-Antoine, Paris, France., Titeca-Beauport D; Service de Néphrologie, CHU d'Amiens, Amiens, France., Fage N; Service de Néphrologie, Département de médecine intensive reanimation-médecine hyperbare, CHU d'Angers, Angers, France., Delmas Y; Service de Néphrologie, CHU de Bordeaux, Bordeaux, France., Quérard AH; Service de Néphrologie, CH de La-Roche-Sur-Yon, La-Roche-Sur-Yon, France., Seret G; Service de Néphrologie, Pole Santé Sud Echo Le Mans, Le Mans, France., Bobot M; Service de Néphrologie, CHU de Marseille; Aix, Marseille Université, INSERM 1263, INRAE 1260, C2VN, CERIMED, Marseille, France., Le Quintrec M; Service de Néphrologie, CHU de Montpellier, Montpellier, France., Ville S; Service de Néphrologie, CHU de Nantes, Nantes, France., von Tokarski F; Service de Néphrologie, Hopital Foch, Paris, France., Chauvet S; Service de Néphrologie, APHP Hopital Européen Georges Pompidou, Paris, France., Wynckel A; Service de Néphrologie, CHU de Reims, Reims, France., Martins M; Service de Néphrologie, CHU de Rennes, Rennes, France., Schurder J; Service de Néphrologie, CH de Saint-Malo, Saint-Malo, France., Barbet C; Service de Néphrologie, CHU de Tours, Tours, France., Sautenet B; Service de Néphrologie, CHU de Tours, Tours, France., Gatault P; Service de Néphrologie, CHU de Tours, U1327, INSERM, ISCHEMIA, Université de Tours, Tours, France., Caillard S; U1246, INSERM, SPHERE, Université de Tours, Université de Nantes, Tours, Nantes, France., Vuiblet V; Service de Pathologie, Institut d'Intelligence Artificielle en Santé, CHU de Reims et Université de Reims Champagne Ardenne, Reims, France., Halimi JM; Service de Néphrologie, CHU de Tours, U1327, INSERM, ISCHEMIA, Université de Tours, Tours, France. Electronic address: halimi@med.univ-tours.fr.
Jazyk:	angličtina
Zdroj:	Kidney international [Kidney Int] 2024 May; Vol. 105 (5), pp. 1100-1112. Date of Electronic Publication: 2024 Feb 29.
DOI:	10.1016/j.kint.2024.02.014
Abstrakt:	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 represents a very high proportion of patients with TMA and results from virtually all known causes of TMA and 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. (Copyright © 2024 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)
Databáze:	MEDLINE
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