Article Dans Une Revue Kidney International Année : 2024

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

1 Service de néphrologie et immunologie clinique [CHRU Tours]
2 SPHERE - methodS in Patients-centered outcomes and HEalth ResEarch
3 CHU Strasbourg - Centre Hospitalier Universitaire [Strasbourg]
4 CoRaKID - CoRaKiD - Maladies rénales fréquentes et rares : des mécanismes moléculaires à la médecine personnalisée
5 CHU Tenon [AP-HP]
6 RID-AGE - Facteurs de Risque et Déterminants Moléculaires des Maladies liées au Vieillissement - U 1167
7 Service de Néphrologie et Transplantation rénale [CHRU-lille]
8 CHUV - Centre Hospitalier Universitaire Vaudois = Lausanne University Hospital [Lausanne]
9 Service de Néphrologie [Rouen]
10 MaMEA Necker - Centre Référence des Maladies Héréditaires du Métabolisme de l'Enfant et de l'Adulte [CHU Necker]
11 Département de Néphrologie [CHU Necker]
12 Centre hospitalier [Valenciennes, Nord]
13 CHRU Tours - Centre Hospitalier Régional Universitaire de Tours
14 CHU Saint-Antoine [AP-HP]
15 CHU Amiens-Picardie
16 MP3CV - Mécanismes physiopathologiques et conséquences des calcifications cardiovasculaires - UR UPJV 7517
17 MITOVASC - MitoVasc - Physiopathologie Cardiovasculaire et Mitochondriale
18 CHU Angers - Centre Hospitalier Universitaire d'Angers
19 Service de Néphrologie-transplantation-dialyse [Bordeaux]
20 CHD Vendée - Centre Hospitalier Départemental - Hôpital de La Roche-sur-Yon
21 Association ECHO [Expansion des Centres d’Hémodialyse de l’Ouest]
22 Service Néphrologie [CH Le Mans]
23 Centre de néphrologie et transplantation rénale [Hôpital de la Conception - APHM]
24 CERIMED - Centre Européen de Recherche en Imagerie médicale
25 C2VN - Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research
26 Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB)
27 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
28 U1064 Inserm - CR2TI - Centre de Recherche en Transplantation et Immunologie - Center for Research in Transplantation and Translational Immunology
29 Team 3 - U1064 Inserm - CR2TI - Team 3 : Integrative transplantation, HLA, Immunology and genomics of kidney injury
30 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
31 Hôpital Foch [Suresnes]
32 CRC (UMR_S_1138 / U1138) - Centre de Recherche des Cordeliers
33 HEGP - Hôpital Européen Georges Pompidou [APHP]
34 CHU Reims - Hôpital universitaire Robert Debré [Reims]
35 Service de néphrologie [Rennes]
36 CH de Saint-Malo [Broussais]
37 ISCHEMIA - Membrane Signalling and Inflammation in reperfusion Injuries
38 URCA - Université de Reims Champagne-Ardenne
Steven Grangé
  • Fonction : Auteur
  • PersonId : 1106103
Paul Coppo
Florent von Tokarski

Résumé

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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Dates et versions

hal-04494315 , version 1 (07-03-2024)

Identifiants

Citer

Valentin Maisons, Anna Duval, Laurent Mesnard, Marie Frimat, Fadi Fakhouri, et al.. Assessment of epidemiology and outcomes of adult patients with kidney-limited thrombotic microangiopathies.. Kidney International, 2024, 105 (5), pp.1100-1112. ⟨10.1016/j.kint.2024.02.014⟩. ⟨hal-04494315⟩
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