| Title: |
Assessment of epidemiology and outcomes of adult patients with kidney-limited thrombotic microangiopathies. |
| Authors: |
Maisons, V.; Duval, A.; Mesnard, L.; Frimat, M.; Fakhouri, F.; Grangé, S.; Servais, A.; Cartery, C.; Fauchier, L.; Coppo, P.; Titeca-Beauport, D.; Fage, N.; Delmas, Y.; Quérard, A.H.; Seret, G.; Bobot, M.; Le Quintrec, M.; Ville, S.; von Tokarski, F.; Chauvet, S.; Wynckel, A.; Martins, M.; Schurder, J.; Barbet, C.; Sautenet, B.; Gatault, P.; Caillard, S.; Vuiblet, V.; Halimi, J.M. |
| Contributors: |
MATRIX Consortium Group |
| Publication Year: |
2024 |
| Collection: |
Université de Lausanne (UNIL): Serval - Serveur académique lausannois |
| Subject Terms: |
Adult; Humans; Kidney/pathology; Thrombotic Microangiopathies/epidemiology; Thrombotic Microangiopathies/therapy; Thrombotic Microangiopathies/pathology; Atypical Hemolytic Uremic Syndrome/drug therapy; Atypical Hemolytic Uremic Syndrome/epidemiology; Complement System Proteins; Kidney Function Tests; biopsy; epidemiology; kidney; malignant hypertension; monoclonal gammopathy; thrombotic microangiopathies |
| Description: |
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. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| ISSN: |
1523-1755 |
| Relation: |
Kidney International; https://iris.unil.ch/handle/iris/215892; serval:BIB_9A8D1EA78FBB; 001299541500001 |
| DOI: |
10.1016/j.kint.2024.02.014 |
| Availability: |
https://iris.unil.ch/handle/iris/215892; https://doi.org/10.1016/j.kint.2024.02.014 |
| Accession Number: |
edsbas.BED3C6A1 |
| Database: |
BASE |