Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis.
Details
Serval ID
serval:BIB_0CF163CB44AB
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis.
Journal
Nephrology, dialysis, transplantation
Working group(s)
EUVAS
Contributor(s)
Andreas K., Irmgard N., Daniel B., Alain L.M., Zdenka H., Vladimir T., Mikkel F., Wladimir S., Agneta E., Anna S., Alfred M., Solange G.C., Marion H., Raoul B., Michaela S., Matthias S., Wilhelm S., Ulf S., Kirsten G., Smaragdi M., John B., Mark L., Gina G., Augusto V., Federico A., Sinico R., Giacomo G., Annalisa C., Marisa S., Maria B.R., Jolanta D., Ingeborg B., Annelies B., Onno T., Maria C., Georgina E., Jose B., Isabel G., Luis Q., Pérez F.E., María FJG, Beatriz S.Á., Thomas H., Thomas N., Carlo C., Jean-François B., Laura M., Annette B., Mårten S., Kerstin W., Anna Å., Iva G., David J., Lorraine H., Oliver F., Raashid L., Steve M., Peter L., Alan S., Karen D., Joe R., Joe B.
ISSN
1460-2385 (Electronic)
ISSN-L
0931-0509
Publication state
Published
Issued date
30/06/2023
Peer-reviewed
Oui
Volume
38
Number
7
Pages
1655-1665
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Publication Status: ppublish
Abstract
Despite newer treatments with immunosuppressive agents, there still exists a considerable morbidity and mortality risk among patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Since 1994 the European Vasculitis Society (EUVAS) has aimed for an improved outcome for patients with AAV, conducting several prospective randomized controlled trials (RCTs). The aim for the present study was to further evaluate the long-term survival of patients with AAV included in seven RCTs conducted by the EUVAS as well as to identify potential prognostic factors.
Long-term follow-up data were collected from questionnaires sent to the principal investigators of the original RCTs (1995-2012): MEPEX, NORAM, CYCAZAREM, CYCLOPS, IMPROVE, RITUXVAS and MYCYC, comprising 848 patients, all newly diagnosed with AAV. Relative survival estimates are presented for the study cohorts. Demographic, clinical and laboratory characteristics at trial entry were studied as potential prognostic factors in multivariable models.
A total of 478 (56%) patients had granulomatosis with polyangiitis (GPA) and 370 (44%) had microscopic polyangiitis (MPA) with a mean age at diagnosis of 58 ± 14 years. The median follow-up time was 8 years (interquartile range 2.9-13.6). During the observation period there were 305 deaths and the main causes were infections (26%), cardiovascular disease (14%) and malignancies (13%). When compared with a matched cohort (regarding country, age group and sex) from the background population there were 14.2% more deaths among our cohort of AAV patients at 5 years, 19.9% at 10 years, 28.8% at 15 years and 36.3% at 20 years. The excess mortality occurred in all age groups. The estimated median survival time (from diagnosis) was 17.8 years (95% confidence interval 15.7-20). Among variables measured at baseline, advanced age, male sex, low estimated glomerular filtration rate and low platelet count were identified as predictors of death in a multivariate Cox model.
Patients with AAV still have an increased risk of mortality compared with the general population despite newer therapeutic regimens. Treatment complications and organ damage are the main causes of limited survival and infections remain the leading cause of mortality among patients with AAV.
Long-term follow-up data were collected from questionnaires sent to the principal investigators of the original RCTs (1995-2012): MEPEX, NORAM, CYCAZAREM, CYCLOPS, IMPROVE, RITUXVAS and MYCYC, comprising 848 patients, all newly diagnosed with AAV. Relative survival estimates are presented for the study cohorts. Demographic, clinical and laboratory characteristics at trial entry were studied as potential prognostic factors in multivariable models.
A total of 478 (56%) patients had granulomatosis with polyangiitis (GPA) and 370 (44%) had microscopic polyangiitis (MPA) with a mean age at diagnosis of 58 ± 14 years. The median follow-up time was 8 years (interquartile range 2.9-13.6). During the observation period there were 305 deaths and the main causes were infections (26%), cardiovascular disease (14%) and malignancies (13%). When compared with a matched cohort (regarding country, age group and sex) from the background population there were 14.2% more deaths among our cohort of AAV patients at 5 years, 19.9% at 10 years, 28.8% at 15 years and 36.3% at 20 years. The excess mortality occurred in all age groups. The estimated median survival time (from diagnosis) was 17.8 years (95% confidence interval 15.7-20). Among variables measured at baseline, advanced age, male sex, low estimated glomerular filtration rate and low platelet count were identified as predictors of death in a multivariate Cox model.
Patients with AAV still have an increased risk of mortality compared with the general population despite newer therapeutic regimens. Treatment complications and organ damage are the main causes of limited survival and infections remain the leading cause of mortality among patients with AAV.
Keywords
Male, Humans, Adult, Middle Aged, Aged, Child, Preschool, Granulomatosis with Polyangiitis/diagnosis, Granulomatosis with Polyangiitis/therapy, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/therapy, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy, Microscopic Polyangiitis/complications, Microscopic Polyangiitis/diagnosis, Antibodies, Antineutrophil Cytoplasmic, Prognosis, ANCA-associated vasculitis, autoimmune diseases, granulomatosis with polyangiitis, microscopic polyangiitis, prognostic factors, survival
Pubmed
Web of science
Open Access
Yes
Create date
17/01/2023 8:46
Last modification date
09/12/2023 7:02
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