Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis.
ANCA-associated vasculitis
autoimmune diseases
granulomatosis with polyangiitis
microscopic polyangiitis
prognostic factors
survival
Journal
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
ISSN: 1460-2385
Titre abrégé: Nephrol Dial Transplant
Pays: England
ID NLM: 8706402
Informations de publication
Date de publication:
30 Jun 2023
30 Jun 2023
Historique:
received:
10
05
2022
medline:
3
7
2023
pubmed:
9
1
2023
entrez:
8
1
2023
Statut:
ppublish
Résumé
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.
Sections du résumé
BACKGROUND
BACKGROUND
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.
METHODS
METHODS
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.
RESULTS
RESULTS
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.
CONCLUSIONS
CONCLUSIONS
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.
Identifiants
pubmed: 36617233
pii: 6973211
doi: 10.1093/ndt/gfac320
doi:
Substances chimiques
Antibodies, Antineutrophil Cytoplasmic
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1655-1665Subventions
Organisme : European Renal Association
Organisme : Vasculitis Foundation
Organisme : Region Skåne
ID : REGSKANE-824921
Organisme : Njurfonden
Investigateurs
Kronbichler Andreas
(K)
Neumann Irmgard
(N)
Blockmans Daniel
(B)
Le Moine Alain
(LM)
Hruskova Zdenka
(H)
Tesar Vladimir
(T)
Faurschou Mikkel
(F)
Szpirt Wladimir
(S)
Ekstrand Agneta
(E)
Salmela Anna
(S)
Mahr Alfred
(M)
Gonzalez Chiappe Solange
(GC)
Haubitz Marion
(H)
Bergner Raoul
(B)
Streubert Michaela
(S)
Schaier Matthias
(S)
Schmitt Wilhelm
(S)
Schönenmarck Ulf
(S)
De Groot Kirsten
(G)
Marinaki Smaragdi
(M)
Boletis John
(B)
Little Mark
(L)
Gregorini Gina
(G)
Vaglio Augusto
(V)
Alberici Federico
(A)
Renato Sinico
(R)
Garibotto Giacomo
(G)
Carta Annalisa
(C)
Santostefano Marisa
(S)
Brugnano Rachele Maria
(BR)
Dadoniene Jolanta
(D)
Bajema Ingeborg
(B)
Berden Annelies
(B)
Teng Onno
(T)
Cid Maria
(C)
Espigol Georgina
(E)
Ballarin Jose
(B)
García Isabel
(G)
Quintana Luis
(Q)
Fernández Elia Pérez
(FE)
Fernández Juárez Gema María
(FJG)
Sánchez Álamo Beatriz
(SÁ)
Hauser Thomas
(H)
Neumann Thomas
(N)
Chizzolini Carlo
(C)
Balavoine Jean-François
(B)
Moi Laura
(M)
Bruchfeld Annette
(B)
Segelmark Mårten
(S)
Westman Kerstin
(W)
Åkesson Anna
(Å)
Gunnarsson Iva
(G)
Jayne David
(J)
Harper Lorraine
(H)
Flossmann Oliver
(F)
Luqmani Raashid
(L)
Mcadoo Steve
(M)
Lanyon Peter
(L)
Salama Alan
(S)
Dahlsveen Karen
(D)
Rosa Joe
(R)
Barrett Joe
(B)
Informations de copyright
© The Author(s) 2023. Published by Oxford University Press on behalf of the ERA.