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Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis

Sánchez Álamo, B. LU ; Moi, Laura ; Bajema, Ingeborg ; Faurschou, Mikkel ; Flossmann, Oliver ; Hauser, Thomas ; Hrušková, Zdenka ; Jayne, David ; Luqmani, Raashid and Mahr, Alfred , et al. (2023) In Nephrology Dialysis Transplantation 38(7). p.1655-1665
Abstract
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: Long-term follow-up data were collected from questionnaires sent to the principal investigators of the original RCTs (1995-2012): MEPEX, NORAM,... (More)
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: 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: 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: 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. © 2023 The Author(s). Published by Oxford University Press on behalf of the ERA. (Less)
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published
subject
keywords
ANCA-associated vasculitis, autoimmune diseases, granulomatosis with polyangiitis, microscopic polyangiitis, prognostic factors, survival, Adult, Aged, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Antibodies, Antineutrophil Cytoplasmic, Child, Preschool, Granulomatosis with Polyangiitis, Humans, Male, Microscopic Polyangiitis, Middle Aged, Prognosis, neutrophil cytoplasmic antibody, adult, aged, ANCA associated vasculitis, Article, cardiovascular disease, cardiovascular mortality, cardiovascular risk factor, cause of death, clinical evaluation, clinical feature, clinical outcome, cohort analysis, controlled study, estimated glomerular filtration rate, female, follow up, human, laboratory test, major clinical study, male, median survival time, platelet count, prognosis, risk factor, survival rate, Wegener granulomatosis, complication, middle aged, preschool child
in
Nephrology Dialysis Transplantation
volume
38
issue
7
pages
11 pages
publisher
Oxford University Press
external identifiers
  • scopus:85163846086
ISSN
0931-0509
DOI
10.1093/ndt/gfac320
language
English
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yes
id
8f5e1fcb-0b3b-437d-ac62-6813efdc22e5
date added to LUP
2023-11-16 15:48:58
date last changed
2023-11-16 15:49:53
@article{8f5e1fcb-0b3b-437d-ac62-6813efdc22e5,
  abstract     = {{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: 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: 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: 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.  © 2023 The Author(s). Published by Oxford University Press on behalf of the ERA.}},
  author       = {{Sánchez Álamo, B. and Moi, Laura and Bajema, Ingeborg and Faurschou, Mikkel and Flossmann, Oliver and Hauser, Thomas and Hrušková, Zdenka and Jayne, David and Luqmani, Raashid and Mahr, Alfred and Åkesson, Anna and Westman, K.}},
  issn         = {{0931-0509}},
  keywords     = {{ANCA-associated vasculitis; autoimmune diseases; granulomatosis with polyangiitis; microscopic polyangiitis; prognostic factors; survival; Adult; Aged; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antibodies, Antineutrophil Cytoplasmic; Child, Preschool; Granulomatosis with Polyangiitis; Humans; Male; Microscopic Polyangiitis; Middle Aged; Prognosis; neutrophil cytoplasmic antibody; adult; aged; ANCA associated vasculitis; Article; cardiovascular disease; cardiovascular mortality; cardiovascular risk factor; cause of death; clinical evaluation; clinical feature; clinical outcome; cohort analysis; controlled study; estimated glomerular filtration rate; female; follow up; human; laboratory test; major clinical study; male; median survival time; platelet count; prognosis; risk factor; survival rate; Wegener granulomatosis; complication; middle aged; preschool child}},
  language     = {{eng}},
  number       = {{7}},
  pages        = {{1655--1665}},
  publisher    = {{Oxford University Press}},
  series       = {{Nephrology Dialysis Transplantation}},
  title        = {{Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis}},
  url          = {{http://dx.doi.org/10.1093/ndt/gfac320}},
  doi          = {{10.1093/ndt/gfac320}},
  volume       = {{38}},
  year         = {{2023}},
}