Serum ANCA and Overall Mortality: A 10-Year Retrospective Cohort Study on 1,024 Italian Subjects

BackgroundAntineutrophil cytoplasmic antibodies (ANCA) are primarily involved in the pathogenesis of ANCA-associated vasculitides (AAV). However, ANCA may also be present in healthy subjects and in patients with autoimmune disorders different from AAV. We hypothesized that serum ANCA are associated...

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Main Authors: Enrico Brunetta, Giacomo Ramponi, Marco Folci, Maria De Santis, Emanuela Morenghi, Elena Vanni, Elena Bredi, Raffaello Furlan, Claudio Angelini, Carlo Selmi
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-09-01
Series:Frontiers in Immunology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2021.714174/full
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author Enrico Brunetta
Enrico Brunetta
Giacomo Ramponi
Marco Folci
Marco Folci
Maria De Santis
Maria De Santis
Emanuela Morenghi
Emanuela Morenghi
Elena Vanni
Elena Vanni
Elena Bredi
Raffaello Furlan
Raffaello Furlan
Claudio Angelini
Claudio Angelini
Carlo Selmi
Carlo Selmi
author_facet Enrico Brunetta
Enrico Brunetta
Giacomo Ramponi
Marco Folci
Marco Folci
Maria De Santis
Maria De Santis
Emanuela Morenghi
Emanuela Morenghi
Elena Vanni
Elena Vanni
Elena Bredi
Raffaello Furlan
Raffaello Furlan
Claudio Angelini
Claudio Angelini
Carlo Selmi
Carlo Selmi
author_sort Enrico Brunetta
collection DOAJ
description BackgroundAntineutrophil cytoplasmic antibodies (ANCA) are primarily involved in the pathogenesis of ANCA-associated vasculitides (AAV). However, ANCA may also be present in healthy subjects and in patients with autoimmune disorders different from AAV. We hypothesized that serum ANCA are associated with a worse prognosis in disorders other than AAV.ObjectiveWe investigated the association between the overall survival and the presence of serum ANCA in 1,024 Italian subjects with various testing indications in a 10-year interval.MethodsIn this retrospective cohort study, a population of 6,285 patients (many of whom were subsequently excluded due to our criteria) who tested for ANCA at a single center in 10 years was considered, and life status and comorbidities of subjects were collected. We compared the overall survival of ANCA-positive and ANCA-negative patients by means of Kaplan-Meier curves, while a multivariable adjusted Cox regression was used to evaluate the association between the ANCA status and the outcome (death) in terms of hazard ratios (HR) with 95% confidence intervals (CI).ResultsThe positivity of perinuclear ANCA (pANCA) increased significantly mortality (HR, 1.60; 95% CI, 1.10–2.32), while cytoplasmic ANCA (cANCA) positivity failed to show a significant association (HR, 1.43; 95% CI, 0.77–2.68). The increased mortality rate was observed for both pANCA and cANCA in patients suffering from rheumatic disorders. No association was found between mortality and anti-MPO (HR, 0.63; 95% CI, 0.20–2.00) or anti-PR3 (HR, 0.98; 95% CI, 0.24–3.96) after adjusting for confounders.ConclusionsSerum pANCA and cANCA are independent negative prognostic factors in patients with concurrent autoimmune diseases.
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spelling doaj.art-ca4867dbe7ce4757b6f267a58e2a19ab2022-12-21T18:38:25ZengFrontiers Media S.A.Frontiers in Immunology1664-32242021-09-011210.3389/fimmu.2021.714174714174Serum ANCA and Overall Mortality: A 10-Year Retrospective Cohort Study on 1,024 Italian SubjectsEnrico Brunetta0Enrico Brunetta1Giacomo Ramponi2Marco Folci3Marco Folci4Maria De Santis5Maria De Santis6Emanuela Morenghi7Emanuela Morenghi8Elena Vanni9Elena Vanni10Elena Bredi11Raffaello Furlan12Raffaello Furlan13Claudio Angelini14Claudio Angelini15Carlo Selmi16Carlo Selmi17IRCCS Humanitas Research Hospital, Rozzano, ItalyDepartment of Biomedical Sciences, Humanitas University, Pieve Emanuele, ItalyIRCCS Humanitas Research Hospital, Rozzano, ItalyIRCCS Humanitas Research Hospital, Rozzano, ItalyDepartment of Biomedical Sciences, Humanitas University, Pieve Emanuele, ItalyDepartment of Biomedical Sciences, Humanitas University, Pieve Emanuele, ItalyUnit of Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, ItalyIRCCS Humanitas Research Hospital, Rozzano, ItalyDepartment of Biomedical Sciences, Humanitas University, Pieve Emanuele, ItalyIRCCS Humanitas Research Hospital, Rozzano, ItalyDepartment of Biomedical Sciences, Humanitas University, Pieve Emanuele, ItalyIRCCS Humanitas Research Hospital, Rozzano, ItalyIRCCS Humanitas Research Hospital, Rozzano, ItalyDepartment of Biomedical Sciences, Humanitas University, Pieve Emanuele, ItalyIRCCS Humanitas Research Hospital, Rozzano, ItalyDepartment of Biomedical Sciences, Humanitas University, Pieve Emanuele, ItalyDepartment of Biomedical Sciences, Humanitas University, Pieve Emanuele, ItalyUnit of Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, ItalyBackgroundAntineutrophil cytoplasmic antibodies (ANCA) are primarily involved in the pathogenesis of ANCA-associated vasculitides (AAV). However, ANCA may also be present in healthy subjects and in patients with autoimmune disorders different from AAV. We hypothesized that serum ANCA are associated with a worse prognosis in disorders other than AAV.ObjectiveWe investigated the association between the overall survival and the presence of serum ANCA in 1,024 Italian subjects with various testing indications in a 10-year interval.MethodsIn this retrospective cohort study, a population of 6,285 patients (many of whom were subsequently excluded due to our criteria) who tested for ANCA at a single center in 10 years was considered, and life status and comorbidities of subjects were collected. We compared the overall survival of ANCA-positive and ANCA-negative patients by means of Kaplan-Meier curves, while a multivariable adjusted Cox regression was used to evaluate the association between the ANCA status and the outcome (death) in terms of hazard ratios (HR) with 95% confidence intervals (CI).ResultsThe positivity of perinuclear ANCA (pANCA) increased significantly mortality (HR, 1.60; 95% CI, 1.10–2.32), while cytoplasmic ANCA (cANCA) positivity failed to show a significant association (HR, 1.43; 95% CI, 0.77–2.68). The increased mortality rate was observed for both pANCA and cANCA in patients suffering from rheumatic disorders. No association was found between mortality and anti-MPO (HR, 0.63; 95% CI, 0.20–2.00) or anti-PR3 (HR, 0.98; 95% CI, 0.24–3.96) after adjusting for confounders.ConclusionsSerum pANCA and cANCA are independent negative prognostic factors in patients with concurrent autoimmune diseases.https://www.frontiersin.org/articles/10.3389/fimmu.2021.714174/fullANCAretrospectivecohortvasculitisrheumatoid arthritis
spellingShingle Enrico Brunetta
Enrico Brunetta
Giacomo Ramponi
Marco Folci
Marco Folci
Maria De Santis
Maria De Santis
Emanuela Morenghi
Emanuela Morenghi
Elena Vanni
Elena Vanni
Elena Bredi
Raffaello Furlan
Raffaello Furlan
Claudio Angelini
Claudio Angelini
Carlo Selmi
Carlo Selmi
Serum ANCA and Overall Mortality: A 10-Year Retrospective Cohort Study on 1,024 Italian Subjects
Frontiers in Immunology
ANCA
retrospective
cohort
vasculitis
rheumatoid arthritis
title Serum ANCA and Overall Mortality: A 10-Year Retrospective Cohort Study on 1,024 Italian Subjects
title_full Serum ANCA and Overall Mortality: A 10-Year Retrospective Cohort Study on 1,024 Italian Subjects
title_fullStr Serum ANCA and Overall Mortality: A 10-Year Retrospective Cohort Study on 1,024 Italian Subjects
title_full_unstemmed Serum ANCA and Overall Mortality: A 10-Year Retrospective Cohort Study on 1,024 Italian Subjects
title_short Serum ANCA and Overall Mortality: A 10-Year Retrospective Cohort Study on 1,024 Italian Subjects
title_sort serum anca and overall mortality a 10 year retrospective cohort study on 1 024 italian subjects
topic ANCA
retrospective
cohort
vasculitis
rheumatoid arthritis
url https://www.frontiersin.org/articles/10.3389/fimmu.2021.714174/full
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