Alveolar haemorrhage in ANCA-associated vasculitis: Long-term outcome and mortality predictors

The Italian Study Group on Lung Involvement in Rheumatic Diseases and the Italian Vasculitis Study Group

Research output: Contribution to journalArticle

Abstract

Introduction: Alveolar haemorrhage (AH) is considered an important cause of morbidity and early mortality in anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV). Objectives: The aim of this study was to identify predictors of outcome in patients with AH-AAV and to evaluate outcome and causes of death in this subset. Materials and methods: A multicenter retrospective study was conducted in 29 Italian Centers. Clinicians were asked to recruit all patients diagnosed with AAV-associated AH during the last 10 years, from 2007 to 2016. Univariate and multivariable analysis were performed. Results: One-hundred and six patients were included (median age at onset of 55 years [IQR 42–67]). The majority were ANCA-positive (PR3 57.1%, MPO 33.7%) and 72.6% had also renal involvement. At presentation, anaemia was shown in 97 (92.4%) patients, hemoptysis in 54 (51.9%), respiratory failure in 68 (66.7%), of whom 48 (70.6%), requiring respiratory support. At the end of the 37 months [IQR 13–77] follow-up, 19/106 (17.9%) patients were dead. The main causes of death were active disease and infections. By stepwise regression analysis, age >65 years (HR 3.66 [95% CI 1.4–9.51], p = 0.008) and the need for respiratory support (HR 4.58 [95% CI 1.51–13.87], p = 0.007) at AH onset were confirmed to be predictive of mortality. Conclusions: Predictors of outcome in AAV-AH were determined. Factors related to the patient's performance status and the severity of the lung involvement strongly influenced the outcome. Balancing harms and benefits for the individual patient in induction and maintenance treatment strategies is crucial.

Original languageEnglish
Article number102397
JournalJournal of Autoimmunity
DOIs
Publication statusAccepted/In press - Jan 1 2020

Fingerprint

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Hemorrhage
Mortality
Cause of Death
Antineutrophil Cytoplasmic Antibodies
Hemoptysis
Age of Onset
Respiratory Insufficiency
Multicenter Studies
Anemia
Retrospective Studies
Regression Analysis
Morbidity
Kidney
Lung

Keywords

  • Alveolar haemorrhage
  • Mortality
  • Outcome
  • Vasculitis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

The Italian Study Group on Lung Involvement in Rheumatic Diseases and the Italian Vasculitis Study Group (Accepted/In press). Alveolar haemorrhage in ANCA-associated vasculitis: Long-term outcome and mortality predictors. Journal of Autoimmunity, [102397]. https://doi.org/10.1016/j.jaut.2019.102397

Alveolar haemorrhage in ANCA-associated vasculitis : Long-term outcome and mortality predictors. / The Italian Study Group on Lung Involvement in Rheumatic Diseases and the Italian Vasculitis Study Group.

In: Journal of Autoimmunity, 01.01.2020.

Research output: Contribution to journalArticle

The Italian Study Group on Lung Involvement in Rheumatic Diseases and the Italian Vasculitis Study Group 2020, 'Alveolar haemorrhage in ANCA-associated vasculitis: Long-term outcome and mortality predictors', Journal of Autoimmunity. https://doi.org/10.1016/j.jaut.2019.102397
The Italian Study Group on Lung Involvement in Rheumatic Diseases and the Italian Vasculitis Study Group. Alveolar haemorrhage in ANCA-associated vasculitis: Long-term outcome and mortality predictors. Journal of Autoimmunity. 2020 Jan 1. 102397. https://doi.org/10.1016/j.jaut.2019.102397
The Italian Study Group on Lung Involvement in Rheumatic Diseases and the Italian Vasculitis Study Group. / Alveolar haemorrhage in ANCA-associated vasculitis : Long-term outcome and mortality predictors. In: Journal of Autoimmunity. 2020.
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abstract = "Introduction: Alveolar haemorrhage (AH) is considered an important cause of morbidity and early mortality in anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV). Objectives: The aim of this study was to identify predictors of outcome in patients with AH-AAV and to evaluate outcome and causes of death in this subset. Materials and methods: A multicenter retrospective study was conducted in 29 Italian Centers. Clinicians were asked to recruit all patients diagnosed with AAV-associated AH during the last 10 years, from 2007 to 2016. Univariate and multivariable analysis were performed. Results: One-hundred and six patients were included (median age at onset of 55 years [IQR 42–67]). The majority were ANCA-positive (PR3 57.1{\%}, MPO 33.7{\%}) and 72.6{\%} had also renal involvement. At presentation, anaemia was shown in 97 (92.4{\%}) patients, hemoptysis in 54 (51.9{\%}), respiratory failure in 68 (66.7{\%}), of whom 48 (70.6{\%}), requiring respiratory support. At the end of the 37 months [IQR 13–77] follow-up, 19/106 (17.9{\%}) patients were dead. The main causes of death were active disease and infections. By stepwise regression analysis, age >65 years (HR 3.66 [95{\%} CI 1.4–9.51], p = 0.008) and the need for respiratory support (HR 4.58 [95{\%} CI 1.51–13.87], p = 0.007) at AH onset were confirmed to be predictive of mortality. Conclusions: Predictors of outcome in AAV-AH were determined. Factors related to the patient's performance status and the severity of the lung involvement strongly influenced the outcome. Balancing harms and benefits for the individual patient in induction and maintenance treatment strategies is crucial.",
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author = "{The Italian Study Group on Lung Involvement in Rheumatic Diseases and the Italian Vasculitis Study Group} and Luca Quartuccio and Milena Bond and Miriam Isola and Sara Monti and Mara Felicetti and Federica Furini and Stefano Murgia and Alvise Berti and Elena Silvestri and Giulia Pazzola and Enrica Bozzolo and Pietro Leccese and Bernd Raffeiner and Simone Parisi and Ilaria Leccese and Francesco Cianci and Silvano Bettio and Pierpaolo Sainaghi and Aurora Ianniello and Viviana Ravagnani and {Bellando Randone}, Silvia and Paola Faggioli and Claudia Lomater and Paolo Stobbione and Francesco Ferro and Michele Colaci and Giuseppina Alfieri and Francesco Carubbi and Erre, {Gian Luca} and Alessandro Giollo and Nicoletta Franzolini and Ditto, {Maria Chiara} and Silvia Balduzzi and Roberto Padoan and Roberto Bortolotti and Alessandra Bortoluzzi and Adriana Cariddi and Angela Padula and {Di Scala}, Gerardo and Elisa Gremese and Fabrizio Conti and Salvatore D'Angelo and {Matucci Cerinic}, Marco and Lorenzo Dagna and Giacomo Emmi and Carlo Salvarani and Giuseppe Paolazzi and Dario Roccatello and Marcello Govoni and Roberto Caporali",
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TY - JOUR

T1 - Alveolar haemorrhage in ANCA-associated vasculitis

T2 - Long-term outcome and mortality predictors

AU - The Italian Study Group on Lung Involvement in Rheumatic Diseases and the Italian Vasculitis Study Group

AU - Quartuccio, Luca

AU - Bond, Milena

AU - Isola, Miriam

AU - Monti, Sara

AU - Felicetti, Mara

AU - Furini, Federica

AU - Murgia, Stefano

AU - Berti, Alvise

AU - Silvestri, Elena

AU - Pazzola, Giulia

AU - Bozzolo, Enrica

AU - Leccese, Pietro

AU - Raffeiner, Bernd

AU - Parisi, Simone

AU - Leccese, Ilaria

AU - Cianci, Francesco

AU - Bettio, Silvano

AU - Sainaghi, Pierpaolo

AU - Ianniello, Aurora

AU - Ravagnani, Viviana

AU - Bellando Randone, Silvia

AU - Faggioli, Paola

AU - Lomater, Claudia

AU - Stobbione, Paolo

AU - Ferro, Francesco

AU - Colaci, Michele

AU - Alfieri, Giuseppina

AU - Carubbi, Francesco

AU - Erre, Gian Luca

AU - Giollo, Alessandro

AU - Franzolini, Nicoletta

AU - Ditto, Maria Chiara

AU - Balduzzi, Silvia

AU - Padoan, Roberto

AU - Bortolotti, Roberto

AU - Bortoluzzi, Alessandra

AU - Cariddi, Adriana

AU - Padula, Angela

AU - Di Scala, Gerardo

AU - Gremese, Elisa

AU - Conti, Fabrizio

AU - D'Angelo, Salvatore

AU - Matucci Cerinic, Marco

AU - Dagna, Lorenzo

AU - Emmi, Giacomo

AU - Salvarani, Carlo

AU - Paolazzi, Giuseppe

AU - Roccatello, Dario

AU - Govoni, Marcello

AU - Caporali, Roberto

PY - 2020/1/1

Y1 - 2020/1/1

N2 - Introduction: Alveolar haemorrhage (AH) is considered an important cause of morbidity and early mortality in anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV). Objectives: The aim of this study was to identify predictors of outcome in patients with AH-AAV and to evaluate outcome and causes of death in this subset. Materials and methods: A multicenter retrospective study was conducted in 29 Italian Centers. Clinicians were asked to recruit all patients diagnosed with AAV-associated AH during the last 10 years, from 2007 to 2016. Univariate and multivariable analysis were performed. Results: One-hundred and six patients were included (median age at onset of 55 years [IQR 42–67]). The majority were ANCA-positive (PR3 57.1%, MPO 33.7%) and 72.6% had also renal involvement. At presentation, anaemia was shown in 97 (92.4%) patients, hemoptysis in 54 (51.9%), respiratory failure in 68 (66.7%), of whom 48 (70.6%), requiring respiratory support. At the end of the 37 months [IQR 13–77] follow-up, 19/106 (17.9%) patients were dead. The main causes of death were active disease and infections. By stepwise regression analysis, age >65 years (HR 3.66 [95% CI 1.4–9.51], p = 0.008) and the need for respiratory support (HR 4.58 [95% CI 1.51–13.87], p = 0.007) at AH onset were confirmed to be predictive of mortality. Conclusions: Predictors of outcome in AAV-AH were determined. Factors related to the patient's performance status and the severity of the lung involvement strongly influenced the outcome. Balancing harms and benefits for the individual patient in induction and maintenance treatment strategies is crucial.

AB - Introduction: Alveolar haemorrhage (AH) is considered an important cause of morbidity and early mortality in anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV). Objectives: The aim of this study was to identify predictors of outcome in patients with AH-AAV and to evaluate outcome and causes of death in this subset. Materials and methods: A multicenter retrospective study was conducted in 29 Italian Centers. Clinicians were asked to recruit all patients diagnosed with AAV-associated AH during the last 10 years, from 2007 to 2016. Univariate and multivariable analysis were performed. Results: One-hundred and six patients were included (median age at onset of 55 years [IQR 42–67]). The majority were ANCA-positive (PR3 57.1%, MPO 33.7%) and 72.6% had also renal involvement. At presentation, anaemia was shown in 97 (92.4%) patients, hemoptysis in 54 (51.9%), respiratory failure in 68 (66.7%), of whom 48 (70.6%), requiring respiratory support. At the end of the 37 months [IQR 13–77] follow-up, 19/106 (17.9%) patients were dead. The main causes of death were active disease and infections. By stepwise regression analysis, age >65 years (HR 3.66 [95% CI 1.4–9.51], p = 0.008) and the need for respiratory support (HR 4.58 [95% CI 1.51–13.87], p = 0.007) at AH onset were confirmed to be predictive of mortality. Conclusions: Predictors of outcome in AAV-AH were determined. Factors related to the patient's performance status and the severity of the lung involvement strongly influenced the outcome. Balancing harms and benefits for the individual patient in induction and maintenance treatment strategies is crucial.

KW - Alveolar haemorrhage

KW - Mortality

KW - Outcome

KW - Vasculitis

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