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 language | English |
|---|---|
| Article number | 102397 |
| Journal | Journal of Autoimmunity |
| DOIs | |
| Publication status | Accepted/In press - Jan 1 2020 |
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Keywords
- Alveolar haemorrhage
- Mortality
- Outcome
- Vasculitis
ASJC Scopus subject areas
- Immunology and Allergy
- Immunology
Cite this
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 journal › Article
}
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
UR - http://www.scopus.com/inward/record.url?scp=85077695116&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85077695116&partnerID=8YFLogxK
U2 - 10.1016/j.jaut.2019.102397
DO - 10.1016/j.jaut.2019.102397
M3 - Article
AN - SCOPUS:85077695116
JO - Journal of Autoimmunity
JF - Journal of Autoimmunity
SN - 0896-8411
M1 - 102397
ER -