Alveolar haemorrhage in ANCA-associated vasculitis: Long-term outcome and mortality predictors.,Journal of Autoimmunity

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Alveolar haemorrhage in ANCA-associated vasculitis: Long-term outcome and mortality predictors.
Journal of Autoimmunity ( IF 7.9 ) Pub Date : 2020-01-09 , DOI: 10.1016/j.jaut.2019.102397
Luca Quartuccio ₁ , Milena Bond ₁ , Miriam Isola ₂ , Sara Monti ₃ , Mara Felicetti ₄ , Federica Furini ₅ , Stefano Murgia ₆ , Alvise Berti ₇ , Elena Silvestri ₈ , Giulia Pazzola ₉ , Enrica Bozzolo ₁₀ , Pietro Leccese ₁₁ , Bernd Raffeiner ₁₂ , Simone Parisi ₁₃ , Ilaria Leccese ₁₄ , Francesco Cianci ₁₅ , Silvano Bettio ₁₆ , Pierpaolo Sainaghi ₁₇ , Aurora Ianniello ₁₈ , Viviana Ravagnani ₁₉ , Silvia Bellando Randone ₂₀ , Paola Faggioli ₂₁ , Claudia Lomater ₂₂ , Paolo Stobbione ₂₃ , Francesco Ferro ₂₄ , Michele Colaci ₂₅ , Giuseppina Alfieri ₂₆ , Francesco Carubbi ₂₇ , Gian Luca Erre ₂₈ , Alessandro Giollo ₂₉ , Nicoletta Franzolini ₃₀ , Maria Chiara Ditto ₃₁ , Silvia Balduzzi ₃₂ , Roberto Padoan ₄ , Roberto Bortolotti ₇ , Alessandra Bortoluzzi ₅ , Adriana Cariddi ₁₀ , Angela Padula ₁₁ , Gerardo Di Scala ₈ , Elisa Gremese ₃₃ , Fabrizio Conti ₁₄ , Salvatore D'Angelo ₁₁ , Marco Matucci Cerinic ₂₀ , Lorenzo Dagna ₁₀ , Giacomo Emmi ₈ , Carlo Salvarani ₉ , Giuseppe Paolazzi ₇ , Dario Roccatello ₆ , Marcello Govoni ₅ , Franco Schiavon ₄ , Roberto Caporali ₃₂ , Salvatore De Vita ₁ ,

Affiliation

Rheumatology Clinic, Department of Medicine, Academic Hospital "Santa Maria della Misericordia", Udine, Italy.
Institute of Statistics, Department of Medical Area, University of Udine, Udine, Italy.
Department of Rheumatology, IRCCS Policlinico S. Matteo Fondazione, Pavia, Italy; University of Pavia, PhD in Experimental Medicine, Pavia, Italy.
Rheumatology Unit, Department of Medicine DIMED, University of Padova, Italy.
Department of Medical Sciences, UOL Rheumatology, University of Ferrara, Ferrara, Italy.
CMID-Center of Research of Nephrology, Rheumatology, and Rare Diseases Interregional Coordinating Center of the Network of Rare Diseases of Piedmont and Aosta Valley, Nephrology and Dialysis Unit (ERK-net Member) G. Bosco Hospital, and University of Turin, Italy.
Department of Rheumatology, Santa Chiara Hospital, Trento, Italy.
Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Azienda USL-IRCCS di Reggio Emilia and University of Modena and Reggio Emilia, Italy.
Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS Ospedale San Raffaele, Milan, Italy.
Rheumatology Department of Lucania - San Carlo Hospital, Potenza, Italy.
Rheumatology Unit, Department of Medicine, Centrale Hospital of Bolzano, Italy.
S.C. Reumatologia, A.O.U. Città della Salute e della Scienza di Torino, Turin, Italy.
Rheumatology Unit, Department of Internal Medicine and Medical Specialties, Sapienza University of Rome, Italy.
Rheumatology Institute, Università Cattolica del Sacro Cuore, Rome, Italy.
Rheumatology Unit, Internal Medicine Department, University Hospital of Cattinara, Trieste, Italy.
Rheumatology Unit, CAAD and DiMet, University of Eastern Piedmont and AOU Maggiore della Carità, Novara, Italy.
Day Hospital Multidisciplinare, Ospedale di Borgomanero, ASL-NO Novara, Novara, Italy.
SSD Allergologia Ed Immunologia Clinica, ASST Mantova, Italy.
Department of Experimental and Clinical Medicine University of Florence and Division of Rheumatology AOUC, Florence, Italy.
ASST Ovest Milanese, UOC Internal Medicine, Legnano, Italy.
SSD Reumatologia, Ospedale Mauriziano, Turin, Italy.
Reumatologia Ospedale Alessandria, Alessandria, Italy.
Rheumatology Unit, University of Pisa, Pisa, Italy.
Dept Clinical and Experimental Medicine University of Catania, Catania, Italy.
Reumatologia ASST Lariana, Como, Italy.
Rheumatology Unit, ASL1 Avezzano-Sulmona-L'Aquila, L'Aquila, Italy.
Reumatologia - AOU Sassari, Sassari, Italy.
Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy.
Presidio Ospedaliero "S. Antonio", San Daniele (Udine), Italy.
S.C. Reumatologia, A.O.U. Città della Salute e della Scienza di Torino, Turin, Italy; University of Padua, PhD Program in Clinical and Experimental Sciences, Padua, Italy.
Department of Rheumatology, IRCCS Policlinico S. Matteo Fondazione, Pavia, Italy.
Rheumatology Institute, Università Cattolica del Sacro Cuore, Rome, Italy; Division of Rheumatology, Fondazione Policlinico Universitario A. Gemelli-IRCCS, Rome, Italy.

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.

中文翻译：

ANCA相关性血管炎的肺泡出血：长期预后和死亡率预测指标。

介绍

肺泡出血（AH）被认为是抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）发病和早期死亡的重要原因。

目标

这项研究的目的是确定AH-AAV患者预后的指标，并评估该亚组的预后和死亡原因。

材料和方法

在29个意大利中心进行了多中心回顾性研究。从2007年到2016年，临床医生被要求招募过去10年中所有被诊断为AAV相关性AH的患者。进行了单因素和多因素分析。

结果

纳入106例患者（中位年龄为55岁[IQR 42-67]）。多数为ANCA阳性（PR3为57.1％，MPO为33.7％），也有72.6％的肾脏受累。演讲时，有97名患者（92.4％）出现贫血，咯血症54名（51.9％），呼吸衰竭68名（66.7％），其中48名（70.6％）需要呼吸支持。在37个月[IQR 13-77]随访结束时，有19/106（17.9％）患者死亡。死亡的主要原因是活动性疾病和感染。通过逐步回归分析，在AH发作时年龄> 65岁（HR 3.66 [95％CI 1.4–9.51]，p = 0.008）和需要呼吸支持（HR 4.58 [95％CI 1.51–13.87]，p = 0.007）。被证实可以预测死亡率。