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

被引:34
作者
Quartuccio, Luca [1 ]
Bond, Milena [1 ]
Isola, Miriam [2 ]
Monti, Sara [3 ,4 ]
Felicetti, Mara [5 ]
Furini, Federica [6 ]
Murgia, Stefano [7 ,8 ]
Berti, Alvise [9 ]
Silvestri, Elena [10 ]
Pazzola, Giulia [11 ,12 ]
Bozzolo, Enrica [13 ]
Leccese, Pietro [14 ]
Raffeiner, Bernd [15 ]
Parisi, Simone [16 ]
Leccese, Ilaria [17 ]
Cianci, Francesco [18 ]
Bettio, Silvano [19 ]
Sainaghi, Pierpaolo [20 ,21 ,22 ]
Ianniello, Aurora [23 ]
Ravagnani, Viviana [24 ]
Randone, Silvia Bellando [10 ,25 ]
Faggioli, Paola [26 ]
Lomater, Claudia [27 ]
Stobbione, Paolo [28 ]
Ferro, Francesco [29 ]
Colaci, Michele [30 ]
Alfieri, Giuseppina [31 ]
Carubbi, Francesco [32 ]
Erre, Gian Luca [33 ]
Giollo, Alessandro [34 ]
Franzolini, Nicoletta [35 ]
Ditto, Maria Chiara [16 ,36 ]
Balduzzi, Silvia [3 ]
Padoan, Roberto [5 ]
Bortolotti, Roberto [9 ]
Bortoluzzi, Alessandra [6 ]
Cariddi, Adriana [13 ]
Padula, Angela [14 ]
Di Scala, Gerardo [10 ]
Gremese, Elisa [18 ,37 ]
Conti, Fabrizio [17 ]
D'Angelo, Salvatore [14 ]
Cerinic, Marco Matucci [10 ,25 ]
Dagna, Lorenzo [13 ]
Emmi, Giacomo [10 ]
Salvarani, Carlo [11 ,12 ]
Paolazzi, Giuseppe [9 ]
Roccatello, Dario [7 ,8 ]
Govoni, Marcello [6 ]
Schiavon, Franco [5 ]
机构
[1] Acad Hosp Santa Maria della Misericordia, Dept Med, Rheumatol Clin, Udine, Italy
[2] Univ Udine, Inst Stat, Dept Med Area, Udine, Italy
[3] IRCCS Policlin S Matteo Fdn, Dept Rheumatol, Pavia, Italy
[4] Univ Pavia, Expt Med, Pavia, Italy
[5] Univ Padua, Dept Med DIMED, Rheumatol Unit, Padua, Italy
[6] Univ Ferrara, Dept Med Sci, UOL Rheumatol, Ferrara, Italy
[7] G Bosco Hosp, CMID Ctr Res Nephrol Rheumatol & Rare Dis, Interreg Coordinating Ctr Network Rare Dis Piedmo, Nephrol & Dialysis Unit, Turin, Italy
[8] Univ Turin, Turin, Italy
[9] Santa Chiara Hosp, Dept Rheumatol, Trento, Italy
[10] Univ Florence, Dept Expt & Clin Med, Florence, Italy
[11] Azienda USL IRCCS Reggio Emilia, Reggio Emilia, Italy
[12] Univ Modena & Reggio Emilia, Reggio Emilia, Italy
[13] IRCCS Osped San Raffaele, Unit Immunol Rheumatol Allergy & Rare Dis, Milan, Italy
[14] Lucania San Carlo Hosp, Rheumatol Dept, Potenza, Italy
[15] Cent Hosp Bolzano, Dept Med, Rheumatol Unit, Bolzano, BZ, Italy
[16] AOU Citta Salute & Sci Torino, SC Reumatol, Turin, Italy
[17] Sapienza Univ Rome, Dept Internal Med & Med Specialties, Rheumatol Unit, Rome, Italy
[18] Univ Cattolica Sacro Cuore, Rheumatol Inst, Rome, Italy
[19] Univ Hosp Cattinara, Internal Med Dept, Rheumatol Unit, Trieste, Italy
[20] Univ Piemonte Orientale, Rheumatol Unit, CAAD, Novara, Italy
[21] Univ Piemonte Orientale, DiMet, Novara, Italy
[22] AOU Maggiore della Carita, Novara, Italy
[23] ASL NO Novara, Day Hosp Multidisciplinare, Osped Borgomanero, Novara, Italy
[24] ASST Mantova, SSD Allergol & Immunol Clin, Mantua, MN, Italy
[25] AOUC, Div Rheumatol, Florence, Italy
[26] ASST Ovest Milanese, UOC Internal Med, Legnano, Italy
[27] Osped Mauriziano Umberto 1, SSD Reumatol, Turin, Italy
[28] Osped Alessandria, Reumatol, Alessandria, Italy
[29] Univ Pisa, Rheumatol Unit, Pisa, Italy
[30] Univ Catania, Dept Clin & Expt Med, Catania, Italy
[31] ASST Lariana, Reumatol, Como, Italy
[32] ASL1 Avezzano Sulmona Aquila, Rheumatol Unit, Laquila, Italy
[33] AOU Sassari, Reumatol, Sassari, Italy
[34] Univ Verona, Dept Med, Rheumatol Sect, Verona, Italy
[35] Presidio Osped S Antonio, Udine, Italy
[36] Univ Padua, PhD Program Clin & Expt Sci, Padua, Italy
[37] Fdn Policlin Univ Gemelli IRCCS, Div Rheumatol, Rome, Italy
关键词
Vasculitis; Alveolar haemorrhage; Mortality; Outcome; CHURG-STRAUSS-SYNDROME; RHEUMATOLOGY; 1990; CRITERIA; PULMONARY HEMORRHAGE; MICROSCOPIC POLYANGIITIS; WEGENERS-GRANULOMATOSIS; SYSTEMIC VASCULITIS; DAMAGE INDEX; DISEASE; CYCLOPHOSPHAMIDE; CLASSIFICATION;
D O I
10.1016/j.jaut.2019.102397
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
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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