Luca Quartuccio1, Milena Bond2, Miriam Isola3, Sara Monti4, Mara Felicetti5, Federica Furini6, Stefano Murgia7, Alvise Berti8, Elena Silvestri9, Giulia Pazzola10, Enrica Bozzolo11, Pietro Leccese12, Bernd Raffeiner13, Simone Parisi14, Ilaria Leccese15, Francesco Cianci16, Silvano Bettio17, Pierpaolo Sainaghi18, Aurora Ianniello19, Viviana Ravagnani20, Silvia Bellando Randone21, Paola Faggioli22, Claudia Lomater23, Paolo Stobbione24, Francesco Ferro25, Michele Colaci26, Giuseppina Alfieri27, Francesco Carubbi28, Gian Luca Erre29, Alessandro Giollo30, Nicoletta Franzolini31, Maria Chiara Ditto32, Silvia Balduzzi33, Roberto Padoan5, Roberto Bortolotti8, Alessandra Bortoluzzi6, Adriana Cariddi11, Angela Padula12, Gerardo Di Scala9, Elisa Gremese34, Fabrizio Conti15, Salvatore D'Angelo12, Marco Matucci Cerinic21, Lorenzo Dagna11, Giacomo Emmi9, Carlo Salvarani10, Giuseppe Paolazzi8, Dario Roccatello7, Marcello Govoni6, Franco Schiavon5, Roberto Caporali33, Salvatore De Vita2. 1. Rheumatology Clinic, Department of Medicine, Academic Hospital "Santa Maria della Misericordia", Udine, Italy. Electronic address: luca.quartuccio@asuiud.sanita.fvg.it. 2. Rheumatology Clinic, Department of Medicine, Academic Hospital "Santa Maria della Misericordia", Udine, Italy. 3. Institute of Statistics, Department of Medical Area, University of Udine, Udine, Italy. 4. Department of Rheumatology, IRCCS Policlinico S. Matteo Fondazione, Pavia, Italy; University of Pavia, PhD in Experimental Medicine, Pavia, Italy. 5. Rheumatology Unit, Department of Medicine DIMED, University of Padova, Italy. 6. Department of Medical Sciences, UOL Rheumatology, University of Ferrara, Ferrara, Italy. 7. 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. 8. Department of Rheumatology, Santa Chiara Hospital, Trento, Italy. 9. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. 10. Azienda USL-IRCCS di Reggio Emilia and University of Modena and Reggio Emilia, Italy. 11. Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS Ospedale San Raffaele, Milan, Italy. 12. Rheumatology Department of Lucania - San Carlo Hospital, Potenza, Italy. 13. Rheumatology Unit, Department of Medicine, Centrale Hospital of Bolzano, Italy. 14. S.C. Reumatologia, A.O.U. Città della Salute e della Scienza di Torino, Turin, Italy. 15. Rheumatology Unit, Department of Internal Medicine and Medical Specialties, Sapienza University of Rome, Italy. 16. Rheumatology Institute, Università Cattolica del Sacro Cuore, Rome, Italy. 17. Rheumatology Unit, Internal Medicine Department, University Hospital of Cattinara, Trieste, Italy. 18. Rheumatology Unit, CAAD and DiMet, University of Eastern Piedmont and AOU Maggiore della Carità, Novara, Italy. 19. Day Hospital Multidisciplinare, Ospedale di Borgomanero, ASL-NO Novara, Novara, Italy. 20. SSD Allergologia Ed Immunologia Clinica, ASST Mantova, Italy. 21. Department of Experimental and Clinical Medicine University of Florence and Division of Rheumatology AOUC, Florence, Italy. 22. ASST Ovest Milanese, UOC Internal Medicine, Legnano, Italy. 23. SSD Reumatologia, Ospedale Mauriziano, Turin, Italy. 24. Reumatologia Ospedale Alessandria, Alessandria, Italy. 25. Rheumatology Unit, University of Pisa, Pisa, Italy. 26. Dept Clinical and Experimental Medicine University of Catania, Catania, Italy. 27. Reumatologia ASST Lariana, Como, Italy. 28. Rheumatology Unit, ASL1 Avezzano-Sulmona-L'Aquila, L'Aquila, Italy. 29. Reumatologia - AOU Sassari, Sassari, Italy. 30. Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. 31. Presidio Ospedaliero "S. Antonio", San Daniele (Udine), Italy. 32. 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. 33. Department of Rheumatology, IRCCS Policlinico S. Matteo Fondazione, Pavia, Italy. 34. Rheumatology Institute, Università Cattolica del Sacro Cuore, Rome, Italy; Division of Rheumatology, Fondazione Policlinico Universitario A. Gemelli-IRCCS, Rome, Italy.
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.
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.
Authors: Hyeok Chan Kwon; Minyoung Kevin Kim; Jason Jungsik Song; Yong Beom Park; Sang Won Lee Journal: Yonsei Med J Date: 2020-08 Impact factor: 2.759