Jean-Paul Makhzoum1, Peter C Grayson2, Cristina Ponte3,4, Joanna Robson5,6, Ravi Suppiah7, Richard A Watts8,9, Raashid Luqmani8, Peter A Merkel10, Christian Pagnoux11. 1. Vasculitis Clinic, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, University of Montreal, Montreal, QC, Canada. 2. Systemic Autoimmunity Branch, NIAMS, National Institutes of Health, Bethesda, MD, USA. 3. Hospital de Santa Maria, Department of Rheumatology, Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal. 4. Rheumatology Research Unit, Instituto de Medicina Molecular, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Faculdade de Medicina, Lisbon, Portugal. 5. Academic Rheumatology Unit, Bristol Royal Infirmary, Faculty of Health and Applied Sciences, University of the West of England, Bristol, UK. 6. School of Clinical Sciences, University of Bristol and Department of Rheumatology, University Hospitals Bristol NHS Trust, Bristol, UK. 7. Department of Rheumatology, Auckland District Health Board, Auckland, New Zealand. 8. Oxford NIHR Biomedical Research Centre, Nuffield Department of Orthopaedics Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK. 9. Department of Medicine, Norwich Medical School, University of East Anglia, Norwich, UK. 10. Division of Rheumatology, Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, PA, USA. 11. Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada.
Abstract
OBJECTIVES: This study describes the spectrum and initial impact of pulmonary manifestations in the primary systemic vasculitides. METHODS: Description and comparison of pulmonary manifestations in adults with Takayasu's arteritis (TAK), GCA, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA), polyarteritis nodosa (PAN) and IgA vasculitis (IgAV), using data collected within the Diagnostic and Classification Criteria in Vasculitis study. RESULTS: Data from 1952 patients with primary vasculitides were included: 170 TAK, 657 GCA, 555 GPA, 223 MPA, 146 EGPA, 153 IgAV and 48 PAN. Pulmonary manifestations were observed in patients with TAK (21.8%), GCA (15.8%), GPA (64.5%), MPA (65.9%), EGPA (89.0%), PAN (27.1%) and IgAV (5.9%). Dyspnoea occurred in patients with TAK (14.7%), GCA (7.8%), GPA (41.8%), MPA (43.5%), EGPA (65.8%), PAN (18.8%) and IgAV (2.6%). Cough was reported in TAK (7.6%), GCA (9.3%), GPA (34.8%), MPA (37.7%), EGPA (55.5%), PAN (16.7%) and IgAV (3.3%). Haemoptysis occurred mainly in patients with ANCA-associated vasculitis (AAV). Fibrosis on imaging at diagnosis was documented in GPA (1.9%), MPA (24.9%) and EGPA (6.3%). Only patients with AAV (GPA 2.7%, MPA 2.7% and EGPA 3.4%) required mechanical ventilation. At 6 months, the presence of at least one pulmonary item in the Vasculitis Damage Index was observed in TAK (4.1%), GCA (3.3%), GPA (15.4%), MPA (28.7%), EGPA (52.7%), PAN (6.2%) and IgAV (1.3%). CONCLUSION: Pulmonary manifestations can occur in all primary systemic vasculitides, but are more frequent and more often associated with permanent damage in AAV.
OBJECTIVES: This study describes the spectrum and initial impact of pulmonary manifestations in the primary systemic vasculitides. METHODS: Description and comparison of pulmonary manifestations in adults with Takayasu's arteritis (TAK), GCA, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA), polyarteritis nodosa (PAN) and IgA vasculitis (IgAV), using data collected within the Diagnostic and Classification Criteria in Vasculitis study. RESULTS: Data from 1952 patients with primary vasculitides were included: 170 TAK, 657 GCA, 555 GPA, 223 MPA, 146 EGPA, 153 IgAV and 48 PAN. Pulmonary manifestations were observed in patients with TAK (21.8%), GCA (15.8%), GPA (64.5%), MPA (65.9%), EGPA (89.0%), PAN (27.1%) and IgAV (5.9%). Dyspnoea occurred in patients with TAK (14.7%), GCA (7.8%), GPA (41.8%), MPA (43.5%), EGPA (65.8%), PAN (18.8%) and IgAV (2.6%). Cough was reported in TAK (7.6%), GCA (9.3%), GPA (34.8%), MPA (37.7%), EGPA (55.5%), PAN (16.7%) and IgAV (3.3%). Haemoptysis occurred mainly in patients with ANCA-associated vasculitis (AAV). Fibrosis on imaging at diagnosis was documented in GPA (1.9%), MPA (24.9%) and EGPA (6.3%). Only patients with AAV (GPA 2.7%, MPA 2.7% and EGPA 3.4%) required mechanical ventilation. At 6 months, the presence of at least one pulmonary item in the Vasculitis Damage Index was observed in TAK (4.1%), GCA (3.3%), GPA (15.4%), MPA (28.7%), EGPA (52.7%), PAN (6.2%) and IgAV (1.3%). CONCLUSION: Pulmonary manifestations can occur in all primary systemic vasculitides, but are more frequent and more often associated with permanent damage in AAV.
Authors: Julien Demiselle; Johann Auchabie; François Beloncle; Philippe Gatault; Steven Grangé; Damien Du Cheyron; Jean Dellamonica; Sonia Boyer; Dimitri Titeca Beauport; Lise Piquilloud; Julien Letheulle; Christophe Guitton; Nicolas Chudeau; Guillaume Geri; François Fourrier; René Robert; Emmanuel Guérot; Julie Boisramé-Helms; Pierre Galichon; Pierre-François Dequin; Alexandre Lautrette; Pierre-Edouard Bollaert; Ferhat Meziani; Loïc Guillevin; Nicolas Lerolle; Jean-François Augusto Journal: Ann Intensive Care Date: 2017-04-05 Impact factor: 6.925
Authors: Edoardo Conticini; Miriana d'Alessandro; Laura Bergantini; Diego Castillo; Paolo Cameli; Bruno Frediani; Luca Cantarini; Elena Bargagli Journal: Biology (Basel) Date: 2022-01-08