Kaitlin A Quinn1,2, Alexander Gelbard3, Cailin Sibley4, Arlene Sirajuddin5, Marcela A Ferrada2, Marcus Chen5, David Cuthbertson6, Simon Carette7, Nader A Khalidi8, Curry L Koening9, Carol A Langford10, Carol A McAlear11, Paul A Monach12, Larry W Moreland13, Christian Pagnoux7, Philip Seo14, Ulrich Specks15, Antoine G Sreih11, Steven R Ytterberg16, Peter A Merkel17, Peter C Grayson2. 1. Division of Rheumatology, MedStar Georgetown University Hospital, Washington, DC, USA. 2. Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD, USA. 3. Department of Otolaryngology - Head and Neck Surgery, Vanderbilt University, Nashville, TN, USA. 4. Division of Arthritis & Rheumatic Diseases, Oregon Health & Science University, Portland, OR, USA. 5. National Institutes of Health, NHLBI, Bethesda, MD, USA. 6. Biostatistics and Informatics, University of South Florida, Tampa, FL, USA. 7. Division of Rheumatology, Mount Sinai Hospital, Toronto, Canada. 8. Division of Rheumatology, McMaster University, Hamilton, ON, Canada. 9. Division of Rheumatology, University of Utah, Salt Lake City, UT, USA. 10. Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, USA. 11. Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, USA. 12. Division of Rheumatology, Brigham and Women's Hospital, Boston, MA, USA. 13. Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA, USA. 14. Division of Rheumatology, Johns Hopkins University, Baltimore, MD, USA. 15. Division of Pulmonary and Critical Care Medicine, MN, USA. 16. Division of Rheumatology, Mayo Clinic College of Medicine, Rochester, MN, USA. 17. Division of Rheumatology and Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, PA, USA.
Abstract
OBJECTIVES: To describe tracheobronchial disease in patients with granulomatosis with polyangiitis (GPA) and evaluate the utility of dynamic expiratory CT to detect large-airway disease. METHODS: Demographic and clinical features associated with the presence of subglottic stenosis (SGS) or endobronchial involvement were assessed in a multicentre, observational cohort of patients with GPA. A subset of patients with GPA from a single-centre cohort underwent dynamic chest CT to evaluate the airways. RESULTS: Among 962 patients with GPA, SGS and endobronchial disease were identified in 95 (10%) and 59 (6%) patients, respectively. Patients with SGS were more likely to be female (72% vs 53%, P < 0.01), younger at time of diagnosis (36 vs 49 years, P < 0.01), and have saddle-nose deformities (28% vs 10%, P < 0.01), but were less likely to have renal involvement (39% vs 62%, P < 0.01). Patients with endobronchial disease were more likely to be PR3-ANCA positive (85% vs 66%, P < 0.01), with more ENT involvement (97% vs 77%, P < 0.01) and less renal involvement (42% vs 62%, P < 0.01). Disease activity in patients with large-airway disease was commonly isolated to the subglottis/upper airway (57%) or bronchi (32%). Seven of 23 patients screened by dynamic chest CT had large-airway pathology, including four patients with chronic, unexplained cough, discovered to have tracheobronchomalacia. CONCLUSION: SGS and endobronchial disease occur in 10% and 6% of patients with GPA, respectively, and may occur without disease activity in other organs. Dynamic expiratory chest CT is a potential non-invasive screening test for large-airway involvement in GPA.
OBJECTIVES: To describe tracheobronchial disease in patients with granulomatosis with polyangiitis (GPA) and evaluate the utility of dynamic expiratory CT to detect large-airway disease. METHODS: Demographic and clinical features associated with the presence of subglottic stenosis (SGS) or endobronchial involvement were assessed in a multicentre, observational cohort of patients with GPA. A subset of patients with GPA from a single-centre cohort underwent dynamic chest CT to evaluate the airways. RESULTS: Among 962 patients with GPA, SGS and endobronchial disease were identified in 95 (10%) and 59 (6%) patients, respectively. Patients with SGS were more likely to be female (72% vs 53%, P < 0.01), younger at time of diagnosis (36 vs 49 years, P < 0.01), and have saddle-nose deformities (28% vs 10%, P < 0.01), but were less likely to have renal involvement (39% vs 62%, P < 0.01). Patients with endobronchial disease were more likely to be PR3-ANCA positive (85% vs 66%, P < 0.01), with more ENT involvement (97% vs 77%, P < 0.01) and less renal involvement (42% vs 62%, P < 0.01). Disease activity in patients with large-airway disease was commonly isolated to the subglottis/upper airway (57%) or bronchi (32%). Seven of 23 patients screened by dynamic chest CT had large-airway pathology, including four patients with chronic, unexplained cough, discovered to have tracheobronchomalacia. CONCLUSION:SGS and endobronchial disease occur in 10% and 6% of patients with GPA, respectively, and may occur without disease activity in other organs. Dynamic expiratory chest CT is a potential non-invasive screening test for large-airway involvement in GPA.
Authors: Lena W Chen; Ioan Lina; Kevin Motz; Alexandra J Berges; Rafael Ospino; Philip Seo; Alexander T Hillel Journal: Otolaryngol Head Neck Surg Date: 2021-04-13 Impact factor: 3.497
Authors: Fernando Guedes; Mariana V Branquinho; Ana C Sousa; Rui D Alvites; António Bugalho; Ana Colette Maurício Journal: BMC Pulm Med Date: 2022-02-19 Impact factor: 3.317