TY - JOUR
T1 - Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis
AU - Quinn, Kaitlin A.
AU - Gelbard, Alexander
AU - Sibley, Cailin
AU - Sirajuddin, Arlene
AU - Ferrada, Marcela A.
AU - Chen, Marcus
AU - Cuthbertson, David
AU - Carette, Simon
AU - Khalidi, Nader A.
AU - Koening, Curry L.
AU - Langford, Carol A.
AU - Mcalear, Carol A.
AU - Monach, Paul A.
AU - Moreland, Larry W.
AU - Pagnoux, Christian
AU - Seo, Philip
AU - Specks, Ulrich
AU - Sreih, Antoine G.
AU - Ytterberg, Steven R.
AU - Merkel, Peter A.
AU - Grayson, Peter C.
N1 - Funding Information:
This work was supported by the Vasculitis Clinical Research Consortium (VCRC) and the Intramural Research Program at the National Institute of Arthritis and Musculoskeletal and Skin Diseases. The Vasculitis Clinical Research Consortium (VCRC) is part of the Rare Diseases Clinical Research Network (RDCRN), an initiative of the Office of Rare Diseases Research (ORDR), National Center for Advancing
Funding Information:
Translational Science (NCATS). The VCRC is funded through collaboration between NCATS, and the National Institute of Arthritis and Musculoskeletal and Skin Diseases (U54 AR057319) and received funding from the National Center for Research Resources (U54 RR019497). KQ received funding from a Vasculitis Clinical Research Consortium (VCRC)/Vasculitis Foundation (VF) Fellowship.
Publisher Copyright:
© 2019 The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.
PY - 2019/12/1
Y1 - 2019/12/1
N2 - 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.
AB - 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.
KW - ANCA-associated vasculitis
KW - granulomatosis with polyangiitis
KW - subglottic stenosis
KW - tracheobronchomalacia
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U2 - 10.1093/rheumatology/kez217
DO - 10.1093/rheumatology/kez217
M3 - Article
C2 - 31199488
AN - SCOPUS:85075813212
VL - 58
SP - 2203
EP - 2211
JO - Rheumatology and Rehabilitation
JF - Rheumatology and Rehabilitation
SN - 1462-0324
IS - 12
ER -