Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis

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.

Keywords: ANCA-associated vasculitis; granulomatosis with polyangiitis; subglottic stenosis; tracheobronchomalacia.

Fig. 1

Organ involvement in patients with active subglottic disease There were 87 patients with known GPA who were evaluated during time of active subglottic inflammation. Patients were divided on the basis of isolated upper airway disease or additional disease outside of the upper airway. Additional features of each group are displayed. GPA: granulomatosis with polyangiitis.

Fig. 2

Organ involvement in patients with active endobronchial disease There were 53 patients evaluated during time of active endobronchial disease. Patients were divided on the basis of isolated endobronchial disease and those who had additional disease features.

Fig. 3

Dynamic expiratory CT chest in a patient with tracheobronchomalacia A 67-year-old male with GPA underwent dynamic expiratory CT. He had a 7-year history of GPA diagnosed on the basis of presence of PR3-ANCA, sinusitis, arthralgias, constitutional symptoms, and diffuse alveolar haemorrhage. He was in long-term clinical remission on repeated courses of rituximab, with persistent, unexplained dry cough. Dynamic CT chest showed normal appearance of trachea on inspiration (A) and tracheal collapse of anterior and lateral tracheal walls (62%) on expiration (B) consistent with tracheobronchomalacia. GPA: granulomatosis with polyangiitis.