Background Localized disease manifestations including inflammatory subglottic stenosis in GPA have been associated with a refractory disease course in case series and small cohorts [1, 2].
Objectives To identify and characterize GPA patients with inflammatory subglottic stenosis (SST) and/or tracheobronchitis (TB) retrospectively within our monocentric cohort of 1184 GPA patients followed from January 1990 to August 2011 with respect to disease stage, disease manifestations, course of disease, treatment (including interventional procedures) and outcome.
Methods All patients who fulfilled the American College of Rheumatology criteria or the Chapel Hill Consensus Conference Definition (n=70) or the EMEA algorithm for GPA (n=20)  or had localised GPA  and developed SST and/or TB were included. Patients were assessed in 4-6 monthly intervals by a standardized interdisciplinary setting  with respect to SST and/or TB (including ENT opinion and/or bronchoscopy and/or high-resolution CT), all other clinical manifestations, disease stages and activity, ANCA-status, duration and intensity of immunosuppressive treatment, interventional procedures, treatment effect, relapse rate, chronic organ damage and side effects of treatment.
Results 1184 GPA patients were seen during the follow-up period, 90 of whom (7.6%; 94.4% ANCA positive, 62.2% female) developed SST and/or TB and had a follow-up of >6 months (median follow-up: 56 months, range: 6-262 months). 33 (36.7%) patients were under immunosuppression when SST/TB occurred; 58 (64.4%) received cyclophosphamide and glucocorticoids for remission induction (47.2% for SST/TB, 52.8% for other disease manifestations), 18 (20%) medium-potent immunosuppression. 78 (86.7%) achieved response/remission, 12 (13.3%) were refractory. Interventional procedures during remission induction were required in 30 patients (33.3%) including tracheostomas in 12 patients. 40 patients (44.4%) developed chronic SST and 19 (21.1%) developed chronic bronchial stenosis, 35 (38.9%) of whom required interventional procedures for scarring. 29 patients (32.2%) had 1-4 relapses.
Conclusions ST/TB is a less common disease manifestation (7.6%) which was treated successfully by immunosuppression in the majority of cases (86.7%) but frequently recurred. SST/TB is associated with a high burden of disease and damage (44.4% chronic SST, 21.1% chronic bronchial stenosis) requiring interventional procedures in 35 patients (38.9%) to secure airways during active disease or to treat scarring processes during remission.
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Holle JU, Gross WL, Holl-Ulrich K, Ambrosch P, Noelle B, Both M et al. Prospective long-term follow-up of patients with localized Wegener’s granulomatosis: does it occur as persistent disease stage? Ann Rheum Dis 2010; 69: 1934-1039.
Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66: 222-227.
Disclosure of Interest None Declared
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