Granulomatosis with polyangiitis: endoscopic management of tracheobronchial stenosis: results from a multicentre experience

Abstract

TBS represents severe and refractory manifestations with a high rate of restenosis. High-dose systemic CSs at the time of the procedure and increased time from GPA diagnosis to bronchoscopic intervention are associated with a better event-free survival. In contrast, bronchial stenoses are associated with a higher rate of restenosis than subglottic stenosis.

Accuracy of Magnetic Resonance Imaging for Grading of Subglottic Stenosis in Patients With Granulomatosis With Polyangiitis: Correlation With Pulmonary Function Tests and Laryngoscopy

“…There were 14 men (32%) and 30 women (68%). Epidemiologic data of this cohort confirm that GPA patients with SGS tend to be younger and more often female .…”

“…Depending on the severity of clinical symptoms, apart from conventional drug therapy, various therapeutic dilative interventional or surgical procedures have been proposed for managing SGS in patients with GPA . Repeated dilative procedures are often required, as the degree of SGS worsens in the course of the disease . Therefore, close and life‐long clinical monitoring of the presence and severity of symptoms is necessary in patients with SGS.…”

Myeloperoxidase–Antineutrophil Cytoplasmic Antibody (ANCA)–Positive Granulomatosis With Polyangiitis (Wegener's) Is a Clinically Distinct Subset of ANCA‐Associated Vasculitis: A Retrospective Analysis of 315 Patients From a German Vasculitis Referral Center

“…The observed rate of 28.8% was significantly higher than that in the PR3‐ANCA–positive GPA cohort (11.0%) and was 2–3 times as high as frequencies reported in GPA by other authors (8.5%–16%) , including a previous study from our center (12%) . It has previously been reported that subglottic stenosis predominantly affects female GPA patients and patients of young age and that rates of renal involvement are lower in GPA patients with subglottic stenosis , which is consistent with our findings.…”

Granulomatose com poliangiite

“…Ciclofosfamida (CYC) modificou a história natural da GPA com melhora na sobrevida e na resposta ao tratamento 1,4,22,23 . É indicada na fase de indução na doença generalizada ou com risco de dano permanente a órgãos ou risco de morte.…”