Systemic and Local Medical or Surgical Therapies for Ear, Nose and/or Throat Manifestations in ANCA-Associated Vasculitis: A Systematic Literature Review

Abstract

Background: Ear, nose and throat (ENT) manifestations are common in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), yet how to treat these manifestations remains controversial. Therefore, we systematically reviewed the literature on the efficacy of therapies on ENT manifestations in AAV. Methods: A systematic review was conducted in accordance with the PRISMA guidelines, searching Medline, Embase and Cochrane libraries, including clinical studies between January 2005 and January 2022, in adults with AAV and ENT involvement, reporting on the effects of local and systemic therapy. The critical appraisal was performed using tools provided by the Cochrane Library and the level of evidence (LoE) was scored according to the Oxford Centre for Evidence-based Medicine. Results: After screening 5609 identified studies, 136 full-text articles were assessed. Finally, 31 articles were included for critical appraisal and data-extraction. Nearly all studies (n = 29) were retrospective and scored low on LoE. The included studies evaluated local interventions (n = 11), glucocorticoids combined with conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs) (n = 8), rituximab (n = 6), or mepolizumab (n = 6). Due to heterogeneity across studies meta-analysis was not performed. Four studies on mepolizumab for sinonasal symptoms (n = 92) showed response in 33–100% and relapse in 35%. Local therapy for subglottic stenosis was effective in 80–100% of patients in 11 studies (n = 157), but relapses were common (up to 83%). In five studies, hearing improvement was observed in 56–100%, with better outcomes when glucocorticoids were combined with csDMARDs compared to glucocorticoids only. Conclusion: Response rates of ENT manifestations varied widely in studies and relapses were observed frequently. Heterogeneity among studies impaired comparison.

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

“…The focus on S. aureus results from the observation that approximately two-thirds of patients with GPA are chronic nasal carriers of this microbe [71]. S. aureus carriage is associated with an increased risk of AAV pulmonary exac-erbations, and trimethoprim-sulfamethoxazole treatment effectively reduces the frequency of non-severe relapses [73,74].…”

Ballon-Dilatation subglottischer Stenosen insbesondere bei Granulomatose mit Polyangiitis

“…Grundsätzlich stellt die Ballondilatation ein erfolgreiches und relativ schonendes Verfahren dar. Diese Erfahrungen gehen auch aus anderen Studien zur Ballondilatation hervor [18,19,20].…”

Medical Maintenance Therapy Following Laser Excision in Patients With Granulomatosis With Polyangiitis (GPA)‐Associated Subglottic Stenosis

“…6 Studies suggest that surgical intervention with endoscopic techniques and concurrent disease stabilization with systemic immunosuppressive therapy confers improved outcomes. 8,9 After the 2010 RAVE trial, rituximab emerged as a therapeutic option for inducing disease remission in systemic GPA and has since been utilized for both induction and maintenance therapy in some patients. 10 Treatment with rituximab appears to be less effective in otolaryngologic manifestations of GPA compared to other disease presentations; however, there remains a paucity of data concerning its therapeutic impact on SGS.…”

“…10 Treatment with rituximab appears to be less effective in otolaryngologic manifestations of GPA compared to other disease presentations; however, there remains a paucity of data concerning its therapeutic impact on SGS. 9,11 With our multidisciplinary experience treating patients with GPA-SGS, we aimed to evaluate the response to medical maintenance treatment with rituximab versus other immunosuppressive agents following endoscopic CO 2 laser excision at our institution.…”