AB0387 SYSTEMIC AND LOCAL THERAPIES FOR EAR, NOSE AND THROAT MANIFESTATIONS IN ANCA-ASSOCIATED VASCULITIS: A SYSTEMATIC REVIEW
Authors: R. Krol, R. Klaasen, H. H. F. Remmelts, M. W. Heijstek, J. Spierings
Journal: Annals of the Rheumatic Diseases (2021)

Background: Ear, nose and throat (ENT)-involvement is common in antineutrophil cytoplasmic autoantibody- (ANCA-)associated vasculitis (AAV), can lead to permanent damage and is correlated with reduced quality of life. Yet, guidelines on management of ENT-manifestations are lacking. Objectives: The aim of this study was to evaluate the efficacy of systemic and local therapies for ENT- involvement in AAV. Methods: A systematic review was conducted. Clinical studies between January 2005 – April 2020, in adults with AAV and ENT-involvement using outcomes reflecting disease activity, relapse and damage were included. Case reports, congress abstracts and non-English articles were excluded. Results: The search yielded 4,586 results. For full-text assessment, 109 articles were selected. Finally, 25 articles were included (Figure 1). Two placebo-controlled trials were available. The other observational studies had a limited level of evidence (LoE) of 2b-4 according to the Oxford Centre for Evidence-based Medicine. Ten studies (n=127) with subglottic stenosis evaluated dilatation (n=94), intralesional steroids (n=63), surgery (n=51) or rituximab (n=8), which mostly resulted in temporary response (12-34 months) (Table 1). Otologic manifestations were treated with various disease modifying anti-rheumatic drugs (DMARDs) in 251 patients in six studies. All studies demonstrated improvement in hearing thresholds, but relapse rates were high (47%). Five studies assessed DMARDs or surgery for sinonasal symptoms showing remission in 21-82%. Six studies, which did not specify ENT-symptoms of the included patients, reported remission in 86-96%. Due to heterogeneity across studies no meta-analysis could be performed. Figure 1. Flowchart of article selection Table 1. Overview of studies on treatment for ENT-involvement. ENT-involvement AAV type per study Therapy Results LoE Otitis Media / Hearing loss OMAAV (n=6) RTX Remission 100% Mean hearing gain AC 22 dB BC 11 dB 2b OMAAV (n=104) Steroids Response (hearing) 56% Relapse 47% 4 OMAAV (n=122) OMAAV (n=8) GPA (n=11) Steroids + csDMARD Relapse 36% Response (hearing) 68% Relapse 0% Response (hearing) 81% Response (hearing) 100% 4 4 4 Vestibular symptoms OMAAV (n=3) Steroids + CYC Remission (patient-reported) 100% 4 Subglottic stenosis GPA (n=9) GPA (n=15) Dilatation Relapse 78% Mean number of procedures 4 4 GPA (n=34) GPA (n=8) Intralesional steroids + dilatation Median response 34 months Response 88% Median response 12 months Mean number of procedures 3 2b 4 GPA (n=18) Intralesional steroids + dilatation + resection Mean intervention-free interval 26 months 2b GPA (n=5) Dilatation + resection Response 80% 4 GPA (n=13) GPA (n=11) Surgery Response (dyspnea) 100% Relapse 39% Higher QoL 77% Response (airway) 91% 4 4 GPA (n=6) Surgery or dilatation + intralesional steroids or csDMARDs Relapse 83.3% 4 GPA (n=8) RTX Remission 38% Response 50% 2b Sinonasal symptoms GPA (n=3) RTX Response: 67% 2b EGPA (n=29) EGPA (n=44) Steroids + csDMARDs Remission 82% Remission 21% Response 32% 2b 2b GPA (n=19) Surgery Response 76% 4 EGPA (n=65) Mepolizumab Remission 28% 1b ENT symptoms NOS AAV (n=7) GPA (n=11)EGPA (n=61) RTX Relapse 22% Drop in daily steroid dosage Drop in daily steroid dosage Response 93% Relapse 17% 2b 4 2b GPA (n=28) EGPA (n=15) Steroids + csDMARDs Remission 96% Relapse 42% Remission 86% 4 4 GPA (n=51) RTX (n=51) MTX 92% of observational period in remission 54% 2b AC: air-conduction, BC: bone-conduction, csDMARD: conventional synthetic disease modifying antirheumatic drug, CYC: cyclophosphamide, dB: decibel, EGPA: eosinophilic GPA, GPA: granulomatosis with polyangiitis, MTX: methotrexate, NOS: not otherwise specified, OMAAV: otitis media associated ANCA-vasculitis, QoL: quality of life, RTX: rituximab. Conclusion: On the treatment of ENT manifestations in AAV 25 studies were available, mainly with small patient numbers and low LoE (1b-4). Heterogeneity in treatments and outcome measures impaired comparison between studies. More placebo-controlled studies are needed to guide treatment. Disclosure of Interests: None declared