R. Krol, R. Klaasen, H. H. F. Remmelts, M. W. Heijstek, J. Spierings
2021 Annals of the Rheumatic Diseases  
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,
more » ... 005 – 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 selectionTable 1.Overview of studies on treatment for ENT-involvement.ENT-involvementAAV type per studyTherapyResultsLoEOtitis Media / Hearing lossOMAAV (n=6)RTXRemission 100%Mean hearing gain AC 22 dBBC 11 dB2bOMAAV (n=104)SteroidsResponse (hearing) 56%Relapse 47%4OMAAV (n=122)OMAAV (n=8)GPA (n=11)Steroids + csDMARDRelapse 36%Response (hearing) 68%Relapse 0%Response (hearing) 81%Response (hearing) 100%444Vestibular symptomsOMAAV (n=3)Steroids + CYCRemission (patient-reported) 100%4Subglottic stenosisGPA (n=9)GPA (n=15)DilatationRelapse 78%Mean number of procedures 44GPA (n=34)GPA (n=8)Intralesional steroids + dilatationMedian response 34 monthsResponse 88%Median response 12 monthsMean number of procedures 32b4GPA (n=18)Intralesional steroids + dilatation + resectionMean intervention-free interval 26 months2bGPA (n=5)Dilatation + resectionResponse 80%4GPA (n=13)GPA (n=11)SurgeryResponse (dyspnea) 100%Relapse 39%Higher QoL 77%Response (airway) 91%44GPA (n=6)Surgery or dilatation + intralesional steroids or csDMARDsRelapse 83.3%4GPA (n=8)RTXRemission 38%Response 50%2bSinonasal symptomsGPA (n=3)RTXResponse: 67%2bEGPA (n=29)EGPA (n=44)Steroids + csDMARDsRemission 82%Remission 21%Response 32%2b2bGPA (n=19)SurgeryResponse 76%4EGPA (n=65)MepolizumabRemission 28%1bENT symptoms NOSAAV (n=7)GPA (n=11)EGPA (n=61)RTXRelapse 22%Drop in daily steroid dosageDrop in daily steroid dosageResponse 93%Relapse 17%2b42bGPA (n=28)EGPA (n=15)Steroids + csDMARDsRemission 96%Relapse 42%Remission 86%44GPA (n=51)RTX (n=51)MTX92% of observational period in remission54%2bAC: 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
doi:10.1136/annrheumdis-2021-eular.678 fatcat:kqndwzlpk5bhtga45knfunbwg4