In patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) who experience ear, nose, and/or throat (ENT) involvement, frequent relapses and variable response rates are reported, according to findings from a systematic literature review published in the Journal of Clinical Medicine.  

Although it is well known that ENT manifestations of disease occur often among individuals with AAV, the ways in which to treat these indicators remain debatable. The researchers conducted a systematic review of the literature on the efficacy of treatments for ENT manifestations in patients with AAV. The review was performed according to Preferred Reporting Items for Systematic Review and Meta-Analysis guidelines.

A research question was devised based on the systemic and local treatment of ENT symptoms. The Population, Intervention, Comparison, Outcome (PICO) method was used:

  • Population: AAV patients with ENT involvement
  • Interventions: local, surgical, and systemic therapies
  • Comparison: findings among the studies selected were compared; because heterogeneity among the studies weakened the comparisons, meta-analysis could not be performed
  • Outcomes: ENT activity, which was defined as disease activity, relapse, and damage

To be included in the analysis, the studies needed to evaluate local and systemic therapies in patients 18 years of age or older with AAV who exhibited ENT manifestations of disease. All clinical studies assessed for inclusion were published between January 2005 and January 2022. Exclusion criteria were as follows:

  • Studies that did not evaluate any treatments
  • Animal studies
  • Articles written in a language other than English
  • Articles in which no full-text script was available
  • Abstracts from a congress
  • Letters to editors
  • Guidelines and case reports that included less than 5 cases

Read more about antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis

The search retrieved a total of 5609 studies, of which 136 full-text articles were evaluated. Ultimately, 31 articles were included for data extraction and critical appraisal. The analysis of the studies was conducted using tools from the Cochrane Library. The level of evidence of the studies was scored based on the Oxford Centre for Evidence-Based Medicine.

In all, 29 of the 31 studies chosen were retrospective in design and scored low on the level of evidence. The following interventions were assessed in the 31 selected studies:

  • Local interventions: n=11
  • Glucocorticoids in combination with conventional systemic disease-modifying antirheumatic drugs (csDMARDs): n=8
  • Rituximab: n=6
  • Mepolizumab: n=6

Results showed that 4 studies on mepolizumab for sinonasal symptoms (n=92) demonstrated a response in 33% to 100% of participants, with relapses reported in 35%. In 11 of the studies evaluated (n=157), although local therapy for subglottic stenosis was effective in 80% to 100% of the participants, relapses were reported in up to 83% of patients.

In 5 of the studies, improvement in hearing was seen in 56% to 100% of participants, with better outcomes reported when glucocorticoids were combined with csDMARDs vs the use of glucocorticoid therapy alone.

“More controlled studies, specifically focusing on ENT involvement, are needed to better guide the management of ENT symptoms in patients with AAV,” the researchers concluded.


Krol RM, Remmelts HHF, Klaasen R, et al. Systemic and local medical or surgical therapies for ear, nose and/or throat manifestations in ANCA-associated vasculitis: a systematic literature review. J Clin Med. Published online April 28, 2023. doi:10.3390/jcm12093173