Background In the management of Granulomatosis with Polyangiitis (GPA), is critical to assess correctly the disease activity in each organ to define the better therapeutic approach and follow up strategy. This statement is also valid for ENT involvement, that we decide to assess with two different organ specific disease activity scores, the already validated Del Pero ENT/GPA DAS1 and a new ENT score developed by our Otolaryngology Unit (Padova,ENT/GPA DAS), composed by 8 items: nasal crusting, reported bloody rhinorrhea, nasal inflammation, laryngoscopy inflammation, objective stridor, inflamed TM/middle ear, siero-mucous otitis and hearing loss.
Objectives Compare the accuracy of two different ENT scores (ENT/GPA DAS and Padova ENT/GPA DAS) and correlate them with the most commonly used GPA Activity Indexes and identify if they are effective to predict relapses that require therapeutic changes.
Methods We selected retrospectively the GPA patients with ENT involvement followed by Rheumatology and Otolaryngology Units from 2006 to 2015. Each ENT evaluation of our patients were matched with temporally nearest Rheumatologic visit to assess the ENT/GPA DAS, the Padova ENT/GPA DAS, the Birmingham Vasculitis Activity Scores (BVAS v3, BVAS-WG), the Physician Global Assessment (PGA), the Vasculatis Damage Index (VDI), the serological biomarkers (ESR and CRP) and treatment strategy.
For statistical analysis, we used Mann Whitney test, Spearman test and Fisher test.
Results The selected ENT/GPA patients were 25 (41.6% of all GPA patient cohort), mainly middle aged (51.7±14.9 years), women (64%) and ANCA positive (80%) with PR3 specificity (75%). The total of ENT assessment were 67, so some patient were seen more than once (mean 2.68, min 1, max 8). The two considered ENT scores presented a significant difference (p<0.0004) from each other and the correlation analysis showed that ENT/GPA DAS was directly correlated to ESR (p<0.0001), CRP (p=0.03), BVAS v3 (p=0.005), BVAS-WG (p=0.004) and PGA (p<0.0001), while our Padova ENT/GPA DAS presented a significant correlation only with VES (p<0.0001) and PGA (p=0.027).
The therapeutic strategies analysis observed that, in all cohort, the change of the immunosuppressive drug (32/67) presented higher BVAS v3 (p<0.0001), BVAS-WG (p<0.0001) and PGA (p<0.0001). Furthermore, in follow up visits of patient in maintenance therapy or induction of remission therapy, excluded those with Cyclophosphamide or Rituximab (25/44), we observed a significant increased rate of therapeutic change in those with ENT/GPA DAS higher than 0 (p=0.025, OR 5.8 IC95% 1.16–24.9)
Conclusions Our analysis showed that ENT/GPA DAS is more performing than Padova ENT/GPA DAS to reflect the disease activity. This is probably a consequence that some items considered in our ENT/GPA DAS, such as all type of hearing loss, express better the damage than active disease. Moreover, the data showed that ENT/GPA DAS could identify patients with an active disease worthy of therapeutic changes, especially in particular patient subsets. This data should be confirmed with further evidences on larger GPA patients cohorts and prospective studies.
Del Pero MM et al Laryngoscope, 123:622–628, 2013.
Disclosure of Interest None declared