Objectives: Ears nose and throat (ENT) involvement is found on a substantial proportion of patients with granulomatosis with polyangiitis (GPA). Structured, reliable ENT assessment is essential in the management of GPA patients. It is the aim of this study to determine the repeatability (intra-rater reliability) and reproducibility (inter-rater reliability) of the ENT Assessment Score (ENTAS 2).

Methods: The ENTAS 2 built the fundament of the prospective randomized trial. Anamnestic, video endoscopic and diagnostic data of 47 patients were used. A single assessor reference was created. GPA/ENT activity and damage were evaluated by three physicians at two time points (T1/T2). GPA/ENT activity was evaluated in dichotomy (yes/no) and grading (none/mild/moderate/high) and GPA/ ENT damage in dichotomy.

Results: ENTAS 2 activity evaluations intra-rater reliability was 80.7% (κ=0.56) in dichotomy and 72.8% (κ=0.41) in grading. ENTAS 2 damage evaluations showed 87.8% (κ=0.74) intra-rater reliability. ENTAS 2 activity inter-rater reliability at T1 was 62.2% (κ=0.43) in dichotomy and 51.1% (κ=0.29) in grading, at T2 it was 68.2% (κ=0.48) in dichotomy and 55.32% (κ=0.33) in grading. Inter-rater reliability of ENTAS 2 damage evaluation was 84.4% (κ=0.79) at T1 and 72.5% (κ=0.64) at T2.

Conclusions: ENTAS 2 intra-rater reliability was high in dichotomous and graded GPA/ENT activity and damage evaluations. Inter-rater reliability was high in dichotomous activity and damage evaluations, but low in graded activity evaluations. The data demonstrate that the ENTAS 2 is a reliable score-system considering GPA/ENT activity and damage evaluations.

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