Assessing vasculitis in Takayasu arteritis by ultrasound: results of OMERACT patient-based reliability exercises

Q: What is the clinical evidence from this study?

Study design: Cross-Sectional. Population: Takayasu arteritis (TAK) (n=10). Intervention: Standardised vascular ultrasound assessment using OMERACT consensus-based definitions of elementary lesions ('macaroni sign', stenosis, occlusion) vs. Healthy control subject. Primary outcome: Inter-rater and intrarater reliability (Fleiss' and Cohen's kappa) of consensus-based definitions of elementary ultrasound lesions ('macaroni sign', stenosis, occlusion) (Fleiss' κ 0.51-0.74 for macaroni sign; Cohen's κ 0.74-0.81 for macaroni sign, 95% CI 0.70-0.78).

Q: What does this research mean for the field?

In Takayasu arteritis, vascular ultrasound assessment of the 'macaroni sign' demonstrates moderate to good inter-rater and good to excellent intrarater reliability, whereas detection of stenosis and occlusion exhibits lower and more variable reproducibility. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Michele di Lernia; Alessandro Tomelleri; Wolfgang Schmidt; Milena Bond; Cristina Ponte; Péter Mandl; Stavros Chrysidis; Eugenio de Miguel; Andreas P. Diamantopoulos; Petra Hánová; Alojzija Hočevar; Durga Prasanna Misra; Sara M. Monti; Berit Dalsgaard Nielsen; Niccolò Possemato; Valentin Sebastian Schäfer; Luca Seitz; Pascal Seitz; Markus Aschwanden; Davide Vignale; Antonio Esposito; Gerlig Widmann; Maria Antonietta D’Agostino; Georgios Filippou; Carlos Pineda; Christina Duftner; Christian Dejaco

doi:10.1136/rmdopen-2026-006884

Key result

Standardized vascular ultrasound demonstrates moderate to good reliability for the macaroni sign in Takayasu arteritis.

Fleiss' κ 0.51-0.74 for macaroni sign; Cohen's κ 0.74-0.81 for macaroni sign
95% CI 0.70-0.78
n=10

Why the study?

The inter-rater and intrarater reliability of consensus-based definitions of elementary ultrasound lesions in Takayasu arteritis needed to be evaluated in live patient-based reliability exercises to establish the methodological basis for a new global OMERACT ultrasound score.

Does standardised vascular ultrasound using OMERACT consensus-based definitions demonstrate reliable inter-rater and intrarater agreement for elementary vascular lesions ('macaroni sign', stenosis, and occlusion) in patients with Takayasu arteritis?

Key Points

To assess the inter-rater and intrarater reliability of consensus-based definitions for elementary ultrasound lesions in Takayasu arteritis using live patient-based exercises.
Conducted two live patient-based exercises with two rounds each, involving 12 vascular sonographers.
Sonographers independently examined five patients with Takayasu arteritis and one control subject across the bilateral common carotid, subclavian, and axillary arteries, as well as the abdominal aorta.
Evaluated the 'macaroni sign', stenosis, and occlusion using Fleiss' and Cohen's kappa (κ) statistics to calculate inter-rater and intrarater reliability.
Inter-rater reliability across both rounds was moderate to good for the 'macaroni sign' (κ 0.51–0.74), fair for stenosis (κ 0.24–0.38), and variable for occlusion (κ 0.02–0.60).
Intrarater reliability across exercises was good to excellent for the 'macaroni sign' (κ 0.74–0.81) and moderate for stenosis (κ 0.55–0.64) and occlusion (κ 0.64–1.00, based on limited assessments).
Reliability for the 'macaroni sign' was highest in the carotid arteries, while agreement was lower across other vessels, other lesion types, and the abdominal aorta.

Study Design

Type

Cross-Sectional (n=10)

Blinding

Examiners were blinded to diagnosis (TAK vs control), all clinical data and the presence or absence of lesions in each arterial segment

Multicenter

No

Structured PICO

Does standardised vascular ultrasound using OMERACT consensus-based definitions demonstrate reliable inter-rater and intrarater agreement for elementary vascular lesions ('macaroni sign', stenosis, and occlusion) in patients with Takayasu arteritis?

P

Population

Two live reliability exercises each involving 5 patients with Takayasu arteritis (2 overlapping across exercises) and 1 healthy control examined by 12 blinded vascular sonographers in Austria.

E

Exposure

Standardised live vascular ultrasound assessment of seven predefined arterial segments (bilateral common carotid, subclavian, and axillary arteries, and the distal abdominal aorta) in longitudinal and transverse scans (allocated 18 minutes scanning per subject across two rounds per exercise), preceded by theoretical training and hands-on calibration sessions (2.5 hours in Exercise 1; expanded to 4 hours in Exercise 2).

O

Outcome

Inter-rater reliability (Fleiss' kappa, κ) and intrarater reliability (Cohen's κ) for consensus-based definitions of three elementary ultrasound lesions in Takayasu arteritis ('macaroni sign', stenosis, and occlusion) overall and by individual vascular territory.surrogate

Main Result

Effect estimate: Fleiss' κ 0.51-0.74 for macaroni sign; Cohen's κ 0.74-0.81 for macaroni sign (95% CI 0.70-0.78)

In live patient-based vascular ultrasound exercises for Takayasu arteritis, the 'macaroni sign' (vessel wall thickening) demonstrated moderate-to-good inter-rater and good-to-excellent intrarater reliability—especially in the carotid and axillary arteries—providing the methodological basis for a standardised OMERACT composite ultrasound score.

Limitations

Small sample size and relatively homogeneous ethnicity, limiting generalisability
Low prevalence of advanced luminal lesions, particularly occlusions, limiting the number of positive findings available for evaluation
Lack of a convex probe for the evaluation of the abdominal aorta, potentially limiting image quality and lesion assessment
The definition of the 'macaroni sign' remains partly qualitative, which may limit its reproducibility
Only one patient had clinically active disease at the time of assessment, and the cross-sectional design cannot distinguish acute inflammation from chronic wall remodelling
Small sample size and relatively homogeneous ethnicity, which may limit generalisability
Low prevalence of advanced luminal lesions, particularly occlusions, limiting the number of positive findings available for evaluation and affecting the robustness of kappa values
Did not apply a convex probe for the evaluation of the abdominal aorta, potentially limiting image quality and lesion assessment in some patients
The consensus definition of the 'macaroni sign' remains partly qualitative, which may limit its reproducibility
Only one patient had clinically active disease at the time of assessment, and the cross-sectional design does not allow distinguishing acute inflammation from chronic wall remodelling

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Implication

Macaroni sign shows moderate-good reliability in TAK ultrasound; extends OMERACT framework but leaves open clinical adoption.

Abstract

Objective To evaluate the inter-rater and intrarater reliability of consensus-based definitions of elementary ultrasound lesions in Takayasu arteritis (TAK) in live patient-based reliability exercises.Methods Two live exercises with two rounds each were conducted, each involving 12 vascular sonographers who independently examined five patients with TAK and one control subject. Bilateral common carotid, subclavian and axillary arteries and the abdominal aorta were investigated to identify key elementary ultrasound lesions in TAK: ‘macaroni sign’, stenosis and occlusion. Inter-rater and intrarater reliability were calculated using Fleiss’ and Cohen’s kappa (κ), respectively.Results Overall, inter-rater reliability across the two rounds of both exercises was moderate to good for the ‘macaroni sign’ (κ 0.51-0.74 across the two rounds of both exercises), fair for stenosis (0.24–0.38) and variable for occlusion (0.02–0.60). Intrarater reliability across the two exercises was good to excellent for the ‘macaroni sign’ (0.74–0.81) and moderate for stenosis (0.55–0.64) and occlusion (0.64–1.00; the latter based on a limited number of assessments). In both exercises, inter-rater reliability was good to excellent for the ‘macaroni sign’ in carotids but worse across other vessels and lesion types; intrarater reliability was good to excellent when analysed per single vessel and lesion though limited for occlusions and for the abdominal aorta.Conclusion Among the ultrasound elementary lesions for TAK, the ‘macaroni sign’ revealed moderate to good reliability, while the reproducibility of stenosis and occlusion was worse and more variable. These results provide the methodological basis to develop a new global Outcome Measures in Rheumatology (OMERACT) ultrasound score in TAK.

Discussion

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Cite This Study

Lernia et al. (2026) conducted a cross-sectional in Takayasu arteritis (TAK) (n=10). Standardised vascular ultrasound assessment using OMERACT consensus-based definitions of elementary lesions ('macaroni sign', stenosis, occlusion) vs. Healthy control subject was evaluated on Inter-rater and intrarater reliability (Fleiss' and Cohen's kappa) of consensus-based definitions of elementary ultrasound lesions ('macaroni sign', stenosis, occlusion) (Fleiss' κ 0.51-0.74 for macaroni sign; Cohen's κ 0.74-0.81 for macaroni sign, 95% CI 0.70-0.78). Standardised vascular ultrasound demonstrated moderate to good inter-rater reliability (κ 0.51–0.74) and good to excellent intrarater reliability (κ 0.74–0.81) for the 'macaroni sign' in Takayasu arteritis.

synapsesocial.com/papers/6af0dba03c845ca7837ceddd https://doi.org/10.1136/rmdopen-2026-006884

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Assessing vasculitis in Takayasu arteritis by ultrasound: results of OMERACT patient-based reliability exercises

Key Points

Study Design

Structured PICO

Main Result

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

Assessing vasculitis in Takayasu arteritis by ultrasound: results of OMERACT patient-based reliability exercises

Key Points

Study Design

Structured PICO

Main Result

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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