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Reliability and Construct Validity of the Physician's Global Assessment of Lung Disease in Systemic Juvenile Idiopathic Arthritis–Associated Lung Disease
Journal article   Open access   Peer reviewed

Reliability and Construct Validity of the Physician's Global Assessment of Lung Disease in Systemic Juvenile Idiopathic Arthritis–Associated Lung Disease

Eileen Rife, Guihua Zhai, Mekibib Altaye, Jennifer Andringa, Hermine I. Brunner, Scott Canna, Lauren A. Henderson, Yukiko Kimura, Scott M. Lieberman, Mona Riskalla, …
ACR open rheumatology, Vol.8(3), e90006
03/2026
DOI: 10.1002/acr2.90006
PMID: 41797235
url
https://doi.org/10.1002/acr2.90006View
Published (Version of record) Open Access

Abstract

Objective The physician global assessment of lung disease (PGALD) is a recently proposed disease activity measure for patients with systemic juvenile idiopathic arthritis–associated lung disease (SJIA-LD). This study evaluates the reliability and construct validity of the PGALD. Methods Fifty-seven pediatric rheumatologists and pulmonologists with experience caring for children with SJIA-LD were invited to rate 20 clinical vignettes using the PGALD, a 10-point Likert scale (0 = inactive SJIA-LD; 10 = highly active SJIA-LD). Raters were also asked to rate a subset of eight repeat vignettes. Interrater and intrarater reliability were assessed using intraclass correlation coefficients (ICCs), whereas SJIA-LD features influencing PGALD ratings were assessed using univariate analysis. Results The ICC for all raters was 0.68 (95% confidence interval [CI] 0.54–0.82), indicating moderate to good interrater reliability. The retest ICC was 0.86 (95% CI 0.82–0.89), indicating good intrarater reliability. Factors associated with higher mean PGALD scores included the presence of crackles on auscultation (5.7 vs 2.9; P = 0.001), hypoxemia on pulse oximeter (5.6 vs 3.2; P = 0.01), current oxygen requirement (6.3 vs 3.5; P = 0.04), suggestive diagnostic imaging features (P ≤ 0.01), and three or more prescribed medications for SJIA-LD (P ≤ 0.01). Pulmonary function measures demonstrated significant negative correlations with PGALD scores: forced vital capacity (r = −0.71; P = 0.01), total lung capacity (r = −0.92; P = 0.01), and lung diffusion capacity (r = −0.97; P = 0.0002). Only C-reactive protein was weakly to moderately correlated with PGALD scores (r = 0.39; P = 0.09), whereas other laboratory markers (ferritin, interleukin 18, CXCL9, and sIL-2R) were not significantly correlated. Conclusions The PGALD is a novel measure of SJIA-LD activity. Its initial validation suggests acceptable construct validity and reliability. Additional studies are needed to assess its responsiveness to change over time.
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