ArticleACR open rheumatology2026
Reliability and Construct Validity of the Physician's Global Assessment of Lung Disease in Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease.
Article in ACR open rheumatology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
objectiveThe 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.
methodsFifty-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.
resultsThe 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.
conclusionsThe 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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