Characterization of Pulmonary Dysfunction in Systemic Juvenile Idiopathic Arthritis Using Xenon and Proton MRI

INTRODUCTION: Systemic juvenile idiopathic arthritis-associated lung disease (SJIA-LD) is increasingly recognized and associated with potentially life-threatening complications. Diagnosis is challenging as SJIA-LD is complex and frequently presents with subtle or no respiratory symptoms, necessitating CT imaging or other tests to detect budding dysfunction. We hypothesized that xenon and proton lung MRI could identify SJIA-LD-associated structural changes and functional deficits. METHODS: Thirty-one participants aged 5-15 (16 healthy, 7 SJIA without diagnosed lung disease [SJIA-non-LD], 8 SJIA-LD) underwent MRI for this cross-sectional study. Participants underwent xenon ventilation MRI (14 healthy/6 SJIA-non-LD/7 SJIA-LD), xenon gas-exchange MRI (12 healthy/3 SJIA-non-LD/5 SJIA-LD), and/or proton ultrashort echo time (UTE) MRI (12 healthy/5 SJIA-non-LD/7 SJIA-LD). Ventilation defect percentage (VDP) and ratios of xenon signal in alveolar membrane (Membrane), red blood cells (RBC), and gas were calculated from xenon MR images. CTs and PFTs were obtained from participant medical records where available for comparison. RESULTS: RBC/Membrane differed significantly (p = 0.014) between healthy individuals and SJIA-LD patients, and corresponded with DLCO results in a limited sample of participants with both measurements. VDP did not differ significantly between groups (p = 0.30), but was abnormally high in 3/7 SJIA-LD patients. SJIA-LD pattern findings in UTE MRI were detected in 4/7 SJIA-LD patients and in no healthy or SJIA-non-LD participants (p < 0.001). CONCLUSION: Xenon and proton lung MRI can identify SJIA-LD-associated pulmonary abnormalities, with RBC/Membrane showing particular promise for characterization of pulmonary diffusion limitation in SJIA-LD. Future studies will evaluate lung MRI for SJIA-LD phenotyping and longitudinal monitoring of SJIA-associated lung function changes.