https://pubmed.ncbi.nlm.nih.gov/35080454/ Autoimmune

AJR Am J Roentgenol. 2022 Jan 26.
doi: 10.2214/AJR.21.27204. Online ahead of print.

Associations Between Quantitative MRI Metrics and Clinical Risk Scores in Children and Young Adults With Autoimmune Liver Disease

Joseph McCrary 1 2, Andrew T Trout 1 2 3, Neeraja Mahalingam 1, Ruchi Singh 4, Cyd Castro Rojas 4, Alexander G Miethke 3 4, Jonathan R Dillman 1 2
Abstract

Background: The Mayo risk score and SCOPE (Sclerosing Cholangitis Outcomes in Pediatrics) Index are clinical risk scores for monitoring progression of primary sclerosing cholangitis (PSC) and predict clinically important endpoints. Objective: To evaluate relationships of quantitative MRI measures of liver disease with clinical risk scores in children and young adults with autoimmune liver disease (AILD). Methods: This prospective study included 58 patients (35 male, 23 female; mean age, 15 years; range, 6-24 years) with AILD [PSC (n=16), autoimmune hepatitis (n=30), autoimmune sclerosing cholangitis (n=12)] who underwent research liver MRI examinations including: MR elastography (MRE), T2*-corrected T1 (cT1), and quantitative MRCP measurements. Associations between quantitative MRI metrics and clinical risk scores were evaluated using Spearman rank-order correlation coefficients and multivariable regression analyses. Results: Mean Mayo risk score was -1.1±0.9 (range: -2.9-1.1); mean SCOPE Index was 2.7±2.2 (range: 0-9). Mean liver stiffness was 2.8±1.1 kPa, mean whole-liver mean cT1 was 875±78 ms, and mean cT1 interquartile range (IQR) was 151±56 ms. Mayo risk score was significantly correlated with liver stiffness (r=0.56; p<.001), cT1 IQR (r=0.58; p<.001), whole-liver mean cT1 (r=0.31; p=.02), and multiple quantitative MRCP measures (r=0.36-0.45, p<.001). SCOPE Index was significantly correlated with liver stiffness (r=0.68; p<.001), cT1 IQR (r=0.51; p<.001), and multiple quantitative MRCP measures (r=0.47-0.49, p<.001). Multivariable linear regression analyses identified liver stiffness, cT1 IQR, and left hepatic duct maximum diameter as significant independent predictors of Mayo risk score (adjusted R2=0.45), and liver stiffness, cT1 IQR, common bile duct maximum (CBD) diameter, and CBD median diameter as significant independent predictors of SCOPE Index (adjusted R2=0.69). On logistic regression analysis, greater than low risk by SCOPE Index was best predicted by liver stiffness [odds ratio (OR)=49.6; 95% CI: 3.1-793.6) and CBD maximum diameter (OR=2.5; 95% CI: 1.3-4.7).

Conclusion: Increased liver stiffness, increased cT1 IQR, and larger bile duct diameters are independently associated with higher (worse) Mayo risk score and SCOPE Index in children and young adults with AILD and may be surrogate markers of clinically meaningful endpoints. Clinical Impact: Multiparametric liver MRI incorporating quantitative metrics may serve as a noninvasive diagnostic and prognostic tool in pediatric AILD.