Abstract
SummaryBackgroundBrain change can occur in primary biliary cholangitis (PBC), potentially as a result of cholestatic and/or inflammatory processes. This change is linked to systemic symptoms of fatigue and cognitive impairment.AimTo identify whether brain change occurs early in PBC. If the change develops early and is progressive, it may explain the difficulty in treating these symptoms.MethodsEarly disease brain change was explored in 13 patients with newly diagnosed biopsy‐proven precirrhotic PBC using magnetisation transfer, diffusion‐weighted imaging and 1H magnetic resonance spectroscopy. Results were compared to 17 healthy volunteers.ResultsCerebral magnetisation transfer ratios were reduced in early PBC, compared to healthy volunteers, in the thalamus, putamen and head of caudate with no greater reduction in patients with greater symptom severity. Mean apparent diffusion coefficients were increased in the thalamus only. No 1H magnetic resonance spectroscopy abnormalities were seen. Serum manganese levels were elevated in all PBC patients, but no relationship was seen with imaging or symptom parameters. There were no correlations between neuroimaging data, laboratory data, symptom severity scores or age.ConclusionsThis is the first study to be performed in this precirrhotic patient population, and we have highlighted that neuroimaging changes are present at a much earlier stage than previously demonstrated. The neuroimaging abnormalities suggest that the brain changes seen in PBC occur early in the pathological process, even before significant liver damage has occurred. If such changes are linked to symptom pathogenesis, this could have important implications for the timing of second‐line‐therapy use.
Highlights
Patients with the autoimmune cholestatic liver disease primary biliary cholangitis [formerly primary biliary cirrhosis (PBC)] frequently exhibit both central nervous system (CNS) symptoms and neurophysiological and functional CNS abnormality
This is the first study to be performed in this precirrhotic patient population, and we have highlighted that neuroimaging changes are present at a much earlier stage than previously demonstrated
The neuroimaging abnormalities suggest that the brain changes seen in PBC occur early in the pathological process, even before significant liver damage has occurred
Summary
Patients with the autoimmune cholestatic liver disease primary biliary cholangitis [formerly primary biliary cirrhosis (PBC)] frequently exhibit both central nervous system (CNS) symptoms and neurophysiological and functional CNS abnormality. Recent data from the large UK-PBC patient cohort have suggested that the severity of both fatigue and cognitive symptoms post-transplant in PBC is similar to that seen in the un-transplanted population, raising the possibility that the process responsible for CNS abnormality is not reversed by transplantation.[8] Prospective studies, albeit in smaller patient numbers, have confirmed ongoing fatigue in post-transplant patients, with a severity similar to that seen in the un-transplanted PBC population.[9] The apparent lack of change in CNS symptomology in precirrhotic PBC following liver transplantation highlights the need for improved therapy earlier in the disease course to change its natural history
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