Department of Ophthalmology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami, Sagamihara, Kanagawa, 252-0374, Japan. nshoji@kitasato-u.ac.jp.
From the Retina Foundation of the Southwest, Dallas, TX (E.E.B., R.M.J., L.A.H., S.E.M., M.D., K.R.K.), Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA (E.E.B., K.R.K.). Electronic address: ebirch@retinafoundation.org.
From the Retina Foundation of the Southwest, Dallas, TX (E.E.B., R.M.J., L.A.H., S.E.M., M.D., K.R.K.), Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA (E.E.B., K.R.K.).
From the Retina Foundation of the Southwest, Dallas, TX (E.E.B., R.M.J., L.A.H., S.E.M., M.D., K.R.K.), Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA (E.E.B., K.R.K.).
From the Retina Foundation of the Southwest, Dallas, TX (E.E.B., R.M.J., L.A.H., S.E.M., M.D., K.R.K.), Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA (E.E.B., K.R.K.).
From the Retina Foundation of the Southwest, Dallas, TX (E.E.B., R.M.J., L.A.H., S.E.M., M.D., K.R.K.), Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA (E.E.B., K.R.K.).
Mossakowski Medical Research Institute, Polish Academy of Sciences; Institute of Radio Electronics and Multimedia Technology, Warsaw University of Technology.
Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease characterized by inflammation of the interlobular bile ducts. Ursodeoxycholic acid (UDCA) is the only FDA approved first-line t...
A total of 257 primary biliary cholangitis (PBC) patients treated with UDCA (13-15 mg/kg/d) were enrolled in this retrospective study. The response to treatment was evaluated based on alkaline phospha...
The results of multiple regression analysis showed that NLR at baseline was positively associated with the nonresponse to UDCA treatment after adjustments for potential confounders (age, sex, BMI, hyp...
Our results suggest that PBC patients with a high NLR had a worse response to UDCA therapy....
Primary biliary cholangitis (PBC) is a rare chronic autoimmune cholangiopathy, characterized by a variable course and response to treatment. We aimed to describe long-term outcomes of PBC patients ref...
This is an ambispective cohort study of PBC patients (retrospective component: diagnosis before 1 January 2019; prospective component: thereafter), including 302 patients: 101 (33%) followed up in Nov...
Among the 302 patients (88% women, median age 55 years, median follow-up 75 months), alkaline phosphatase (ALP) levels significantly decreased during treatment with ursodeoxycholic acid (UDCA, P < 0.0...
In PBC, both short-term response to UDCA and long-term survival can be predicted by simple conventional biomarkers of disease severity, obtained at diagnosis....
ENHANCE was a phase 3 study that evaluated efficacy and safety of seladelpar, a selective peroxisome proliferator-activated receptor-δ (PPAR) agonist, versus placebo in patients with primary biliary c...
Patients were randomized 1:1:1 to oral seladelpar 5 mg (n=89), 10 mg (n=89), placebo (n=87) daily (with UDCA, as appropriate). Primary end point was a composite biochemical response [alkaline phosphat...
Patients with primary biliary cholangitis (PBC) with inadequate response or intolerance to UDCA who were treated with seladelpar 10 mg had significant improvements in liver biochemistry and pruritus. ...
Several ursodeoxycholic acid (UDCA) treatment response definitions have been introduced in primary biliary cholangitis (PBC). However, the lack of a gold standard results in heterogeneity in second-li...
This study aimed to explore which UDCA treatment response endpoint serves as the most accurate predictive model of long-term outcome....
A systematic review and meta-analysis of UDCA treatment response endpoints (and corresponding validations) were performed....
Sixteen individual UDCA treatment response endpoints and 96 external validations were found. Barcelona, Paris-1, Paris-2, Rotterdam, Toronto and GLOBE and UK-PBC Risk Scores are currently most robustl...
The GLOBE and UK-PBC Risk Scores are the most suitable for the real-world allocation of second-line therapies (obeticholic acid and fibrates). However, in the wake of the recent findings, alkaline pho...
After 1 year of ursodeoxycholic acid (UDCA), patients with primary biliary cholangitis (PBC) may have a normal GLOBE score despite high alkaline phosphatase (ALP) levels....
To assess the association between ALP and liver transplantation (LT)-free survival according to the GLOBE score METHODS: Among patients with a normal or elevated GLOBE score in the Global PBC cohort, ...
After 1 year of UDCA, ALP was associated with the risk of LT/death (aHR 1.31, 95% CI 1.003-1.72, p = 0.048) among 2729 patients with a normal GLOBE score. The 10-year LT-free survival among these pati...
The LT-free survival of patients with PBC with a normal GLOBE score is optimal in case of normal ALP levels, also in relation to the general population. Despite their generally favourable prognosis, a...
Cholestasis is a clinical syndrome triggered by the accumulation and aggregation of bile acids by subsequent inflammatory responses. The present study investigated the protective effect of glycyrrheti...
Primary sclerosing cholangitis is a progressive cholestatic liver disease that causes stricturing of the intra and extrahepatic bile ducts that can lead to cirrhosis and end stage liver disease. Effec...
Fenofibrate (FF) has shown potential benefits in patients with primary biliary cholangitis (PBC) who have an incomplete response to ursodeoxycholic acid (UDCA). However, the efficacy and safety of FF ...
It remains unclear whether ursodeoxycholic acid (UDCA) treatment improves long-term outcomes in patients with primary sclerosing cholangitis (PSC). In this study, we investigated whether UDCA treatmen...
We used retrospective data from the Japanese PSC registry that included 435 patients with PSC. In this study, we enrolled patients with a complete dataset at diagnosis, along with the diagnosis year, ...
Among 435 patients with PSC, 110 were excluded due to insufficient or missing data, and the remaining 325 patients (male, 187 (58%); mean age at diagnosis, 45.8 years) were enrolled. The mean follow-u...
In this Japanese PSC cohort, UDCA treatment was significantly associated with improved LT-free survival....
Response to ursodeoxycholic acid (UDCA) in primary biliary cholangitis (PBC) has been traditionally assessed 1 to 2 years after treatment initiation. With the development of new drugs, some patients m...
This study aims to identify whether well-validated response criteria could correctly identify individuals likely to benefit from add-on second-line therapy at 6 months....
Analysis of a multicenter retrospective cohort which included only patients with clear-cut PBC....
206 patients with PBC (96.6% women; mean age 54 ± 12 years) were included. Kappa concordance was substantial for Toronto (0.67), Rotterdam (0.65), Paris 1 (0.63) and 2 (0.63) criteria at 6 and 12 mont...
After 6 months of treatment with UDCA, the absence of response by different criteria could properly identify patients who could benefit from early addition of second-line therapies, especially in pati...
