Clinical characteristics of newly diagnosed patients with primary biliary cholangitis (PBC) indicate the need for better awareness on timely diagnosis and adequate UDCA therapy.

Guidelines on primary biliary cholangitis (PBC) recommend therapy with 13-15 mg/kg ursodeoxycholic acid (UDCA) and assessment of treatment response after 12 months. We evaluated to which extent these recommendations are followed in newly diagnosed patients.The German PBC Registry recruited three subgroups: Adequate or inadequate UDCA treatment responders (Paris II criteria) and newly diagnosed patients (<6 months prior to recruitment). We focus on newly diagnosed patients with UDCA monotherapy.82 patients were recruited (43 at 12 tertiary and 39 at 9 secondary centers) thereof 22% with cirrhosis. Individuals with cirrhosis were older (71 ± 9 vs. 55 ± 14 years, p<0.001) and presented more frequently with diabetes mellitus (44% vs. 13%, p=0.0054) and arterial hypertension (78% vs. 42%, p=0.0076) compared to cases without cirrhosis.12 months follow-up data were available in 62 patients. UDCA underdosing (<13 mg/kg/d) occurred in 47% and 74% of cases (p=0.013) at tertiary and secondary care at treatment initiation and in 29% and 73% (p=0.002) after 12 months, respectively. Paris II criteria were achieved in 74% and a deep UDCA response (alkaline phosphatase < ULN and bilirubin < 0.6 × ULN) in 32% of cases.Newly diagnosed PBC patients include a substantial proportion of late presenters with cirrhosis. UDCA dosage is suboptimal in many cases. Time point of diagnosis and UDCA dosage should be improved.