Gaps in the patient journey through the diagnosis and management of primary biliary cholangitis

Document Type

Conference Proceeding

Publication Date


Publication Title

Am J Gastroenterol


Introduction: Primary biliary cirrhosis cholangitis (PBC) is a chronic, inflammatory, autoimmune disease primarily targeting the hepatic bile duct cholangiocytes that is associated with systemic manifestations. Longterm management is essential. Published guidance on both diagnosis and management are expected to give guidance to practitioners regarding their patients with PBC. The aim of our study was to identify potential gaps in both the diagnosis and management of PBC amongst providers Methods: Patients ≥ 18yo diagnosed with PBC from 7 institutions were included. Participating providers were GI/Hepatology. Diagnosis of PBC was based on current AASLD criteria. Patients were included if they had ≥ 4 documented PBC laboratory assessments within 3 years or 5 prior office visits. Both prospective and retrospective chart analysis was performed. Data was collected and analyzed centrally via RedCap with support of the Chronic Liver Disease Foundation, a non-profit educational organization dedicated to increasing awareness of the effects of chronic liver disease in the US Results: 118 patients were included (87% F, 13% M, average age 63). 32% of patients were diagnosed by the participating provider with the remaining 68% of patients being previously referred. Of those referred, the majority were diagnosed by a specialist (GI 64%, Hepatology 21%, IM 3%, unknown 10%). 70% underwent liver biopsy(bx) at diagnosis or referral. Initial bx stage 1 (27%), 2 (28%), 3 (9%) 4 (6%). Of the remaining 30%, fibroscan was used infrequently (6%) at diagnosis or following. Symptoms were common (fatigue 32%, pruritus 30%). Pruritus management included antihistamines 61%, cholestyramine 28%, Rifampicin 7%, SSRI 4%. Providers reported patient monitoring as follows: biannual visits 79% and labs 41%. 93% were receiving or planned to receive ursodeoxycholic acid (UDCA). 47% of patient lab results met criteria for OCA treatment defined as ALP >160 with only 19% receiving or planned to receive. Over the study period, ALP decreased in 75% of patients with 45% falling into the normal range (Figure 1). 77% of patients on OCA (n=22) had a decrease of ALP on treatment as compared with 60% of patients on UDCA alone Conclusion: These data suggest opportunity for staging, follow up and medical management of patients with PBC. Institution of planned educational efforts toward providers around current published guidelines for PBC are necessary to close these gaps.

PubMed ID

Not assigned.





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