A large, real-world study of primary biliary cholangitis has revealed that patients who are female, older, or have other autoimmune diseases are likely to have a more progressed and aggressive disease profile.
In the, researchers reported the findings of a medical records database study involving 15,875 patients with primary biliary cholangitis (PBC) – previously known as primary biliary cirrhosis – a chronic, autoimmune form of liver disease.
Overall, more than one-third of patients (38.3%) had high levels of alkaline phosphatase – a marker for treatment nonresponse, defined as at least 1.5 times the upper limit of the normal range, which is also an indicator of adverse outcomes and of progression to high-risk liver disease.
These patients were more likely to be female, less likely to be insured by Medicaid, and more likely to have been diagnosed more than 1 year ago than patients whose alkaline phosphatase levels were not high. They were also more likely to be older, from the Midwest or Southern regions of the United States, have cirrhosis, or have other autoimmune diseases such as Sjögren’s syndrome and RA.
Patients with high alkaline phosphatase also showed higher aminotransferase and bilirubin, more cirrhosis, pruritus, and jaundice, but lower albumin.
Conversely, male patients had a higher incidence of cirrhosis, the study found. Other factors independently associated with cirrhosis included older age, having Medicaid insurance, having high alkaline phosphatase, and certain autoimmune conditions including type 1 diabetes, autoimmune hepatitis, and ulcerative colitis.
In patients with cirrhosis, the authors saw higher serum levels of AST and bilirubin, but lower albumin and platelets.
, from the Center for Liver Diseases at Inova Fairfax Hospital, Falls Church, Virginia, and his coauthors said the results suggest many patients with PBC have progressed further in their condition than previously thought.
“This implies that a heightened focus on these patients with a goal toward treating more optimally should be considered to reduce their probability of disease progression,” they wrote. “Once cirrhosis develops, adverse patient outcomes such as increased mortality and adverse health care system outcomes such as excessive resource utilization increases substantially.”
The authors noted that most patients were female and white – consistent with previous reports of PBC – but the mean age of 60 years was older than expected.
“Our data suggest that PBC patients may be getting older and this could have major implications for Medicare,” they wrote. The study also examined how patients used health care resources, and found those with alkaline phosphatase levels more than 1.5 times the upper range of normal had significantly higher use. For example, they had significantly more all-cause and disease-related visits to the doctor and more use of outpatient resources for all causes.
They also had significantly more cumulative days of treatment with ursodeoxycholic acid – the standard treatment for PBC – at 528.4 days, compared with 41.6 days in individuals without high alkaline phosphatase levels. However they were no more likely to undergo imaging procedures.
Patients with cirrhosis were also more likely to have higher levels of health care utilization, compared with patients without cirrhosis, particularly use of outpatient services, inpatient stays, and ED visits. The authors also noted that patients with Medicaid but not Medicare had a higher rate of abdominal procedures.
Given that more advanced disease and presence of cirrhosis were both major drivers of increased health care use, the authors called for better identification and treatment of these patients. “This should not only potentially improve patients’ long-term outcomes but also aid in the reduction or delay of conceivably costly health resource utilization,” they wrote.
Two authors declared research funding or consulting fees from the pharmaceutical industry, and one author was an employee of Intercept Pharmaceuticals. No other conflicts of interest were declared.
SOURCE: Younossi ZM et al. J Clin Gastroenterol. 2018 Aug 24. .