8 recent reports on PSC, PBC autoimmune liver diseases

Researchers are continually evaluating the autoimmune diseases primary sclerosing cholangitis and primary biliary cholangitis and their effects on bile duct inflammation and liver damage.

Some of the latest studies demonstrated that obeticholic acid improved GLOBE scores in PBC and relieved pruritus symptoms in PSC. Other recent reports include discussion of a novel PSC patient-reported outcome tool, cases of hepatitis E found among patients with PSC and inflammatory bowel disease, and demographic risk factors.

Below, Healio.com/Hepatology has compiled a list of some of the most recent reports on PSC and PBC for 2017.

PBC risk increases among relatives of diagnosed patients

A population-based study in Iceland showed that first-, second- and third-degree relatives of patients with primary biliary cholangitis have a significantly increased risk for the disease.

“The results substantially extend previous observations on the familiality of PBC, by showing that an increased risk of disease extends significantly to both second- and third-degree relatives,” the researchers wrote. “This supports a strong role of genetics in the pathogenesis of PBC and provides a further rationale for utilizing genetic research methods to uncover novel genetic factors associated with development of the disease.” Read more

Novel PSC patient-reported outcome tool demonstrates high validity

A novel primary sclerosing cholangitis patient-reported outcome instrument demonstrated significant consistency and discriminant validity, according to a recently published study.

“Symptoms of PSC are not only reflective of disease severity, but can also cause substantial impairment of patients’ health-related quality of life and other patient-reported outcomes such as fatigue or reduced work productivity; coexistent [inflammatory bowel disease] can additionally impair [patient-reported outcomes (PROs),” Zobair M. Younossi, MD, MPH, from the Inova Fairfax Hospital, Virginia, and colleagues wrote. “Increasingly recognized as important, PROs reflect patients’ experience with their disease and its treatment. In this context, development of a validated PRO instrument for PSC to complement clinical outcomes of these patients is needed.” Read more

VIDEO: Expert discusses cases of hepatitis E, PSC in patients with IBD

ORLANDO — In this exclusive video from Advances in IBD 2017, Edward V. Loftus, Jr., MD, professor of medicine in the division of gastroenterology and hepatology at the Mayo Clinic, discusses a panel presentation on liver disease in patients with inflammatory bowel disease.

The cases included a patient with hepatitis E virus infection, a patient who developed autoimmune hepatitis during treatment with Remicade (infliximab, Janssen), and a patient with Crohn’s disease who developed primary sclerosing cholangitis. Watch here

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PSC more common among African-Americans than previously thought

Recent data revealed that primary sclerosing cholangitis occurs more frequently among African-Americans than previously published studies have shown.

“Although these data do not demonstrate that PSC is as common in black and white patients, PSC in blacks is not as rare as what would be interpreted based on published data,” David Goldberg, MD, MSCE, and colleagues wrote. “This suggests that existing literature may reflect selection bias related to the demographics of the underlying population from where these studies emerge, rather than a clinical disease largely restricted to white patients.” Read more

Obeticholic acid decreases GLOBE score in PBC

Patients treated with obeticholic acid for primary biliary cholangitis were significantly more likely to achieve a GLOBE score of 0.3 or lower compared with placebo, according to a presentation at the World Congress of Gastroenterology at ACG 2017.

According to Marlyn J. Mayo, MD, a GLOBE score of 0.3 or lower is correlated with liver transplant-free survival like that of sex- and age-matched normal population, whereas a score above 0.3 is correlated with significantly diminished liver transplant-free survival. Read more

Pediatric PSC outcomes still poor; effective treatment needed

Nearly half of pediatric patients with primary sclerosing cholangitis developed an adverse liver outcome within 10 years of diagnosis, according to a recent retrospective review.

“Pediatric PSC has a chronic, progressive, and relentless course,” Mark R. Deneau, MD, from the University of Utah, and colleagues wrote. “The identification of a portal hypertensive or biliary complication represents a tipping point in the natural history, with a subsequent median survival with native liver of only 3 years. These outcomes occurred despite treatment of the vast majority of patients with chronic ursodeoxycholic acid therapy. Effective treatments are needed to change the course of this disease.” Read more

Combination therapy improves outcomes, pruritus in PBC

Long-term treatment with combination ursodeoxycholic acid and bezafibrate resulted in normalized alkaline phosphatase among more than 50% of patients with primary biliary cholangitis in a recently published study.

The combination therapy was most effective among those with lower fibrosis and severity of cholestasis, but provided complete or partial itching relief in most patients. Read more

VIDEO: Obeticholic acid decreases alkaline phosphatase in PSC

WASHINGTON — In this exclusive video from The Liver Meeting 2017, Kris V. Kowdley, MD, FAASLD, of the Swedish Medical Center, Liver Care Network, Seattle, discusses findings on obeticholic acid on pruritus among patients with primary sclerosing cholangitis.

“A lot of clinicians are somewhat hesitant at treating people who inject drugs because they believe that they’ll become reinfected and unsuccessfully cured,” he told Healio Gastroenterology and Liver Disease. “What we’ve found so far ... is that there are cases of reinfection, but the overall incidence of reinfection is relatively low.” Watch here