Autoimmune liver disease requires lifelong monitoring as presentation may change
SAN ANTONIO — At this year’s National Liver Congress, Gideon M. Hirschfield, MD, presented real-life cases of patients with primary sclerosing cholangitis along with an overview of how autoimmune liver disease may change over time from initial presentation.
“When it comes to PSC, what I want you to think about are the four C’s of the disease, which the patient fears and we fear: cholangitis, cirrhosis, colitis, and — the topic of conversation no one wants have, but we need to talk about — cancer,” Hirschfield, from the Toronto Center for Liver Disease and the University of Toronto in Canada, said during his presentation. “That cancer risk in patients with chronic primary sclerosing cholangitis is substantial.”
He explained that of 100 patients with ulcerative colitis, the all-cause mortality will be approximately 18% at 10 years. In contrast, the mortality rates for those with UC and PSC range from 41% in those who developed UC first to 55% in those who developed PSC first.
“The other thing regarding the challenges of treating autoimmune liver disease is that you must always recognize what you treated, and do you still need to keep treating?” he said.
To further highlight change in autoimmune liver disease, he presented data from his clinic of 150 patients followed from childhood for approximately 15 years. Among those who presented with autoimmune hepatitis as a child, 20% had biliary-dominate overlap in adulthood. Almost all patients who presented with PSC retained the same diagnosis (94%). Finally, most of those who presented with autoimmune sclerosing cholangitis or biliary overlap later presented as “classic” PSC in adulthood (62%).
“It doesn’t matter if you’re treating one disease or two, what matters is what are you using to treat and is the patient deriving benefit, because that will tell you what to do going forward,” Hirschfeld said. “PSC is a very difficult disease and can be tiring for clinicians because many patients have bad outcomes, but your management needs to be focused on monitoring, understanding, rating the quality of care, and at present, it is about following the ongoing clinical trials.” – by Talitha Bennett
Reference: Hirschfield GM. AIH, PBC, PSC Panel Discussion. Presented at: National Liver Congress; Oct. 25, 2019; San Antonio.
Disclosure: Hirschfield reports advisory committee or review panel roles with GlaxoSmithKline and Intercept; consulting roles with CymaBay and Novartis; and grant or research support from BioTie, Falk and Takeda.