Study underway into rare chronic liver disorder

PBC is a rare, chronic liver disease that affects fewer than 200,000 Americans

KENDALL, Fla. – Mayra Solomon was living a busy and active life when suddenly in 2003, her routine blood work came back abnormal.

"Finding out the diagnosis was a shock," she said.

Solomon was told she had primary piliary cholangitis, or PBC, a rare-but-chronic liver disease that affects fewer than 200,000 Americans.

"It affects predominantly women, especially after the age of 50, so predominantly middle-age women. We could see someone women in their 30s and in some cases men, but 9-out-of-10 patients in these cases are women," said Dr. Cynthia Levy, assistant director of the Schiff Center for Liver Diseases at the University of Miami Health System.

PBC causes the body's immune system to attack healthy cells and tissue, slowly destroying the bile ducts in the liver but it's not clear what causes the disease.

"It could be genetics. It could be environmental factors, like nail polish," said Levy.

"This is like a hit-and-run mechanism. Something that happened in the past, many years before, may lead to disease development decades later." Levy is taking part in one of several clinical trials investigating new treatment options for PBC.

"It's an amazing opportunity for patients because we know if you don't respond to treatment, there's a chance you will continue to progress so being able to offer them therapies with the potential to halt disease progression is really great," Levy said.

When current medications didn't work for Solomon, she ultimately needed a liver transplant.

"I hope the new therapies will help others avoid transplantation and allow them to live a full life with PBC. That's the hope," she said.

The warning signs of PBC may initially include fatigue, itchy skin, and dry eyes and mouth. It can progress to pain in the upper right abdomen, swollen feet and ankles and weight loss among a host of other symptoms.

For details on clinical trials into PBC go to www.ClinicalTrials.gov and www.pbcstudies.com.

About the Authors: