Editor’s Note: Patient Worthy is honored to share this article, originally submitted to us by Andres Contreras.
Primary biliary cholangitis (PBC) is a long-term liver disease in which the immune system mistakenly attacks the small bile ducts inside the liver. These ducts carry bile out of the liver. When they are damaged, bile builds up, and over time this can lead to scarring, cirrhosis, and in some cases liver failure.
PBC is uncommon. In the United States, it is estimated to affect about 40 out of every 100,000 people. The majority of those diagnosed are women: 9 out of 10 cases occur in women, usually between the ages of 40 and 60. Many people with PBC also have another autoimmune condition, such as thyroid disease, dry eyes and mouth, or arthritis.
The most common symptoms are persistent fatigue and itchy skin (pruritus). The fatigue can be debilitating and does not always improve with standard treatment. The itching can also be intense. But what makes this disease especially dangerous is that about half of patients have no symptoms at the time of diagnosis.
October is Liver Disease Awareness Month, a time to draw attention to conditions like PBC that often go unrecognized.
The problem is not the lack of treatment. It is the lack of diagnosis
A recent real-world study of 84 patients with PBC found that the average time between the first warning sign in a blood test (elevated alkaline phosphatase levels) and the diagnostic test was 114 weeks. More than two years. In patients with higher levels of this enzyme, the delay was even longer: nearly three and a half years.
Another retrospective study of 163 patients found an average time from first symptoms or abnormal blood work to diagnosis of approximately three years. Patients who showed only two or three warning signs experienced the longest delays.
Why does this happen? PBC is often diagnosed in hepatology, but the first symptoms — itching, fatigue — lead patients to dermatologists, rheumatologists, or primary care physicians. These specialists rarely order the antimitochondrial antibody (AMA) test, which is the diagnostic key.
A review of 12,000 women over the age of 40 found that only 12% received the AMA test and only 9% were referred to hepatology.
Early diagnosis matters. There is a first-line treatment (ursodeoxycholic acid) that can slow the progression of the disease, but it is most effective when started early.
If you have persistent fatigue or unexplained itching, and especially if you are a woman over 40, it is worth asking your doctor about PBC. A blood test may be enough to rule it out or confirm it.
If you are living with PBC, your story matters. Sharing your experience — how you were diagnosed, what you have learned, what you want others to know — can help someone else get diagnosed sooner.
References
Levy C, Buchanan-Peart KA, MacEwan JP, et al. A nationwide study of primary biliary cholangitis prevalence, geographic distribution, and health care providers. Hepatology Communications. 2025;9(5):e0677. doi:10.1097/HC9.0000000000000677
Vafapour P, Butler M, Mattison K, et al. P50 Real-world delays in primary biliary cholangitis diagnosis: a 17-year retrospective audit of time from ALP elevation to diagnosis. Gut. 2025;74(Suppl 4):A46.1-A46.
Davila AM, et al. Diagnostic delays in Primary Biliary Cholangitis: a retrospective study of 163 patients. Abstract presented at: PBC Health Hub; September 2025.
Editor’s Note: Patient Worthy’s partner, The PBCers Organization, can offer more information and support here.
