Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an autoimmune disorder in which a person’s bile ducts become inflamed and then slowly destroyed. In autoimmune diseases, the body’s immune system mistakenly attacks healthy tissue and cells.
Bile is a fluid that is secreted by the liver; it assists with digestion, with the absorption of certain vitamins and with the elimination of wastes from the body. Bile helps the body to absorb fats, as well as to eliminate cholesterol, worn-out red blood cells and toxins.
When inflammation persists in a person’s liver, this can lead to bile duct inflammation and damage, which is termed cholangitis. Occasionally, this can result in permanent scarring of the liver tissue, which is called cirrhosis. Eventually, cholangitis can cause liver failure in some individuals.
The prevalence of definite or probable PBC is approximately 35 cases per 100,000 patient population. Annual incidence of the condition is between two and three cases per 100,000 patient population.
What are the signs and symptoms of PBC?
Symptoms vary among patients with PBC. Fatigue, which is the most common symptom, is reported in up to 78% of individuals with the disorder. These patients may experience feelings of helplessness, being overwhelmed, and difficulty functioning.
Other symptoms often reported among patients with PBC include pruritus (itchy skin), brain fog, dry mouth and/or eyes, joint pain and stiffness, sleep disorders, and abdominal pain. All of the symptoms experienced by a patient can have a negative effect on health-related quality of life.
Learn more about PBC signs and symptoms
What sleep disorders are common among patients with PBC?
It is well known that excessive daytime sleepiness (EDS) and poor quality of sleep are reported among individuals with PBC. In fact, poor sleep quality can impair a person’s ability to focus.
Realizing that you’re not getting enough sleep is easy. Figuring out why your sleep is not restful is more difficult to accomplish, but it is certainly possible.
What measures are used to classify fatigue among patients with PBC?
In patients with PBC, the Epworth Sleepiness Scale (ESS) is used to measure EDS. Higher scores on the ESS are reported among those with the disorder.
Sleep quality and sleep disturbances among those with PBC are evaluated with use of the Pittsburgh Sleep Quality Index (PSQI). Higher PSQI scores are indicative of worse quality of sleep.
In fact, patients with PBC experience a delay in sleep timing that is linked to impaired sleep quality and quality of life. Pruritus seems to be associated with the feeling of sleepiness.
How to improve sleep with PBC
In patients with PBC who exhibit EDS, treatment with the stimulant medication modafinil has been studied. One open-label study published in 2007 demonstrated improved ESS and associated fatigue with modafinil therapy. In a randomized, placebo-controlled study published in 2017, however, treatment with modafinil was not associated with any beneficial effects on fatigue among individuals with PBC.
Another strategy that has been used for improving sleep among patients with PBC is to target their pruritus—a commonly reported problem in this population. Treatment with naltrexone—a opiate receptor antagonist that is used to treat pruritus—seems to improve sleep quality.
The antihistamine hydroxyzine has demonstrated improvement in both subjective and objective sleep quality. It is unclear, however, if hydroxyzine acts directly on pruritus or provides benefits via its sedating effects.
Morning bright light therapy appears to exhibit positive effects on daytime sleepiness, subjective sleep quality, and sleep timing in patients with PBC.
In spite of these potential therapies, a systematic review and meta-analysis published in 2019, in which a variety of treatments for PBC-associated fatigue were evaluated, could not identify any beneficial intervention other than the use of liver transplantation in this patient population.
