By Natalia Gurevich, SWHR Communications Intern
What is PBC? Not to be confused with other, similar acronyms (PBS is a TV station, guys), Primary Biliary Cholangitis, or PBC, is a chronic disease of the liver that slowly destroys the medium-sized bile ducts within the liver. It disproportionately affects women [1] with 25 to 335 per million women impacted by the disease at any given time [6].
Recently, the health community weighed in overwhelmingly to change the name of Primary Biliary Cirrhosis to Primary Biliary Cholangitis. This change allows for a better understanding of the disease and its progression, because cirrhosis occurs only in the late stage [1]. The name Primary Biliary Cirrhosis is actually a misnomer for patients in the earlier stages of the illness, when cirrhosis has not occurred yet [1]. A more accurate name for this disease will serve to help the affected community better.
PBC is an incredibly difficult disease to track and predict. Many who have it will carry the disease but not show symptoms for years. Even those affected have to take multiple liver tests before doctors can confirm that they have PBC [1].
As hard as PBC is to diagnose, it’s even harder to pinpoint a cause. The main cause is still unknown, although some theorize it may be related to problems in the immune system [2]. PBC is technically not a hereditary disease [5]; however, there appears to be some common link among siblings and in families where one member has been affected [1].
Women are much more likely to suffer from PBC, accounting for 90 percent of all cases. The disease is most common in patients ages 35 to 60 [3]. The main difference in sexes is relatively unknown, but certain epidemiologic studies have shown different environmental risk factors that might be related. Some factors include hair dye use, recurrent urinary tract infections, smoking, and estrogen deficiency, all of which may contribute to increased disease in women [3].
Those who are affected can go 10 to 15 years before symptoms present themselves, especially if the disease is caught early and treated immediately [1]. When symptoms do occur, the most common is pruritus, or intense itching of the skin, often in the arms, legs and back. Other symptoms may include fatigue, jaundice fluid build-up in the ankles and abdomen, darkening of the skin, and collection of fatty deposits in the skin around the eye [2].
PBC affects such an integral part of the body that many other disorders present themselves as a result. The most common is problems with the tear and salivary glands, causing dry eyes or mouth [2]. In older patients, arthritis and thyroid problems, renal stones and gallstones may develop. PBC can also cause certain vitamin deficiencies, leading to bone softening and fragility which can result in fractures in the late stages of the disease [1].
The recent change of PBC’s official name, is a big step for the affected community toward better understanding of the disease. However, the causes for this disease are still relatively unknown, and treatments are only geared toward abetting symptoms. Currently, there is no cure. More research must be conducted on this disease, and more studies geared to the biological sex differences behind its origin [4].
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