The Impact of Primary Biliary Cholangitis (PBC): A Rare Autoimmune Disease

October 09, 2024 at 08:01 AM EDT

(BPT) - Imagine waking up every day feeling utterly exhausted, no matter how much sleep you get. Now, add an unbearable itch that just won't go away, dry eyes and mouth, and stomach pain. This is the reality for some people living with primary biliary cholangitis (PBC).

A Closer Look at Primary Biliary Cholangitis

Primary biliary cholangitis is a rare autoimmune disease in which the immune system mistakenly attacks and slowly destroys the bile ducts in the liver. Over time, this can lead to inflammation of the bile duct and scarring of the liver and may cause serious complications.¹

The symptoms of PBC can at first be mild and get worse over time, and it may take 5 to 20 years to develop.¹ Common signs are chronic itch (pruritus) and extreme fatigue, both of which may impact daily life.^1,2 Some patients may have no symptoms, therefore the disease is oftentimes only discovered through routine liver blood tests.³

The Challenges of Getting a PBC Diagnosis

"The symptoms aren't always obvious," shares Naveen, a patient with PBC. "There haven't been many treatments available for me in the past, which can be disheartening, especially with the intense symptoms that really affect my life on a daily basis."

For many, the journey to diagnosis may involve numerous tests and procedures.⁴ The experience can be emotionally and physically exhausting, especially given the impact that untreated PBC can have on a person's health.²

A Disease That Disproportionately Affects Women

PBC is more common in women, particularly those who are over 40 years old, with approximately 1 in 1000 women in this age group affected and 65 out of every 100,000 women in the US diagnosed with the condition.^3,5 Although the disease is more prevalent in women, an increasing number of men are now being diagnosed. On average, women receive a diagnosis within 12 months, while for men, the process can take up to three years.⁶

If left untreated, PBC can lead to permanent liver damage, and even severe cases of liver failure.¹ Early diagnosis and proactive management of the disease can play an important role in reducing the risk of complications and may help in improving the health of those affected.^7,8

Living With PBC: The Daily Reality

Living with PBC means more than just managing the physical symptoms; it's also about coping with the emotional and psychological toll that the disease can take. This could be incessant itching, debilitating fatigue, or other symptoms that can make it difficult to maintain a normal lifestyle, impacting work, social relationships, and overall well-being.²

For those with PBC, connecting with patient advocacy groups and support communities may offer both practical advice and emotional comfort, helping to navigate the complexities of living with this chronic condition.^9,10

Moving Forward

While the challenges of living with PBC can be significant, advancements in understanding the disease and ongoing research into new treatment options offer hope.¹¹ For people living with PBC, some treatment goals include improving or normalizing alkaline phosphatase (ALP) levels and managing symptoms of the disease, such as pruritus.^2,12,13 A treatment plan that helps sustain ALP levels and more effectively fights debilitating symptoms may help improve disease outcomes.^2,12,13

If you or someone you know is experiencing symptoms that might suggest PBC, it's important to seek medical advice. Early detection and proactive management may help make a difference in the course of the disease and the outcomes of those affected.^7,8

References

Mayo Clinic. Primary biliary cholangitis. Accessed August 29, 2024. Available at: https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874
Sivakumar T, Kowdley KV. Anxiety and depression in patients with primary biliary cholangitis: current insights and impact on quality of life. Hepat Med. 2021;13:83-92. doi:10.2147/HMER.S256692
American Liver Foundation. Primary biliary cholangitis. Accessed August 29, 2024. Available at: https://liverfoundation.org/liver-diseases/autoimmune-liver-diseases/primary-biliary-cholangitis-pbc/
Mayo Clinic. Primary biliary cholangitis diagnosis. Accessed September 17, 2024. Available at: https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880
Hohenester S, Oude-Elferink RP, Beuers U. Primary biliary cirrhosis. Semin Immunopathol. 2009;31(3):283-307. doi:10.1007/s00281-009-0164-5
Abdulkarim M, Zenouzi R, Sebode M, et al. Sex differences in clinical presentation and prognosis in patients with primary biliary cholangitis. Scand J Gastroenterol. 2019;54(11):1391-1396. doi:10.1080/00365521.2019.1683226
Martini F, Balducci D, Mancinelli M, et al. Risk stratification in primary biliary cholangitis. J Clin Med. 2023;12(17):5713. doi:10.3390/jcm12175713
Cleveland Clinic. Primary biliary cholangitis (PBC): living with this chronic disease. Accessed August 29, 2024. Available at: https://health.clevelandclinic.org/living-with-pbc
National Organization for Rare Disorders. Primary biliary cholangitis organization. Accessed September 17, 2024. Available at: https://rarediseases.org/organizations/primary-biliary-cholangitis-organization/
Global Liver Institute. Primary biliary cholangitis (PBC) externally-led patient-focused drug development (EL-PFDD). Accessed September 17, 2024. Available at: https://globalliver.org/wp-content/uploads/2023/02/PBC-EL-PFDD.pdf
Bernal RB, Castro CM, Ferrigno B, et al. Management of primary biliary cholangitis: current treatment and future perspectives. Turk J Gastroenterol. 2023;34(2):89-100. doi:10.5152/tjg.2023.22239
Murillo Perez CF, Harms MH, Lindor KD, et al. Goals of treatment for improved survival in primary biliary cholangitis: treatment target should be bilirubin within the normal range and normalization of alkaline phosphatase. Am J Gastroenterol. 2020;115(7):1066-1074. doi:10.14309/ajg.0000000000000557
Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019;69(1):394-419. doi:10.1002/hep.30145

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The Impact of Primary Biliary Cholangitis (PBC): A Rare Autoimmune Disease

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