While primary biliary cholangitis (PBC) is most commonly diagnosed in middle-aged women between the ages of 35 and 55 years, it can also be found in an older population, or those older than 65 years. In men for example, while cases of PBC are rare, it is often diagnosed at a later age.
PBC is a rare autoimmune disease that affects the liver. The body’s immune system attacks the bile ducts within the liver, breaking them down over time. This leads to scarring of the liver tissue, damaging its function and progressing to fibrosis and cirrhosis of the liver. Liver failure is the final stage of PBC, and the only cure is a liver transplant.
No-one knows what specifically causes PBC, with genetic and environmental factors thought to play a role. Its onset is not related to diet or alcohol consumption. There is no cure for PBC, but treatment aims to slow disease progression and help manage the symptom burden.
Symptoms are not always immediately present and can develop over the course of the disease. They include fatigue, pruritus (itchy skin), abdominal pain, brain fog, jaundice and can heavily impact the quality of life of people with PBC.
PBC in later life
A U.K. study found that older people with PBC have a slightly different experience of the disease to that of younger patients, who appear to suffer more in terms of symptom burden and quality of life. This may be due to a reported lower response to the first-line treatment, ursodeoxycholic acid (UDCA). Younger people may also cope less successfully with the effects of chronic fatigue and incessant itching.
As a result of improved screening, PBC is often detected in the early stages of the disease, leading to better outcomes. In patients over 65, PBC may be detected in routine blood liver tests or as part of investigations into other suspected conditions. As people with PBC can be asymptomatic in the early stages of the disease, it can be difficult to diagnose.
PBC can also develop in people with other autoimmune diseases, such as thyroid diseases, celiac disease, Raynaud’s disease, Sjögren’s syndrome, scleroderma and autoimmune hepatitis.
Prognosis in later life
Prognosis in patients diagnosed with PBC later in life depends on the stage at which it is diagnosed, overall general health of the person and response to treatment. In patients who are first diagnosed with PBC over the age of 65 years, the rate of liver transplant is lower due to higher risks, and liver-related mortality is higher.