Primary biliary cholangitis (PBC) is a progressive disease that can be divided into four different stages. However, not all people living with PBC will experience the four stages, as how the disease moves from stage to stage depends on the individual. Early diagnosis and treatment in combination with a healthy lifestyle play an important role in slowing disease progression.
The four stages of PBC
Though the stages of PBC are helpful for understanding disease progression, in the real world patients may have lab results and symptoms that overlap stages, and not fit neatly into one stage or the next. It may help to think of the stages of PBC as sections of a winding path, rather than the distinct steps of a staircase.
Stage 1: No or minimal progression
The initial stage of PBC involves the immune system starting to attack the small bile ducts, causing them to become inflamed. It is often asymptomatic, and therefore difficult to detect.
While symptoms are unlikely, routine blood work that shows elevated levels of alkaline phosphatase (ALP), a liver enzyme, can signal PBC at this stage.
If diagnosis is confirmed at this early stage, treatment is usually more effective in managing symptoms and slowing disease progression. A good response to treatment can mean that a patient doesn’t progress to later stages.
Stage 2: Mild progression
In the next stage, the bile ducts begin to scar. Inflammation spreads from the small bile ducts to the liver, which begins to cause liver inflammation. PBC may still be asymptomatic at this stage.
Stage 3: Moderate progression
As PBC progresses the liver becomes increasingly impacted by scar tissue, affecting its function. At this stage, bile flow becomes slow or blocked (called cholestasis).
This is the stage where the majority of patients start to have symptoms, namely fatigue and itching. Dry eyes and mouth, abdominal pain, bone and joint pain and edema are also possible.
Stage 4: Advanced progression
The most advanced stage of PBC is marked by cirrhosis, advanced scaring of the liver. The liver structure becomes irreparably damaged and cannot function properly.
Symptoms intensify and expand to include an enlarged liver or spleen, edema, jaundice, enlarged veins (varices), portal hypertension and fluid retention. This stage ends in liver failure and the patient will require liver transplantation.
What impacts PBC progression?
If not treated, it takes an average of one and a half to two years for PBC to progress from one stage to the next. However, this can vary greatly between cases.
The PBC patient journey depends on many factors, such as how early or late someone is diagnosed, their age and overall health at diagnosis, any pre-existing medical conditions and the impact of unhealthy lifestyle habits. All these factors, as well as how patients respond to treatment, will affect their symptom burden, quality of life and how the disease progresses.
Your healthcare team will closely track any signs of disease progression by monitoring your liver function and symptoms. Keeping your doctor informed of any changes can help guide your treatment and care, and slow the progression of PBC.
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