Some patients have complications of PBC that require therapy. In many cases, doctors find that symptoms can be controlled by lifestyle modifications and medications.
Itching can be a very troublesome symptom of PBC. Itching may range from mild to severe, where a patient’s lifestyle may be impaired. Itching may be worse with fatty meals or at night, interfering with sleep. The initial medications typically used by doctors include diphenhydramine, cholestyramine and colestipol. Rifampin is used in patients who fail or are intolerant to cholestyramine and colestipol. In resistant cases, opioid antagonists such as nalmefene, naloxone and naltrexone are used. Plasmapheresis and ultraviolet light have been utilized in extreme cases. Liver transplantation is indicated for patients with uncontrolled itching.
When the diagnosis of PBC is made, a bone mineral density scan (DEXA scan) should be performed to determine if a patient has osteoporosis or bone thinning. In many cases, doctors may suggest that their patients start exercising if possible, stop smoking and take calcium with vitamin D. Hormone replacement therapy (estrogen) is safe in PBC and is recommended where appropriate. When osteoporosis is evident, therapy with medications such as Fosamax® (alendronate) and Actonel® may be helpful in PBC.
- Fat-soluble vitamin deficiencies
As PBC progresses, some patients lose the ability to absorb fat-soluble vitamins A, D, E and K. Measurement and replacement of these vitamins is recommended.
- Undiagnosed and Untreated PBC
Undiagnosed and untreated PBC can lead to liver cirrhosis, which can then have complications like Esophageal varies, ascites, hepatic encephalopathy or hepatocellular carcinoma. Patients with PRBC who have advanced fibrosis or cirrhosis should undergo EGD for screening of varies.
- Fatigue is the most common and clinically relevant complication in PBC
- Obeticholic Acid
Obeticholic Acid is now an FDA approved medication for for PBC in patients who are intolerant to UDCA or have inadequate response to UDCA. We should add a section on Fibrates as an investigational drug for use in PBC.
- Liver Transplantation
For those people who reach the late stages of PBC, liver transplantation may be considered. The signs and symptoms of liver failure, which may or (depending on the specific aspects of the symptoms / disease) may not prompt consideration of any potential role for liver transplant, are the following:
- Persistent jaundice
- Fluid retention with swelling of hands, feet and abdomen
- Variceal bleeding (internal bleeding from swollen intestinal blood vessels, commonly in upper stomach and esophagus)
- Bone thinning and/or fractures
- Hepatic coma or encephalopathy
The survival for PBC patients after liver transplantation is excellent although some patients may develop recurrent PBC over time. With better understanding of the nature of PBC and its complications, many patients will have improved outcomes.
Author(s) and Publication Date(s)
Malav Parikh, MD, SUNY Downstate Health Sciences University, Brooklyn, NY – Updated July 2022.
Malav Parikh, MD, SUNY Downstate Health Sciences University, Brooklyn, NY – Updated June 2021.
Young-Mee Lee, MD, and Marvin Jose Lopez, MD, New England Medical Center, Boston, MA – Published December 2004. Updated January 2010.
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