Guide for Mitigating Pruritus in Primary Biliary Cholangitis

Primary Biliary Cholangitis (PBC) is a liver disease that often causes itching, known as cholestatic pruritus. Healthcare providers manage this itching through a stepwise approach, starting from non-pharmacologic to pharmacologic therapies.

Basic Skincare and Supportive Measures

Fundamental skin care is recommended for all PBC patients to alleviate irritation from scratching.

First-line Pharmacotherapy: Cholestyramine

Cholestyramine, a bile acid sequestrant, is typically used initially for mild pruritus. It binds bile acids in the gut to reduce systemic levels that contribute to itching. However, it may cause side effects like nausea, constipation, and abdominal bloating, limiting its tolerability.

Second-line Agents: Rifampicin and Bezafibrate

Rifampicin (150–300 mg twice daily) can be effective in those not responding to cholestyramine. It acts by inducing hepatic enzymes and altering bile acid metabolism but requires monitoring for liver toxicity, hemolytic anemia, and kidney effects.
Bezafibrate, a fibrate drug, has shown significant reductions in itching by improving cholestasis and has been supported by randomized-controlled trials specifically for PBC-related itch relief.

IBAT (Ileal Bile Acid Transporter) Inhibitors – Emerging Targeted Therapies

Linerixibat (brand name Livdelzi) is a novel IBAT inhibitor designed to block bile acid reabsorption in the intestines, directly targeting the cause of cholestatic pruritus. Clinical trials have shown about 56% of PBC patients achieving clinically meaningful itch reduction, with improvements starting as early as two weeks and sustained over 24 weeks.

Other Treatments

Antihistamines and opioid-based therapies are sometimes used but provide only partial relief and have significant side effects, thus they are not the mainstay of treatment.
SSRIs (Selective serotonin reuptake inhibitors) can be a benefit for people with PBC itch, as they work in the same pathway that leads to itching. However, they may require a strong dose to affect itch, and the mood-altering effects may not be tolerable in some people.
PPAR antagonists and PPAR agonists may help improve the quality of life for people dealing with PBC itch, and they may be an option for some people to both reduce itching and manage PBC.
Naltrexone is a 3rd-line agent used to counteract central opioid pathways related to itching in PBC.

Summary

In managing PBC itch, a stepwise approach is used, starting with cholestyramine, followed by rifampicin or bezafibrate for refractory cases, and increasingly with IBAT inhibitors like linerixibat that target bile acid pathways more directly and effectively. Monitoring for drug side effects and tailoring therapy based on symptom severity and tolerance is standard clinical practice. If dealing with itchiness as a result of PBC, it's recommended to talk with a healthcare provider to find the best strategy for management.

Expanding on the Treatment Options

In addition to the aforementioned therapies, dietary modifications and lifestyle changes can alleviate itching in PBC patients. This includes maintaining a balanced diet rich in fruits, vegetables, and lean proteins, while minimizing saturated fats, sodium, and cholesterol intake.
Regular exercise, particularly mild to moderate cardiovascular activities, aids in overall health and wellness, contributing to better management of PBC symptoms.
Some digestive health issues may co-occur with PBC, necessitating close monitoring and specific therapies and treatments. Probiotics, for instance, could help address any digestive imbalances.
Eye-health concerns affecting PBC patients are managed similarly to the general population, with routine visits to ophthalmologists for regular check-ups.
Hearing loss, a common side effect of long-term PBC treatment, is often reversible with appropriate intervention. Regular audiology screenings are advised.
Skin conditions such as psoriasis, a potential autoimmune disorder, should be treated with appropriate topical or systemic therapies to prevent worsening skin symptoms.
In terms of mental-health, individuals with PBC are at an increased risk for depression and anxiety. Fitness and exercise, along with counseling or therapy, can help manage these psychological impacts.
Men's health and sexual health concerns might arise during PBC treatment, necessitating open communication with healthcare providers to address any relevant issues.
Women's health, particularly breast-cancer risk and reproductive health matters, can further complicate PBC management. Responsible family planning is emphasized, with additional screenings as recommended by healthcare providers.
Learning about and managing parenting responsibilities might be challenging for those dealing with chronic diseases like PBC. Support groups, medical literature, and healthcare professionals can offer valuable guidance on how to balance being a parent while managing a chronic condition.
Cardiovascular health is crucial for overall well-being during PBC treatment, as chronic liver diseases increase the risk of heart conditions. Regular screenings and lifestyle modifications, such as quit smoking, maintaining a healthy weight, and practicing stress management techniques, are essential in managing cardiovascular health.
Medicare provides essential coverage for PBC-related treatments, but managing expenses and understanding the coverage provided is essential to ensure access to necessary therapies and treatments.
Rheumatoid-arthritis, an autoimmune disorder, can develop concurrently with PBC, necessitating shared care among specialists like rheumatologists and liver specialists to monitor and manage disease progression.
Controlling chronic-kidney-disease is vital in managing PBC, with appropriate therapies and treatments to prevent complications and maintain kidney function.
COPD, or chronic obstructive pulmonary disease, can co-occur with PBC and require respiratory-condition-specific therapies and treatments to prevent worsening lung function.
Type-2-diabetes is a common comorbidity among individuals with PBC, highlighting the importance of close monitoring and appropriate management strategies for blood sugar levels.