Advancing the management of primary biliary cholangitis: From pathogenesis to emerging therapies

Table 1 Extrahepatic conditions associated with primary biliary cholangitis

Condition	Prevalence in PBC (%)
Sjogren’s syndrome	21.4
Raynaud’s phenomenon	12.3
Rheumatoid arthritis-like polyarthritis	5
Systemic sclerosis	3.7
Systemic lupus erythematosus	2
Thyroid disorders	11.3
Autoimmune thyroiditis	9.9
Osteoporosis	21.1
Celiac disease	1
Chronic bronchitis	4.6

PBC: Primary biliary cholangitis.

Table 2 Effect of primary biliary cholangitis therapy on fatigue and pruritus

Therapy	Effect on fatigue	Effect on pruritus
UDCA[43,45]	No significant improvement2	Often insufficient2
OCA[70,92]	No improvement2	Worsening or new-onset, dose-dependent3
Fibrates[84]	Mild to moderate improvement1	Clinically meaningful reduction1
Elafibranor[95]	Mild to moderate improvement1	Clinically meaningful reduction1
Seladelpar[98]	Mild to moderate improvement1	Significant reduction1
Budesonide[108]	Little or no documented effect2	Marginal or no specific effect2
IBAT inhibitors (e.g., linerixibat, maralixibat)[107]	Data limited	Marked improvement1

¹Improvement.

²No meaningful change.

³Worsening or increase.

UDCA: Ursodeoxycholic acid; OCA: Obeticholic acid; IBAT: Ileal bile acid transporter.