(LE 5, GR C1) Rifampicin is effective against dermal pruritus in PBC patients. (LE 1a, GR B) Osteoporosis is frequently observed in patients with PBC because intestinal absorption of fat-soluble vitamins is disturbed due to reduced secretion of bile acids, and PBC is common in middle-aged and postmenopausal women. For prevention of osteoporosis, abundant oral intake of calcium (1 to 1.2 g/day) and vitamin D (plentiful in fish and mushrooms) and weight-bearing exercise are recommended, and medical treatment should be given if necessary. Bisphosphonates, bioactive
vitamin D3 agents, and vitamin K2 are prescribed. Among bisphosphonates, alendronate improves bone density more than etidronate. Nevertheless, there is no evidence BI 2536 chemical structure that alendronate suppresses bone fracture. Administration once weekly is preferable to daily administration. Alendronate is contraindicated for cases with esophageal stenosis due to sclerotherapy for esophageal varices. GS 1101 Vitamin D3 and vitamin K2 formulations have frequently been prescribed for PBC in Japan. Both drugs have been proven to be effective for osteoporosis itself, and are regarded as Grade B in guidelines for the prevention and treatment of osteoporosis.
Recommendations: It is desirable to start treatment for the prevention of fractures in cases with a T score below −1.5. (LE 4, GR C1) Alendronate improves bone density in PBC patients. (LE 1b, GR A) Although there is scarce evidence in PBC patients, vitamin D3 and vitamin K2 formulations can be effective for osteoporosis. (LE 1b, GR C1) Hypercholesterolemia is likely to develop in PBC due to cholestasis. Xanthoma is seen around the eyelids. No specific treatment for hypercholesterolemia in PBC is required in most cases,
while bezafibrate is expected to be effective for both PBC and hypercholesterolemia. Sicca syndrome, a major symptom of Sjögren’s syndrome, is frequently complicated with PBC. The diagnosis of Sjögren’s syndrome should be made by detection of serum anti-SS-A/SS-B antibodies, presence of corneal erosion, and lip biopsy if necessary. Artificial lachrymal fluids are indicated for eye symptoms. If the response is not favorable, pilocarpine hydrochloride and cevimeline hydrochloride hydrate are used under the guidance of ophthalmologists. As for oral symptoms, artificial saliva medchemexpress should be used first, and pilocarpine hydrochloride and cevimeline hydrochloride hydrate can also be prescribed. Recommendations: Cevimeline hydrochloride and pilocarpine hydrochloride may be effective for xerostomia in PBC, although there are no studies evaluating their potential to alleviate the symptoms occurring in PBC patients with concurrent Sjögren’s syndrome. (LE 6, GR B) Patients with PBC frequently experience cholestasis, comorbid autoimmune diseases, and symptoms associated with liver injury and cirrhosis. Prevention and management of these symptoms are required.