Primary biliary cirrhosis medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dildar Hussain, MBBS [2]

Overview

Pharmacologic medical therapies for Primary biliary cirrhosis include Immunomodulators,antifibrotics and anticholestatics. The anticholestatic Ursodeoxycholic acid (UDCA) is recommended as the first line medical therapy for PBC.

Medical Therapy

• Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
• Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
• Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
• Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Primary biliary cirrhosis

• 1 Anticholestatics
  • 1.1 Preferred regimen : Ursodeoxycholic acid (UDCA) 13-15 mg PO q12h for 10-21 days (Contraindications/specific instructions)

• 2 Immunomodulator
  • 2.1 Glucocorticoid
    • Preferred regimen :Budesonide 6 to 9 mg per day PO (contraindicated for patients who have hypersensitivity to budesonide)
    • Alternative regimen:Cyclosporine: 5-10 mg PO q24h
• 3 Farnesoid-X-receptor (FXR) agonist
  • 3.1 Obetocholic acid
• 4 Peroxisome proliferator-activated receptor agonist
  • Fibrates

Symptomatic Therapy

1.Pruritus

• 1st line
  • Cholestyramine 4 g/d (before + after breakfast)
• 2nd line
  • Rifampin 150 mg bid
• 3rd line
  • Sertraline (anti-depressant)
• 4th line
  • Naloxone, by an experienced physician
• 5th line
  • Liver transplantation

'2.'Supportive

• UV light,Sunlight

'3.'Emergency

• Plasmapheresis

'4.'Raynauds

• 1st line:Ca channel blockers
• 2nd line:Alternative: prostacyclin and its derivatives, endothelin receptor antagonists and phosphodiesterase inhibitors

'5.'Sicca syndrome

• Dry eyes
  • Artificial tears
• Dry mouth
  • Dental hygiene
    • Dental visit every 3–6 months
• Dry vagina
  • Vaginal lubricants