Additional Associations and Complications

Associations and potential complications of primary biliary cholangitis (PBC) may extend beyond symptoms of pruritus, fatigue, and depression and may include the following:

  • Thyroid disease1
  • Systemic sclerosis including its variant, CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia)1,2
  • Celiac disease1
  • Osteoporosis2
  • Hyperlipidemia2
  • Renal tubular acidosis2
  • Malabsorption of fats, resulting in steatorrhea and fat-soluble vitamin deficiency (uncommon except in the late stages of the disease)2
  • Portal hypertension2
  • Ascites2
  • Variceal hemorrhage2
  • Hepatic encephalopathy2
  • Jaundice2
  • Liver failure2

Other potential complications


  • Additionally, as many as one-third of patients with PBC are diagnosed with another autoimmune disease. The most frequent concomitant diseases include Raynaud syndrome, rheumatoid arthritis, autoimmune thyroid disease, and Sjögren syndrome5,6

aIn a study of 1032 patients, the NIH enrolled patients from 23 tertiary referral centers for liver diseases in the United States. Random digit-dialed controls (n=1041) were matched for sex, age, race, and geographical location. The study evaluated associations between the rates of comorbidity in affected individuals.3

References:  1. Al-Harthy N, Kumagi N. Natural history and management of primary biliary cirrhosis. Hepat Med. 2012;4:61-71. doi:10.2147/HMER.S25998.  2. Crosignani A, Battezzati PM, Invernizzi P, Selmi C, Prina E, Possa M. Clinical features and management of primary biliary cirrhosis. World J Gastroenterol. 2008;14(21):3313-3327. doi:10.3748/wjg.14.3313.  3. Jacoby A, Rannard A, Buck D, et al. Development, validation, and evaluation of the PBC-40, a disease specific health related quality of life measure for primary biliary cirrhosis. Gut. 2005;54(11):1622-1629. doi:10.1136/gut.2005.065862.  4. Newton JL, Hollingsworth KG, Taylor R, et al. Cognitive impairment in primary biliary cirrhosis: symptom impact and potential etiology. Hepatology. 2008;48(2):541-549. doi:10.1002/hep.22371.  5. Gershwin ME, Selmi C, Worman HJ, et al; USA PBC Epidemiology Group. Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview-based study of 1032 patients. Hepatology. 2005;42(5):1194-1202. doi:10.1002/hep.20907.  6. Selmi C, Bowlus CL, Gershwin ME, Coppel RL. Primary biliary cirrhosis. Lancet. 2011;377(9777):1600-1609. doi:10.1016/S0140-6736(10)61965-4.