Concomitant Autoimmune Diseases

32% of patients with primary biliary cholangitis (PBC) also have another autoimmune disease1,a

Select Association with Autoimmune Diseases in Patients with PBC

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Any of the following Raynaud syndrome Rheumatoid arthritis Sjögren’s syndrome Autoimmune thyroid disease Systemic lupus erythematosus Scleroderma Polymyositis
32.0% 12.0% 10.0% 10.0% 9.0% 3.0% 2.0% 0.6%
13.0% 2.0% 8.0% 0.5% 1.0% 0.5% 0.0% 0.1%
PBC cases (n=1032)
Control cases (n=1041)

 Relatives of PBC cases (n=1032)Relatives of control cases (n=1041)

Any of the following 32.0% 13.0%
Raynaud syndrome 12.0% 2.0%
Rheumatoid arthritis 10.0% 8.0%
Sjögren syndrome 10.0% 0.5%
Autoimmune thyroid disease 9.0% 1.0%
Systemic lupus erythematosus 3.0% 0.5%
Scleroderma 2.0% 0.0%
Polymyositis 0.6% 0.1%
  • The most frequent autoimmune conditions found in patients with PBC were Raynaud syndrome, rheumatoid arthritis, Sjögren’s syndrome, and autoimmune thyroid disease1,2
  • Prevalence rates for Raynaud syndrome and Sjögren’s syndrome were 6 and 20 times higher, respectively, in patients with PBC compared with controls (P<.0001)1
  • Significantly higher prevalence of systemic lupus erythematosus, scleroderma, and autoimmune thyroid disease were seen in patients with PBC compared with controls (P<.0001 for all 3 comparisons)1
  • The frequency of a prior diagnosis of rheumatoid arthritis did not differ between patients with PBC (10%) and controls (8%) (P =.129)1

aIn a study of 1032 patients, the NIH enrolled patients with PBC 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.1

References:  1. 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.  2. 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.