Extrahepatic manifestations of primary biliary cholangitis (PBC) occur in 73% of patients, with Sjögren’s syndrome, thyroid dysfunction, and systemic sclerosis being the most common, according to a literature review from Sara Chalifoux, MD, and her associates.

Sjögren’s syndrome occurs in 3.5%-73% of PBC patients, usually presenting with dry eyes and oral complications. Sjögren’s treatment in PBC patients involves symptom management associated with exocrine gland infiltration.

Thyroid diseases are present in 5.6%-23.6% of PBC patients. Hashimoto’s thyroiditis is the most common hypothyroidism in PBC patients, presenting with symptoms such as constipation, bradycardia, oligomenorrhea, and inability to concentrate. Grave’s disease is the most common hyperthyroidism, presenting with symptoms such as palpitations, tremulousness, heat intolerance, and weight loss.

Systemic sclerosis occurs in 1.4%-12.3% of PBC patients. Multiple studies found that limited cutaneous systemic sclerosis was more common in PBC patients than was the diffuse form of the disease.

Other diseases that may have a connection to PBC but lack solid, compelling evidence to make a firm association include rheumatoid arthritis, systemic lupus erythematosus, and celiac disease. While many PBC patients have irritable bowel disorder, there is no significant association between the two conditions.

“The patient care team should include practitioners in rheumatology, endocrinology, pulmonology, and cardiology when indicated. Patients should follow up regularly with their primary care physicians. As some of these extrahepatic manifestations can lead to diseases with a poor prognosis, vigilant screening and close follow-up will lead to prompt identification and treatment,” the investigators noted.

The investigators reported no financial conflicts of interest.

Find the full study in Gut and Liver ( doi: 10.5009/gnl16365 ).