Primary Biliary Cholangitis and CREST Syndrome: A Rare, Overlapping Presentation With a Review of the Literature

Abstract

CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder often manifesting as a consequence of superimposed autoimmune hepatitis. Herein, we present a case of a 40-year-old female with a past one-year history of hepatitis C presenting with the chief complaints of progressive thickness and tightness of the skin of hands and face and dysphagia for the past three months, along with arthralgia of the hands for the past two months, suggestive of CREST syndrome. Through this case, we intend to emphasize the association between extrahepatic manifestations and the emergence of autoantibodies in patients with hepatitis C virus (HCV) infection and discuss the clinical relevance of the autoantibodies in extrahepatic disorders, in our case, CREST syndrome. It is well-known that chronic HCV infection plays a significant part in the production of non-organ-specific autoantibodies, including antinuclear antibodies (ANA) and smooth muscle antibodies, and organ-specific autoantibodies. Clinicians must be aware of the possibility of such liver damage in patients with systemic sclerosis. Copyright © 2020, Khan et al.