Systemic sclerosis and primary biliary cholangitis: An overlapping entity?

Abstract

Systemic sclerosis (SSc) is a complex autoimmune disease that may lead to skin and internal organ fibrosis. Based on skin involvement, two subsets of the disease are recognized (limited cutaneous SSc and diffuse cutaneous SSc). The new 2013 American College of Rheumatology/European League against Rheumatism classification criteria allow to identify SSc patients at the early stage of the disease that allows new research avenues. The aetiology of the disease is still unknown, but it has an important autoimmune basis and its association with other autoimmune diseases has been reproducibly reported. Among them, primary biliary cholangitis is considered the most common liver disease in SSc. The aim of this review is to provide an overview on recent findings about SSc associated to primary biliary cholangitis. Although the aetiology of the two diseases is still unknown, data suggest that these two disorders share the expression of fibrogenic cytokines, involved both in generation and function of T lymphocytes subpopulation (Th17 cells) and regulatory T lymphocytes. In addition, the relationships between SSc and primary biliary cholangitis may be closer as suggested by the presence of primary biliary cholangitis–specific antibodies in SSc patients and vice versa. Recent findings confirm a prevalence of overt primary biliary cholangitis in about 2% of SSc population, in particular in patients with limited cutaneous SSc and positive anticentromere antibodies. The prevalence increases if also patients with only primary biliary cholangitis–specific antibodies are considered. Data regarding SSc prevalence in primary biliary cholangitis patients have also been recently clarified. Altogether, stimulating results are moving the field forward regarding the relationships of these two autoimmune and fibrotic disorders that may belong to an overlapping entity.