Primary biliary cholangitis in patients with systemic sclerosis: Unmasking the true face of Reynold's syndrome

Abstract

Aim of the workHepatic involvement is uncommon in patients with SSc and often associated with primary biliary cholangitis (PBC) an association known as 'Reynold's syndrome'. Anti-mitochondrial antibody (AMA) and anti-centromere antibody (ACA) indicate increased PBC risk in SSc patients. This study aimed to evaluate the relationship between SSc and PBC, and also associated antibodies. Patients and methodsIn this study, 128 SSc patients with diffuse cutaneous (dcSSc n = 59) and limited cutaneous (lcSSc n = 69) subtypes were enrolled. PBC was diagnosed with any 2 of the following: cholestasis based on alkaline phosphatase elevation, presence of AMA and histologic evidence of destruction of interlobular bile ducts and nonsuppurative destructive cholangitis. ResultsThe mean age of the patients was 54.8 ± 13.2 years and disease duration 2.4 ± 1.4 years and they were 106 (82.8%) females and 22 males. ANA was positive in 80.4% of all patients, anti-Scl-70 in 36.7% and ACA in 37.5%. PBC was present in 8 patients (4 in each subtype). In patients with PBC, ACA was positive in 75% compared to 35% in those without (p = 0.03). The gamma-glutamyltransferase (GGT) and direct bilirubin were significantly higher in those with Reynolds syndrome (57.5 ± 26.9 U/L and 0.3 ± 0.1 mg/dl) compared to those without (19.7 ± 9.7 U/L and 0.19 ± 0.1 mg/dl; p < 0.001 and p = 0.02 respectively). A significant relation was found between AMA-M2 with AMA and ACA (p = 0.02). ConclusionAutoantibodies may provide information about organ involvement in SSc. AMA, AMA-M2, and ACA may predict hepatic disease in asymptomatic SSc patients with elevated GGT levels.