Clinical Relevance of Liver Involvement in the Clinical Course of Systemic Sclerosis

Liver involvement in systemic sclerosis (SSc) is rare. We evaluated the prevalence of liver fibrosis and hepatic autoimmunity in SSc patients in a retrospective observational cohort (97 SSc or mixed connective tissue disease with sclerodermic manifestations patients undergoing transient elastography, evaluating liver stiffness (LS) and controlled attenuation parameter (CAP), due to clinical indications along with biochemistry assessments and major antibodies associated to liver autoimmunity). Among them, 11 had LS >= 7.5 kPa and 5 showed an LS compatible with cirrhosis (LS >= 12.5 kPa). Predictors of LS >= 7.5 fibrosis were alcohol consumption (>14 or >7 alcoholic units/week for men and women, respectively), waist circumference (>102 or >88 cm for men and women, respectively), elevated alkaline phosphatase, and anti-La and anti-mitochondrial antibody (AMA) positivity. Six patients had CAP values compatible with severe steatosis (>= 280 dB/m). Waist circumference, body mass index and diabetes mellitus were significant predictors of steatosis. Out of 97 patients, 19 were positive for AMA, 4 for anti-Sp100, 1 for anti-Gp210 and 7 were diagnosed with primary biliary cholangitis. Among SSc patients, hepatic fibrosis biomarkers and AMA prevalence are relatively high, suggesting the opportunity of performing a transient elastography and a screening for hepatic autoimmunity at diagnosis and/or during disease progression.

Automated summary

This study evaluated the prevalence of liver fibrosis and hepatic autoimmunity in systemic sclerosis (SSc) patients. The results showed that some patients had liver fibrosis and steatosis, which were associated with alcohol consumption, waist circumference, biochemistry markers, and specific antibody positivity. The study suggests the importance of liver examination and autoimmune screening during diagnosis and disease progression.