BetaEntity Annotation Prototype
← Back to institutions

Annotated abstract

P.165 Prevalence and characterization of liver disease in systemic sclerosis: a multicentric cohort study

jsrd · 2026-06-05 · canonical JSON source

7 visible annotations · policy: published · automated confidence ≥ 75.00%

Document resource

Introduction Systemic Sclerosis (SSc) is a complex, immune-mediated connective tissue disease, characterized by vasculopathy and fibrosis of the skin and internal organs, resulting in significant morbidity and mortality. While pulmonary, cardiac, and gastrointestinal involvement are well studied, hepatic manifestations are less commonly characterized and often underrecognized. Among them, autoimmune liver diseases such as primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) are the most reported. This study aimed to characterize liver involvement in SSc.Material and Methods Retrospective cohort study including consecutive patients fulfilling the ACR/EULAR 2013 Classification Criteria for SSc, followed at the Rheumatology Departments of Unidade Local de Saúde (ULS) de Coimbra and ULS Região de Aveiro. Clinical records, laboratory data, and imaging studies were reviewed to identify cases of liver disease. Patients with autoimmune hepatic disease (PBC and AIH) were diagnosed based on compatible autoantibody profiles and biochemical abnormalities, and either liver biopsy findings or sustained laboratory changes consistent with chronic hepatic involvement. Descriptive and statistical analysis were performed, with p-value less or equal to 0.05 considered statistically significant.Results A total of 112 patients with SSc were included, 83.0% female, mean age 63.5 ± 12.6 years ( table 1). Limited Cutaneous SSc (lcSSc) was predominant (69.6%). Liver disease/abnormalities were identified in 23.2% of patients. Ten cases were attributed to autoimmune liver disease: 8 patients (7.1%) had PBC and 2 (1.8%) had AIH. Additionally, 8 patients presented abnormal liver autoantibodies (4.5%) or elevated liver enzymes without a definitive hepatic diagnosis (2.7%). Hepatic steatosis was identified in 10 patients (8.9%), 9 of whom had metabolic risk factors. Among PBC patients, all were female with biochemical evidence of cholestasis; 25% also presented cytolysis. Most (87.5%) had lcSSc, and one patient had SSc sine scleroderma. Anticentromere antibodies were present in 87.5%, while one patient was positive for anti-RNA polymerase III. Only one patient was antimitochondrial antibodies negative, with the diagnosis confirmed by liver histology. The mean time from first SSc manifestation to PBC diagnosis was 10.4 ± 5.5 years, with all PBC diagnosed after SSc. Cirrhosis was documented in two patients: one with PBC and one with AIH. No significant differences were found between PBC patients and those without liver involvement regarding age or SSc features.Conclusions In our cohort, the most common liver abnormalities were metabolic steatosis and autoimmune-related diseases, particularly PBC. Early recognition and multidisciplinary management are essential to optimize care. Larger studies are needed to clarify the clinical impact of hepatic disease in SSc.Abstract P.165 Table 1Demographic, clinical, and immunological features of systemic sclerosis patients