Abstract: Objective This paper aimed too summarize the clinical characteristics of patients with systemic sclerosis (SSc) complicated with primary biliary cholangitis (PBC) and portal hypertension. Methods Twelve patients with SSc complicated with PBC and portal hypertension were hospitalized in Beijing You'an Hospital Affiliated to Capital Medical University between January 2010 and March 2022, and the clinical symptoms, laboratory tests, abdominal imaging and gastroscopy data were analyzed. Results Among the 12 patients, 5 patients went to see doctors for hematemesis and/or melena as the first symptom; 8 patients were diagnosed with SSc earlier than PBC; 10 patients had decreased peripheral blood hemoglobin (HGB) levels, and 9 patients had decreased blood PLT counts; 8 patients had elevated serum GGT and ALP levels, showing cholestasis; 10 patients had electronic gastroscopic findings, suggesting esophageal varices, 12 patients had splenomegaly by CT/ultrasonography, and 9 patients had varying degrees of ascites, 8 patients had formation of collateral circulation. Conclusion The clinical manifestations of patients with SSc, PBC and portal hypertension are similar to patients with PBC alone. The clinical manifestations of portal hypertension are more obvious, and the cholestasis is more obvious than the increase of serum transaminases levles. It might be necessary to actively find PBC, when the SSc is diagnosed. Early diagnosis and early intervention might improve the outcome of patients with those entities.

Key words: Primary biliary cholangitis, Systemic sclerosis, Scleroderma, Gastrointestinal bleeding, Esophageal varices