Abstract: Objective To improve the understanding of the clinical characteristics of connective tissue disease with portal venous system thrombosis. Methods The clinical features of 16 patients of connective tissue disease with portal venous system thrombosis were reviewed. Results Among 16 patients, 6 cases were SLE, followed by 2 case of APS, 2 case of RA, 2 cases of pSS; 1 case of MCTD, 1 case of CREST syndrome and 2 cases of unspecific CTD. Two cases were male and 14 cases were female with the median age of 48 years. Eleven cases had portal hypertension, 4 patients had deep venous thrombosis. The most common thrombosis site was portal vein (9 cases). Acute PVST could be manifested with acute abdominal pain or distention, chronic PVST could have chronic incomplete intestinal obstruction, or hematemesis/melena related to portal hypertension. Conclusion Portal venous system thrombosis can be the initial or the complicated sign in the connective tissue disease, physicians should be alert to this disease.

Key words: connective tissue disease, portal venous system, thrombosis, portal hypertension