Portal vein thrombosis in adult-onset Still's disease: a case report and literature review

Abstract

Adult-onset Still's disease (AOSD) is a systemic inflammatory disease in which a variety of thrombotic events may occur. However, to our knowledge, portal vein thrombosis (PVT), a potential cause of gastrointestinal bleeding, has not been described in the English literature. A previously well 47-year-old man diagnosed with AOSD showed a transient increase in serum transaminase level. A careful observation led to a prompt discovery of a re-increase in serum transaminase level and PVT that involved an entire left portal vein plus parts of supramesenteric vein. PVT resolved completely in 6 months, and the patient remained well for at least 1 year receiving anticoagulants plus an immunosuppressant that selectively inhibits guanosine monophosphate synthesis in the purine metabolism pathway. Thrombotic events, if not lethal, deteriorate the quality of life in AOSD patients. This report illustrates the spectrum of AOSD and underlines the need to include PVT in differential diagnosis if serum liver enzyme levels fluctuate.