Segmental portal hypertension in mixed connective tissue disease

A 64-year-old Japanese woman was diagnosed with mixed connective tissue disease based on the clinical evidence; myositis with infiltration of CD45RO+ T cells and histiocytes, interstitial pneumonia, isolated high titer of anti-U1RNP antibody and hypocomplementemia. Endoscopic examination revealed gastric varices due to isolated splenic vein obstruction. Antiphospholipid antibody, antilymphocyte antibody, anti-U1RNP antibody and miscellaneous pathogens from the intestines were possible causes for obstruction of the splenic vein.