A case of systemic lupus erythematosus (SLE) associated with idiopathic portal hypertention (IPH) was reported. In February 1969, a 48 years old woman was admitted because of general fatigue and polyarthralgia. She had polyarthritis, butterfly rash, splenomegaly, leucopenia and antinuclear antibody. She was diagnosed as SLE, and treated with paramethason. She responded rapidly to the treatment except splenomegaly. In 1974, she developed hair lossand thrombocytopenia. In 1977, esophagial varices

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... were demonstrated by upper gastrointestinal X-ray examination. In April 1978, she was transfered to our hospital. The examinations of liver scan, celiacangiography and hepatic venography were performed, which demonstrated marked splenomegaly and esophagial varices due to portal hypertention. She was therefore diagnosed as SLE associated with IPH, and esophagial transection was done. In 1983, the patient was admitted because of nausea and vomitting. In October 1983, she was died due to hematoemesis. At autopsy there was a marked splenomegaly. Pathological findings of the liver were compatible with characteristic features of IPH. Complication of IPH in SLE is rare and pathogenesis of IPH is unknown. It might be suggested that the common immunological mechanism playes a role on the etiology of both SLE and IPH because IPH developed at the onset of SLE in this case.