Spontaneous hepatic encephalopathy is very uncommon in extrahepatic portal venous obstruction (EHPVO), except when large spontaneous portosystemic shunts have developed. We present a case of a young female who was diagnosed as EHPVO and now presented as overt hepatic encephalopathy due to spontaneous lienorenal shunt and later she was diagnosed with lupus nephritis.Methods
A 36-year-old female hospitalised in July 2007 with complaints of disorientation and personality changes which were present for last one month. She was diagnosed EHPVO in 1998 when she had left hypochondriac pain, bilateral oedema feet and oedema over the face with a history of infertility and coil embolization for splenic artery aneurysm. On examination, she was conscious, not oriented and focal neurological signs were absent. General and abdominal examination revealed pallor, bilateral pitting oedema feet and splenomegaly. Her investigations revealed anaemia, hypothyroidism, and albumin/globulin ratio reversal. CT abdomen had shown shrunken liver, portal vein thrombosis, coils in splenic artery with splenomegaly and extensive portosystemic collaterals. Gastroscopy revealed antral gastritis, no varices. Liver biopsy revealed portal fibrosis, no cirrhosis.Results
Over 48 hours she responded to anti encephalopathic measures. She was apparently well for three years with thyroid hormone replacement, diuretics and lactulose. She had been hospitalised again in 2010 with anasarca and massive right-sided pleural effusion. She had nephrotic range proteinuria and diagnosed as lupus nephritis by renal biopsy and was started on oral steroids. She had developed second episode of overt encephalopathy which was spontaneous in 2010, and she had responded to medical treatment for encephalopathy.Conclusions
Though she was diagnosed with lupus nephritis many years after the diagnosis of EHPVO, portal vein thrombosis may be due to acquired thrombophilic disorder – antiphospholipid syndrome which was not recognised earlier. Presence of weakly positive lupus antibody, history of infertility, portal vein thrombosis and nephrotic range proteinuria may represent a spectrum of manifestation of antiphospholipid syndrome in this case. Defective acquired fibrinolysis and antiphospholipid syndrome are proposed as a cause of portal vein thrombosis in SLE. Surgical ligation of portosystemic shunt is occasionally of benefit when hepatic encephalopathy is refractory to other treatment.