We report a case of an 82-year-old man admitted to the hospital with melaena and dizziness on standing.
He had a past medical history of SSc diagnosed 3 years before and pulmonary fibrosis. He was on prednisolone and azathioprine. There was no history of liver disease or alcohol abuse.
On examination his blood pressure of 95/65 mmHg, pulse 90/min. He had bilateral fine inspiratory crackles. The abdomen was soft and non-tender. Rectal examination revealed melaena.
White blood count was 12,700/mm3, haemoglobin 10.9 g/dl, platelets 186,000/mm3 and MCV 99.4 fl. Blood urea was 12.5 mmol/l, creatinine 68 μmol/l, electrolytes were normal, glucose 6.3 mmol/l. Serum alanine aminotransferase was 12 u/l, bilirubin 12 μmol/l, γ-glutamyl transferase 54 u/l, alkaline phosphatase 35 u/l, total protein 52 g/l, albumin 27 g/l and C-reactive protein 12. Coagulation studies were normal. Factor V Leiden mutation was not present. Hepatitis viral serology and autoantibody screen were negative and α-1-antitrypsin was normal. Ferritin was 103 μmol/l, caeruloplasmin was 0.25 μmol/l and α-fetoprotein was 0.9 kU/L.
Urgent gastroscopy revealed oesophageal varices, fresh blood and clots in the stomach. During the procedure the oesophageal varices started bleeding. Haemostasis was not achieved despite sclerotherapy and therefore a Sengstaken tube was inserted. The patient was admitted to the High Dependency Unit and commenced on intravenous glypressin, antibiotics and was transfused four units of blood. The following day a repeat gastroscopy showed two columns of grade II oesophageal varices, two oesophageal ulcers (probably secondary to sclerotherapy) and mild portal gastropathy.
Ultrasound scan of the abdomen revealed a small liver with coarse texture and no focal lesion. Hepato-portal flow was demonstrated in the portal vein. The common bile duct was of normal caliber. The spleen was enlarged (bipolar length 12.8 cm). A moderate amount of free peritoneal fluid was noted. CT scan confirmed the absence of portal vein thrombosis.
The patient was discharged three weeks following admission on propranolol 40 mg twice daily, azathioprine 50 mg twice daily, spironolactone 100 mg once daily, prednisolone 7.5 mg once daily, lansoprazole, vitamin D and calcium. He had two further endoscopies, one and five weeks following discharge and his varices were successfully treated with banding. One month following discharge he had a liver biopsy. There was no evidence of any excess inflammation and no features to suggest cirrhosis or drug-induced liver disease. Overall this was essentially normal liver tissue.
Taking into account the liver histology and the absence of evidence of portal vein thrombosis on imaging we concluded that the patient had IPH. We decided not to proceed to portal pressure measurement because this would not contribute to the management of the patient.
Two months following discharge the patient was reviewed and was well. He had not had any further episodes of haematemesis or melaena. He had normal full blood count and biochemistry results.