A 24-year-old woman with a 2-year history of scleroderma with Raynaud's phenomenon visited our rheumatism center in 1998 due to pain and swelling of the joints of both hands and wrists. She had also been suffering from heartburn and dysphagia. Esophageal manometry revealed no peristalsis or contraction of the lower esophagus, and poor relaxation of the lower esophageal sphincter (Figure 1). Omeprazole (Losec ) and cisapride (Prepulsid ) were prescribed with diltiazem (Herben ) and D-penicillamine until 1999.
In 2001, she revisited our hospital at 28 weeks' gestation. And, at 34 weeks' gestation, although her condition remained relatively stable, we noted a decreased fetal movement on the ultrasound scan. A week later, she was admitted to our hospital due to premature rupture of membrane. On examination at admission, generalized pitting edema and albuminuria were found. An intrauterine fetal death was diagnosed by ultrasound scan, and plans were made to terminate the pregnancy.
The day after her admission, intractable vomiting and retching developed, which led to approximately 300 mL of hematemesis. She was pale and moderately dehydrated, though her blood pressure (130/90 mmHg) was within the normal range and her heart rate (106/min) slightly increased. Her hemoglobin level had fallen from 9.7 g/dL to 7.6 g/dL within 12 hours, but the other blood counts, prothrombin time, partial thromboplastin time, electrolytes and liver enzymes were normal.
Despite conservative management and blood transfusion, she continued to bleed. Therefore, an emergency upper gastrointestinal endoscopy was performed, which revealed large blood clots in the distal esophagus and stomach. After removing the blood clots, a Mallory-Weiss tear was discovered near the EG junction (Figure 2). The stomach and duodenum appeared normal. Mucosal ligation and hypertonic saline injection were performed for hemostasis.
Two hours after endoscopy, she developed a generalized tonic-clonic type seizure. Her airway was maintained and oxygen was supplied. Diazepam (Valium ) and magnesium sulfate were given intravenously. Her blood pressure increased from 130/90 mmHg to 150/100 mmHg and her pulse rate normalized. An urgent Caesarean section was performed for eclampsia. Post-operatively, she experienced 5 further seizures. Diffuse brain swelling and slightly low-density lesions in the external capsules and in the subcortical white matter were found by CT due to hypertensive encephalopathy. Magnesium sulfate and 20% mannitol were administered by intravenous injection for 3 days.
A total of 10 units of blood were transfused during her 9 day hospital stay. At discharge, there was no more evidence of hemorrhage, and her condition was totally stabilized. She remained stable without further manifestations of gastrointestinal hemorrhage over the following 8 months, when she was lost to follow-up.