Massive gangrene of the stomach due to primary antiphospholipid syndrome: report of two cases.

Antiphospholipid syndrome (APS) is a systemic autoimmune disease, which may be primary or secondary to other autoimmune diseases. It produces thrombosis of arteries and veins of any caliber, and no organ is immune to its insult. This report describes two cases of massive gastric gangrene due to primary APS, which presented in a span of 2 years. In the first case a multiparous, 40-year-old woman presented with acute abdominal pain, hematemesis, and progressive abdominal distension, and was in azotemia and shock. A laparotomy revealed gangrene of the stomach without any other organ involvement. She was managed with a total gastrectomy and esophagojejunal anastomosis. Postoperative serology revealed a persistent elevation of anticardiolipin antibody with no other apparent predisposing cause. The histopathological examination of the specimen revealed characteristic extensive intramural vascular thrombosis without inflammatory changes in the vessel wall, confirming antiphospholipid syndrome. The second patient was a primiparous, 26-year-old woman who had severe abdominal pain in the first trimester followed by shock. An exploratory laparotomy revealed massive gangrene of the stomach with complete loss of the posterior wall and hemoperitoneum. She also underwent a total gastrectomy with esophagogastric anastomosis and was later managed in the intensive care unit, where she succumbed within 8 days. Her serology showed a highly elevated anticardiolipin antibody titer, and histopathological examination of the stomach revealed characteristic intramural vascular thrombosis without inflammatory cellular infiltrate in the vessel wall. Patients undergoing a total gastrectomy following acute gastric necrosis have very high mortality (50%-80%). Its association with APS is rare and it has not been previously reported. The combination is a formidable challenge to the physician and a dangerous disease for the patient. The rarity of the condition and its grave prognosis is highlighted.