A 22–year–old woman with a history of systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome on chronic immunosuppression and anticoagulation presented with one week of post–prandial upper abdominal pain and fevers. Her vital signs were unremarkable and her abdominal exam was benign. Abdominal CT revealed multiple gallstones without evidence of cholecystitis. With concerns for choledocholithiasis, she underwent an uncomplicated laparoscopic cholecystectomy with improvement in her abdominal pain and fevers. On post–operative day 4, in the setting of a supra–therapeutic heparin drip, she became hemodynamically unstable and was found to have a large intra–peritoneal bleed. Hemostasis was achieved by splenic artery embolization. Visceral angiography revealed a previously undiagnosed pan–vasculitis with hundreds of pseudo–aneurysms of small, medium, and large mesenteric arteries. The pathology of her gallbladder revealed chronic cholecystitis and active small and medium vessel vasculitis. Given evidence of severe, active vasculitis, she was treated with a course of high–dose cyclophosphamide. Unfortunately, she failed to respond to aggressive therapy and developed recurrent small bowel ischemia from vasculitis. Eight weeks into her hospital course, she became pulseless and unresponsive and was unable to be revived despite prolonged cardiopulmonary resuscitation. Autopsy revealed multiple pseudo–aneurysms and evidence of treated pan–vasculitis involving multiple abdominal vessels.
Hospitalists may face patients with lupus who present with abdominal pain and should recognize that mesenteric vasculitis is a common cause. While lupus mesenteric vasculitis (LMV) affects a minority of SLE patients, it actually accounts for more than half of patients with active SLE who present with acute abdominal pain. LMV is typically a small vessel vasculitis, thought to be secondary to immune–complex deposition in vessel walls and/or thrombotic events caused by antiphospholipid antibodies. Medical management involves high–dose IV steroid therapy, however cyclophosphamide can be used in steroid–refractory patients. Infrequent, severe sequelae of LMV include bowel ischemia and perforation, which often require surgical intervention.
LMV affects a minority of SLE patients, but should be kept high on the differential diagnosis when patients present with acute abdominal pain. Bowel ischemia caused by LMV can lead to perforation and hemorrhage, and has a high mortality of up to 50%.
Figure 1Visceral angiogram showing hundreds of pseudo–aneurysms of small, medium and large mesenteric arteries.