Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening condition of antiphospholipid syndrome (APS). Treatment and management of CAPS remain challenging and the mortality rate is approximately 50% among cases. We describe a successfully treated case of a CAPS patient who had undergone massive bowel resection due to obstruction of superior mesenteric artery.
A 40-year-old male patient was admitted to our hospital with acute abdominal pain, melena, and a history of deep vein thrombosis in both legs for over 10 years, there was no previous diagnosis of APS.
The patient was diagnosed as CAPS with bowel necrosis due to obstruction of superior mesenteric artery based on the presence of antiphospholipid antibodies, computed tomography scan, and histopathological examination.
Emergency surgery was performed to remove approximately 6 meters of the necrotic small intestine, of which the length of the remaining small intestine was 40 cm from the duodenum and 80 cm from the ileocaecal valve. Anticoagulants were prescribed with low molecular weight heparin. After discharging, APS was managed with direct oral anticoagulants (DOACs) for secondary thromboprophylaxis because the patient was unable to reach target International Normalized Ratio (INR) with vitamin K antagonists (VKAs).
During 24 months of follow-up until now, the patient did not develop new thrombosis or relapse CAPS and his state remained stable.
While VKAs is amongst the most important and fundamental treatment, physicians should be aware that VKAs are absorbed via the small intestine. For CAPS cases who had undergone massive bowel resection, DOACs is a reasonable alternative which has been found to be as safe and effective as VKAs in terms of thrombosis prevention.