Objectives: We aim to describe a rare case of a young patient with a catastrophic antiphospholipid syndrome (cAPS) who evolved to systemic lupus erythematosus (SLE).Methodology: Case report description.
Case report: A 15 years old girl came to the emergency department with acute peritonitis secondary to ischemic perforation of the sigmoid. Angiotomography was suggestive of a thrombotic occlusion of the upper mesenteric artery. Laboratory tests revealed a positive lupus anticoagulant and antinuclear antibodies. A cAPS diagnosis was determined based on more than three different sites of thrombosis (lung, kidney, spleen and sigmoid) in less than one week with a lupus anticoagulant. She was treated with intravenous methylprednisolone 1 mg/kg/day associated with intravenous heparin followed by intravenous immunoglobulin. She had a very good outcome with a total improvement and was discharged from the hospital after 2 months. After 12 weeks, the lupus anticoagulant remained positive. After two months, a systemic lupus erythematosus was diagnosed. She was treated with increasing dose of prednisone and azathioprine 100 mg/day and hydroxychloroquine were added to the treatment. After 2 weeks, she was asymptomatic. She is currently well, asymptomatic using hydroxychloroquine, azathioprine and warfarin.
Conclusion: This article reports a very unusual presentation which is rarely reported and clinicians should be alert to the possibility that CAPS may be the presenting event for SLE patients, condition known in adults and should also be remembered in pediatric scenario.
Keywords: Systemic lupus erythematosus; antiphospholipid syndrome; catastrophic antiphospholipid syndrome (CAPS).