Background The catastrophic antiphospholipid antibody syndrome (CAPS) is an uncommon disorder, characterised by widespread micro and macrovascular changes due to intravascular thrombosis. We report five cases of CAPS occurred at our department over the past 7 years.
Methods All patients were females between 13 and 49 years, all having SLE; two of them had no identifiable precipitant factor, two underwent major surgery, and the last one had a respiratory tract infection and a SLE flare as probable precipitant events; four women had deep vein thrombosis and the other one a peripheric arterial thrombosis; stroke occurred in 4 patients, one of them concomitantly in the right and left middle cerebral arteries terrritories; intraalveolar haemorrhage occurred in two cases and one patient developed ARDS.
Treatment options were different for each patient and all had steroids and anticoagulation. Two had cyclophosphamide synchronised with plasmapheresis.
Results Unfortunately 4 of the patients died, the only survivor was treated with steroids and anticoagulation therapy.
Conclusion CAPS is a rare event in our cohort of patients with antiphospholipid antibody syndrome (6,9%), having a high mortality rate. Most of the times it was possible to identify a precipitant factor, namely major surgery or infection. Agressive treatment must be startted as soon as possible.
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