129 Catastrophic antiphospholipid syndrome and pregnancy: A case report

Abstract

Introduction Association between antiphospholipid antibodies and poor gestational outcomes is recognized for decades. For the diagnosis of Catastrophic Antiphospholipid Syndrome (CAPS) is necessary high clinical suspicion and intensive care support is essential to save the patients in this really catastrophic disorder. Objectives Description of a case report and literature review of CAPS in gestation, looking updates in relation to the early diagnosis and appropriate management of these patients. Methods Case report of CAPS during pregnancy and literature review on Pubmed. Results Z.M.S.S, 33, G5P2, previously diagnosed with primary APS sought care at Hospital Sao Paulo on February 7, 2014, with a 34 weeks pregnancy, presenting hypertension, lower limb edema associated with laboratory findings of proteinuria and thrombocytopenia - Hellp syndrome class II (platelet 57.000). After pressure control, anticonvulsant therapy with magnesium sulfate, she was submitted to an emergency C/S with general anestesia, giving birth to a newborn female, weighing 2130 grams, Apgar 8/8. Evolved rapidly with coagulation disorder, severe epigastric pain radiating to the lower back, in association with sudden onset of pain in the left upper limb, having been referred to intensive care unit where she stayed until February 26. During research, we identified multiple vascular occlusion sites (hepatic microcirculation thrombosis, renal cortical and left brachial vein). The paciente underwent to immunosuppressive therapy with methylprednisolone, immunoglobulin and anticoagulation, with progressive improvement, and was home discharged on March 4, 2014 in good health. Catastrophic Antiphospholipid Syndrome (CAPS), also known as Asherson Syndrome, it’s a potentially fatal variant, that affects less than 1% of patients with antiphospholipid syndrome (APS). It was first described in 1992 by Asherson, is characterized by multiple vascular occlusive events, mainly affecting small vessels. Affects concomitantly different tissues, with progressive and rapid deterioration, leading to multiple organ failure and high mortality. Usually presents thrombocytopenia, hemolytic anemia associated with antiphospholipid antibodies in high titers. It may be the first manifestation of APS in a large percentage of cases. Among the triggers are infections, surgery, trauma, anticoagulation interruption and pregnancy. Clinical support is essential and includes removal of provocative factor, corticosteroid therapy, anticoagulation, intravenous immunoglobulin and plasmapheresis. Conclusion Although less than 1% of patients with APS develop this condition and despite all of the recommended therapies, this potentially fatal outcome (near 50%) shows their importance in modern medicine. Finally, we would like to thank the support of our Intensive Care Unit and Reumatology teams at UNIFESP.