Catastrophic Antiphospholipid Syndrome: a challenge to continue living

Authors

  • Dennis Flores Instituto Salvadoreño del Seguro Social
  • Roberto Acosta Instituto Salvadoreño del Seguro Social
  • Rubén Montúfar Instituto Salvadoreño del Seguro Social

Keywords:

Catastrophic Antiphospholipid Syndrome (CAPS), Plasmapheresis, Rituximab, El Salvador

Abstract

Antiphospholipid Syndrome (APS) is a prothrombotic state characterized by arterial and venous thrombosis in any organ or vascular tissue of the body, the most frequent clinical manifestations being venous thrombosis of the
lower limbs, cerebral arteries and obstetric complications related to alterations in the placental circulation. Catastrophic Antiphospholipid Syndrome (CAPS) is a variant of APS, characterized by thrombosis in multiple vascular niches, which leads to multisystem failure and is associated with high mortality. The case of a patient with Generalized Lupus Erythematosus of short evolution time, with joint, mucocutaneous, hematological, renal manifestations and positive antiphospholipid antibodies is presented below; who, being in a stable state of their disease, suddenly begins an episode of thrombosis in different parts of the body and in less than a week of evolution; phenomenon that began with occlusive episodes in the veins of the lower limbs, then in the cerebral arterial system and finally in the pulmonary arteries; despite effective anticoagulation, which led to a sudden and progressive deterioration of the patient's health, leading to a critical condition, for which she was initially treated with boluses of methylprednisolone, low molecular weight heparin, aspirin and statins, but Due to the deterioration of the patient and the episode of multiple thrombosis, plasmapheresis sessions were indicated and finally rituximab, with a satisfactory outcome. Currently, the patient is stable, and following her treatment with low doses of steroids, immunosuppressants, and anticoagulation.

Author Biographies

Dennis Flores, Instituto Salvadoreño del Seguro Social

specialty clinic internist

Roberto Acosta, Instituto Salvadoreño del Seguro Social

specialty clinic internist

Rubén Montúfar, Instituto Salvadoreño del Seguro Social

specialty clinic internist

Published

10-09-2022

Issue

Section

Case Reports

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