A case of catastrophic antiphospholipid syndrome presented with diffuse myelopathy

Abstract

Background: Antiphospholipid syndrome (APS) could present numerous clinical features associated with antiphospholipid antibodies. Among them, the symptom complex of predominant microvasculopathy and multiple organ failure is known as‘catastrophic’ antiphospholipid syndrome (CAPS) or Asherson’s syndrome. Many precipitating factors of CAPS include trauma (either major or minor), malignancies, warfarin withdrawal,“flares” of systemic lupus erythematosus etc. The antiphospholipid antibodies (aPL) mainly effect on the coagulation pathway, however, there are increasing evidences of multifactorial actions of aPL. Case Report: A 27-year-old woman presented with dysuria and paraparesis for one day after upper respiratory infection. Neurological examination was compatible to myelopathy at thoracic level. Brain and spinal MRI showed lesions on hypothalamus and diffuse spinal cord. Despite of steroid therapy, probable ischemic cardiomyopathy, livedo reticularis, thrombocytopenia and comatous mental status were developed. She improved partially after plasma exchanges. Conclusion: We present a CAPS patient with thrombotic and nonthrombotic manifestations triggered by upper respiratory infection.