ECULIZUMAB IN THE MANAGEMENT OF CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME WITH MULTIORGAN INVOLVEMENT: A CASE REPORT

PV306 / #753 Case Report Poster Topic: AS03 - Antiphospholipid Syndrome Late-Breaking Abstract Introduction Catastrophic antiphospholipid syndrome (CAPS) is a severe and rapidly progressive form of antiphospholipid syndrome that results in multiorgan failure due to thrombosis in the microvasculature. It is rare, occurring in only 1% of patients with antiphospholipid syndrome, but it carries a high mortality rate and can be the first manifestation of the disease. Eculizumab, a C5-blocking monoclonal antibody, is effective in the treatment of thrombotic microangiopathy by controlling complement system hyperactivation. Due to the rarity of CAPS, there is limited literature regarding the appropriate management of these patients, and there is little clinical experience with Eculizumab treatment. This case report aims to provide clinical insight to improve the management of patients with catastrophic antiphospholipid syndrome and multiorgan involvement. Case Presentation With Investigation We present the case of a 62-year-old female patient with a debut diagnosis of CAPS secondary to systemic lupus erythematosus. She exhibited renal involvement with significant deterioration of kidney function, which required hemodialysis. Renal biopsy revealed thrombotic microangiopathy affecting small and medium-sized vessels and glomeruli. Additionally, she exhibited cardiopulmonar involvement, including nonischemic dilated cardiomyopathy and pulmonary hypertension. Laboratory findings showed hemolytic anemia, thrombocytopenia, renal failure, complement consumption, and positivity for ANA, anticardiolipin IgM, and beta-2 glycoprotein antibodies. In the first weeks following the initial diagnosis, the patient received treatment with hydroxychloroquine (200 mg every 12 hours), methylprednisolone boluses (500 mg for 3 days), followed by prednisone at a tapering dose, immunoglobulin therapy (weight-adjusted for 5 days), mycophenolate (500 mg every 12 hours), and 3 cycles of Rituximab 1 g. Anticoagulation was managed with bemiparin. Additionally, treatment with Eculizumab was initiated with 4 weekly doses, followed by Eculizumab every 2 weeks for 3 months. It was interrupted due to the patient’s admission for bacteremia, with a good clinical outcome. Afterward, it was not reintroduced as the patient remained stable with her disease and showed very good clinical progress, as well as normalization of laboratory values, with no significant incidents during 18 months. Literature Review [1.] Almeida C. Autoimmun Rev 2015;14:1087-96. [2.] Guerra N. Acta Colombiana de Cuidado Intensivo 2019;19:154-9. Discussion Given the severity and high mortality of CAPS, early diagnosis and appropriate treatment are critical to prevent irreversible structural damage to target organs. Early treatment with anticoagulation and intravenous corticosteroids, followed by plasmapheresis, rituximab, and eculizumab in the early stages, is crucial to achieve disease remission and prevent irreversible structural damage to target organs.