Treatment of catastrophic antiphospholipid syndrome

Abstract

Purpose of review Catastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of APS. While affecting only 1% of patients with APS, the condition is frequently fatal if not recognized and treated early. Here, we will review the current approach to diagnosis and treatment of CAPS. Recent findings Data from the international “CAPS registry,” spearheaded by the European Forum on Antiphospholipid Antibodies, have improved our understanding of at-risk patients, typical clinical features, and associated/precipitating diagnoses. Current guidelines also continue to support a role for anticoagulants and glucocorticoids as foundation therapy in all patients. Finally, new basic science and case series suggest that novel therapies, such as rituximab and eculizumab warrant further study. Summary Attention to associated diagnoses such as infection and systemic lupus erythematosus (SLE) are critical at the time of diagnosis. All patients should be treated with anticoagulation, corticosteroids, and possibly plasma exchange. In patients with SLE, cyclophosphamide should also be considered. In refractory or relapsing cases, new therapies such as rituximab and possibly eculizumab may be options, but need further study.

Call for Vigilance – Red Flags in Systemic Lupus Erythematous

“…Additionally, the patient in case two also required plasma exchange. 4 Anticoagulants are required in CAPS whereas extracorporeal membrane oxygenation (ECMO) can be used in severe cases of DAH. 6 A high index of suspicion, early recognition of symptoms and treatment with immunosuppressive agents remain the keys to improve quality of care, prevent progression of organ failure and hence prevent mortality in CAPS and DAH.…”

Síndrome antifosfolípidos catastrófico. Reporte de caso y revisión bibliográfica

“…De los tratamientos individuales, los más empleados son anticoagulantes (82%), esteroides (76%), plasmaféresis (35%) e inmunoglobulina intravenosa (27%). Entre los tratamientos combinados (mayor efectividad, en teoría) destacan los anticoagulantes con esteroides (19%) y los anticoagulantes + esteroides + plasmaféresis/inmunoglobulina intravenosa (18%) [9][10][11] . Recientemente se han empleado anticuerpos monoclonales para el tratamiento del SAF catastrófico, pero la evidencia aún es escasa y solo hay reportes de casos anecdóticos.…”

Cutaneous gangrene of the arms and legs after cardiopulmonary resuscitation: A rare presentation of catastrophic antiphospholipid syndrome

“…With a mortality rate of approximately 50%, early diagnosis and aggressive therapy are essential to the management of CAPS. Current guidelines support anticoagulants and systemic glucocorticoids as the foundation of therapy for all patients, and plasma exchange and intravenous immunoglobulin (IVIG) may be options according to the patient's clinical context [3]. Here, we will describe a rare case of CAPS treated at our institution with good results.…”

“…According to the recent literature [3,9], a treatment that significantly reduces mortality in patients with CAPS is combination therapy including anticoagulation (AC), glucocorticoids (CS), and therapeutic plasma exchange (PE) with or without IVIG. AC + CS is the most commonly used regimen (19.8%), followed by AC + CS + PE and/or IVIG (17.4%).…”