14th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome

Cutaneous necrotizing vasculitis as a first manifestation of antiphospholipid syndrome with catastrophic development

“…8 Brain involvement in CAPS occurs in 55.9% of cases and the most frequent cerebral manifestations are encephalopathy (40.2%), stroke (35.2%), seizures (14.6%), headache (8.5%) and coma (6.1%), according to the CAPS registry. 5 The peculiarity of this case is the symmetry of the cerebral ischemic lesions and the development in outbreaks. Regarding the cutaneous manifestation of APS, the most frequent is livedo reticularis that occurs in a quarter of all patients; the presence of leg ulcers 6 is more unusual (<10% of cases).…”

“…3,4 It occurs in less than 1% of APS cases, but early diagnosis is very important because of high mortality and morbility. 5 Other thrombotic microangiopathic conditions need to be considered in the differential diagnosis: thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, hemolysis, elevated liver enzymes, low platelets syndrome. 6 …”

Síndrome antifosfolípidos catastrófico. Reporte de caso y revisión bibliográfica

“…-Los criterios 1, 3 y 4, pero con el desarrollo de un tercer evento entre 8 y 30 días del primer evento, pese a la anticoagulación [4][5][6][7][8] . Actualmente, en el último reporte del International CAPS Registry (2016) se aprecia que la mortalidad ha disminuido desde un 50% (en los primeros reportes) hasta un 33%, probablemente por la mayor identificación del cuadro en sus etapas iniciales y el tratamiento oportuno, aunque se reporta que los casos de SAF catastrófico asociados a lupus eritematoso sistémico tienen la mayor tasa de mortalidad, con un 48%.…”

Catastrophic antiphospholipid syndrome: A case with unusual findings revealed in autopsy and a brief literature update

“…Catastrophic antiphospholipid syndrome may develop in individuals with previously diagnosed APS, or it may present as the first manifestation of APS. (1) The prevalence of CAPS is currently estimated to be 1%, with a mortality rate of 37%. In most cases, a triggering event can be identified including infection, withdrawal of (or subtherapeutic levels of ) anticoagulation, and coexistence of systemic lupus erythematosus (SLE) flare (1,2).…”

“…(1) The prevalence of CAPS is currently estimated to be 1%, with a mortality rate of 37%. In most cases, a triggering event can be identified including infection, withdrawal of (or subtherapeutic levels of ) anticoagulation, and coexistence of systemic lupus erythematosus (SLE) flare (1,2). When CAPS is suspected, prompt initiation of treatment in the intensive care setting is crucial for an optimal outcome.…”