16th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome

Abstract

The Task Force on Catastrophic Antiphospholipid Syndrome (CAPS) met again on occasion of the 16th International Congress on Antiphospholipid Antibodies (aPL) that was held in Manchester, England, in September 2019. Its aims were to assess the up-to-date knowledge on pathogenesis, clinical and laboratory features, diagnosis and classification, precipitating factors, and treatment of CAPS. This article summarizes the main aspects that were presented during the Task Force meeting at that Congress.

Cost-effectiveness analysis of treatments for the first episode of catastrophic antiphospholipid syndrome: A study based on the catastrophic antiphospholipid syndrome registry

“…5 Among other alternatives, not to be taken as a first-line treatment, we found cyclophosphamide (for SLEassociated CAPS) and biological therapies such as rituximab or eculizumab as rescue therapies in refractory cases, and when prominent microangiopathic/microthrombotic manifestations are primary concerns. 5,6 Cost-effectiveness studies are proposed as an analytical method to quantify the relative benefits and costs between two or more alternative interventions within a common conceptual framework. They seek to line the proposition of results from the health sector perspective, incorporating costs, benefits, and harms both for payers and patients; thus, accounting for a social perspective, 7 being a determining factor for their applicability in decision-making and public policy formulation contexts.…”

“…According to the most recent review of the CAPS Registry, published in 2020 in the frame of the 16th International Congress on Antiphospholipid Antibodies, the main affected organs were kidneys (74%), lungs (55%), brain (56%), heart (53%), and skin (45%). 5 Early diagnosis in conjunction with aggressive treatment is set as the mainstay of its management as a response to high associated mortality. Although most of the evidence is of low quality and is conditioned by the rarity of the disease, recently it has been suggested as part of the first line of treatment, to use the combined therapy of glucocorticoids (GCS), therapeutic-dose anticoagulation (AC), and plasma exchange (PE) and/or intravenous IgG immunoglobulin (IVIG).…”

Expert Perspective: Management of Microvascular and Catastrophic Antiphospholipid Syndrome

“…Thus, most of our knowledge is based on case reports and series and analysis of an international web‐based CAPS registry (40). Anticoagulation therapy, glucocorticoids, IVIG, and plasma exchange are the most commonly used treatment strategies (41); B cell and complement inhibition can be considered in select cases (further discussed above and below) (42).…”

Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis

“…Criteria for diagnosis of CAPS includes: 1) clinical evidence of multiple organ involvement developing over a short period of time; 2) histopathological evidence of small vessel occlusions; 3) laboratory confirmation of aPL (lupus anticoagulant (LA) test; anticardiolipin antibody (aCL) enzyme linked immunosorbent assay (ELISA), and/or anti-β2-glycoprotein-I antibody (aβ2GPI) ELISA). The 14 th International Congress on aPL Task Force maintains previous recommendations for classifying a probable or definite CAPS (8). Considering these recommendations, this patient would have initially been catalogued as a probable CAPS.…”