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Objectives: To explore the prevalence and clinical significance of low complement levels in patients with catastrophic antiphospholipid syndrome (CAPS). Methods: We reviewed data from the "CAPS Registry" on C3 and/or C4 complement plasma protein levels during acute CAPS episodes. Patients were classified into those with low and normal complement levels. Data on clinical presentation, with special focus on thrombotic microangiopathy (TMA) features, diagnosis of systemic lupus erythematosus (SLE), and antiphospholipid antibody (aPL) profile were reviewed. The chi-square exact test was performed to evaluate differences between categorical data. Results: The "CAPS Registry" includes 566 patients with a total of 578 episodes of CAPS. Data on complement plasma protein levels was available in 73 episodes from the same number of patients. Low levels of C3 and/or C4 complement plasma proteins were detected in 42 (58%) CAPS episodes. Low complement levels were more common in SLE patients (55% SLE vs. 19% No SLE; p<0.001). The frequencies of clinical TMA (72% vs. 80%; p=0.4) or TMA syndrome (86% vs. 84%, p=0.9), frequency of triple aPL triple positivity (67% vs 33%; p=0.3), or the mortality (35% vs. 31%; p=0.7) were similar between low and normal complement groups. Conclusion: In our study, low levels of C3 and C4 plasma proteins are detected in 58% episodes of CAPS, which were not associated with clinical presentation including TMA features, aPL triple positivity, or mortality. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract: The 'Task Force on Catastrophic Antiphospholipid Syndrome (CAPS)' was developed on the occasion of the 14th International Congress on Antiphospholipid Antibodies. The objectives of this Task Force were to assess the current knowledge on pathogenesis, clinical and laboratory features, diagnosis and classification, precipitating factors and treatment of this condition in order to address recommendations for future research. This article summarizes the studies analyzed by the Task Force, its recommendations and the future research agenda. [Copyright &y& Elsevier]

The classification criteria for antiphospholipid syndrome (APS) generate discussion, with a growing impression that certain patients not fulfilling these criteria might be inadequately excluded from the classification. Nonetheless, these "non-criteria" patients are heterogeneously defined across different publications. We reviewed the "non-criteria" APS subgroups depicted in the literature and attempted to organize these subsets in a nomenclature proposal that could be used for research purposes. We established four potential patient profiles, grouped under the broad term "non-criteria APS": (A) "Seronegative APS": patients fulfilling clinical criteria, plus "non-criteria" manifestations, with persistently negative antiphospholipid antibodies (aPL); (B) "Clinical non-criteria APS": patients with "non-criteria" manifestations, plus aPL positivity fulfilling the classification criteria; (C) "Incomplete laboratory APS": patients fulfilling clinical criteria, plus positive aPL, but not fulfilling the classification criteria (low titer aPL); and (D) "Laboratory non-criteria APS": patients fulfilling clinical criteria, with negative or low titer criteria aPL, plus positive "non-criteria" aPL. This categorization could allow for a more homogeneous research approach to APS, enabling more sustained and universal conclusions. • "Non-criteria" antiphospholipid syndrome (APS) is heterogeneously described across the literature. • We describe four subsets: "Seronegative APS", "Clinical non-criteria APS", "Incomplete laboratory APS", and "Laboratory non-criteria APS". • This nomenclature aims to allow for a more uniform research approach to APS. [ABSTRACT FROM AUTHOR]