Development of a New International Antiphospholipid Syndrome Classification Criteria Phase I/II Report: Generation and Reduction of Candidate Criteria

Abstract

Objective An international multidisciplinary initiative, jointly supported by the American College of Rheumatology and European Alliance of Associations for Rheumatology, is underway to develop new rigorous classification criteria to identify patients with high likelihood of antiphospholipid syndrome (APS) for research purposes. The present study was undertaken to apply an evidence‐ and consensus‐based approach to identify candidate criteria and develop a hierarchical organization of criteria within domains. Methods During phase I, the APS classification criteria steering committee used systematic literature reviews and surveys of international APS physician scientists to generate a comprehensive list of items related to APS. In phase II, we reviewed the literature, administered surveys, formed domain subcommittees, and used Delphi exercises and nominal group technique to reduce potential APS candidate criteria. Candidate criteria were hierarchically organized into clinical and laboratory domains. Results Phase I generated 152 candidate criteria, expanded to 261 items with the addition of subgroups and candidate criteria with potential negative weights. Using iterative item reduction techniques in phase II, we initially reduced these items to 64 potential candidate criteria organized into 10 clinical and laboratory domains. Subsequent item reduction methods resulted in 27 candidate criteria, hierarchically organized into 6 additive domains (laboratory, macrovascular, microvascular, obstetric, cardiac, and hematologic) for APS classification. Conclusion Using data‐ and consensus‐driven methodology, we identified 27 APS candidate criteria in 6 clinical or laboratory domains. In the next phase, the proposed candidate criteria will be used for real‐world case collection and further refined, organized, and weighted to determine an aggregate score and threshold for APS classification.

16th International congress on antiphospholipid antibodies task force report on clinical manifestations of antiphospholipid syndrome

“…research purposes, APS is defined as thrombosis and/or pregnancy morbidity in the concomitant to persistent aPL. 1 While the Task Force acknowledged the ongoing international effort to develop new APS classification criteria, 9 the consensus was that, for diagnostic purposes, a more comprehensive definition of "APS" or aPL-related clinical symptoms would be helpful for clinicians.…”

Anticardiolipin and other antiphospholipid antibodies in critically ill COVID-19 positive and negative patients

“…1 APLAs that are components of APS criteria include IgG and/or IgM anticardiolipin (aCL), anti-β2-glycoprotein1 (anti-β2GP1) and the 'lupus anticoagulant' (LAC). 2 Other non-criteria APLA such as antiphosphatidylserine/prothrombin (PS/PT) complex, anti-PT and antidomain 1 of β2-GP1 have also found a diagnostic niche in APS. 3 4 One of the salient features of COVID-19 is the development of thrombotic events associated with severe morbidity and mortality.…”

Expert Perspective: Management of Microvascular and Catastrophic Antiphospholipid Syndrome

“…In conclusion, the management of MAPS, CAPS, and even nonthrombotic APS can be scary and challenging, given that there are no strong evidence–based treatments for these patients, who can deteriorate quickly. In parallel to our increased understanding of aPL‐mediated mechanisms, and an ongoing international effort to develop new APS classification criteria (59), it is critical that future clinical studies address these immunosuppressive pathways and potentially other novel pathways (58) in well‐designed clinical trials to accumulate more evidence for their efficacy.…”