Damage Index for Antiphospholipid Syndrome (DIAPS): An Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) "Damage" working group report on strengths and limitations

Gustavo G M Balbi; Pedro Gaspar; Hannah Cohen; David A Isenberg; Doruk Erkan; Danieli Andrade; APS ACTION Extended “Damage” Working Group

doi:10.1016/j.semarthrit.2024.152605

Damage Index for Antiphospholipid Syndrome (DIAPS): An Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) "Damage" working group report on strengths and limitations

Semin Arthritis Rheum. 2024 Dec 4:70:152605. doi: 10.1016/j.semarthrit.2024.152605. Online ahead of print.

Authors

Gustavo G M Balbi¹, Pedro Gaspar², Hannah Cohen³, David A Isenberg⁴, Doruk Erkan⁵, Danieli Andrade⁶; APS ACTION Extended “Damage” Working Group

Affiliations

¹ Discipline of Rheumatology, Universidade Federal de Juiz de Fora (UFJF), Juiz de Fora, Minas Gerais, Brazil.
² Internal Medicine Department, Hospital Santa Maria, Unidade Local de Saúde Santa Maria, Lisbon, Portugal; Gulbenkian Institute for Molecular Medicine, Lisbon, Portugal; Centro Académico de Medicina de Lisboa, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal.
³ Haemostasis Research Unit, Department of Haematology, University College London, London, UK; Department of Haematology, University College London Hospitals NHS Foundation Trust, London, UK.
⁴ Centre for Rheumatology, Division of Medicine, University College London, London, UK; Department of Rheumatology, University College London Hospitals NHS Foundation Trust, London, UK.
⁵ Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY, USA.
⁶ Discipline of Rheumatology, University of São Paulo (USP), Av Dr. Arnaldo 455, Third Floor, LIM 17, São Paulo, São Paulo 01246-903, Brazil. Electronic address: [email protected].

PMID: 39662300
DOI: 10.1016/j.semarthrit.2024.152605

Abstract

Objectives: To gather the perspectives of APS ACTION members regarding the strengths and limitations of Damage Index for Antiphospholipid Syndrome (DIAPS); and establish recommendations for the improvement of DIAPS.

Methods: APS ACTION members were invited to answer a survey regarding their satisfaction with DIAPS scoring system and individual items. The level of agreement (LoA) among members with the inclusion of individual items in DIAPS was calculated (LoA of <75% was considered disagreement). Respondents' open-ended comments about DIAPS limitations were also collected, which helped formulate our recommendations for DIAPS improvement.

Results: Forty-two APS ACTION members (58.3%) answered the survey. Of them, 26 (61.9%) were satisfied, 4 (9.5%) were neutral, and 12 (28.6%) were dissatisfied with the current DIAPS scoring system. Fifteen items (39.5%) presented a LoA <75% regarding the inclusion in DIAPS. Respondents provided comments that were grouped under six main categories related to concerns about: a) definitions and attribution of damage (including causality and temporal relationship); b) scoring system; c) overlapping items; d) specific items (exclusion of redundant items and inclusion of additional ones); e) the need to incorporate multiple events; and f) feasibility and practicality. Finally, the APS ACTION "Damage" Working Group developed 7 recommendations that should be considered for the next generation DIAPS.

Conclusion: Approximately 60% of respondents were satisfied with DIAPS and its definitions; however, our survey demonstrated that there is substantial room to improve the current damage index for APS. Efforts for updating DIAPS should consider the APS ACTION "Damage" Working Group recommendations.

Keywords: Antiphospholipid antibodies; Antiphospholipid syndrome; DIAPS; Damage index; Health status indicators; Severity of illness index; Thrombosis.