Guidelines on the investigation and management of antiphospholipid syndrome

Arachchillage, Deepa J.; Platton, Sean; Hickey, Kieron; Chu, Justin; Pickering, Matthew; Sommerville, Peter; MacCallum, Peter; Breen, Karen; ,

doi:10.1111/bjh.19635

Br J Haematol

2024

DOI: 10.1111/bjh.19635

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Guidelines on the investigation and management of antiphospholipid syndrome

Deepa J. Arachchillage,

Sean Platton,

Kieron Hickey

et al.

Abstract: This guideline was compiled according to the British Society for Haematology (BSH) process at (https:// b-s-h. org. uk/ media/ 16732/ bsh-guida nce-devel opmen t-proce ss-dec-5-18. pdf). The Grading of Recommendations Assessment, Development and Evaluation (GRADE) nomenclature was used to evaluate the levels of evidence and to assess the strength of recommendations. The GRADE criteria can be found at http:// www. grade worki nggro up. org. A literature search was carried out using the terms given in the append… Show more

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Cited by 5 publications

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Hematological causes of acute ischemic stroke in younger individuals

O’Toole,

Swan,

Connors

et al. 2024

Journal of Thrombosis and Haemostasis

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Hematological causes of acute ischemic stroke in younger individuals

O’Toole,

Swan,

Connors

et al. 2024

Journal of Thrombosis and Haemostasis

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Direct oral anticoagulants in thrombotic antiphospholipid syndrome: Addressing misinterpretations in subgroup analysis

Khairani,

Bejjani,

Bikdeli

2024

Br J Haematol

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Authors reply to direct oral anticoagulants in thrombotic anti‐phospholipid syndrome: Addressing misinterpretations in subgroup analysis

Arachchillage,

Platton,

Hickey

et al. 2024

Br J Haematol

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We thank Khairani and colleagues for their letter 1 regarding our recently published British Society for Haematology (BSH) Guidelines on the investigation and management of anti-phospholipid syndrome. 2 Khairani et al., have stated that we have misinterpreted their findings from the meta-analysis 3 of the four randomised controlled studies (RCTs) comparing vitamin K antagonist (VKA)/warfarin versus direct-acting oral anticoagulants (DOACs) in patients with thrombotic APS 1 where we noted that 'pre-specified subgroup analysis showed only a trend towards a higher risk of recurrent arterial thrombosis in single or double anti-phospholipid antibody (aPL)-positive APS patients with rivaroxaban or apixaban compared with warfarin'. 2 Furthermore, Khairani et al. state: 'it would be restating outdated recommendations and misinterpretation of findings of our study to suggest that DOACs may be safe in patients with thrombotic APS with single or double-positive anti-phospholipid antibodies (aPL)'. 1 Notably, these authors support their statement: 'The current best evidence suggests that the traditional doses of DOACs are inferior to warfarin for protection against future thrombosis' by a review co-authored by themselves, rather than any additional data. 4 It should be emphasised, as stated by Khairani et al., 1 that subgroup analyses must be interpreted with caution because they are not based on randomised comparisons, but rather they are observational in nature and are also subject to type II error. Nevertheless, the DOAC trials, although heterogeneous, did not find an increased risk of venous thromboembolism (VTE) compared with VKA/warfarin. [5][6][7][8] In keeping with our statement on the risk of recurrent arterial thrombosis with rivaroxaban or apixaban compared with VKA/warfarin, and review of the individual RCTs of DOAC versus warfarin/VKA in APS, cited in the guideline, 5-8 our recommendation for APS patients with arterial thrombosis is: 'We recommend against the initiation of DOACs for treatment or secondary prophylaxis in patients with APS with stroke (1B)'. In the absence of clear evidence on the risk of recurrent thrombosis in single or double aPL-positive APS patients with VTE, our recommendations are:

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Guidelines on the investigation and management of antiphospholipid syndrome

Cited by 5 publications

References 198 publications

Hematological causes of acute ischemic stroke in younger individuals

Hematological causes of acute ischemic stroke in younger individuals

Direct oral anticoagulants in thrombotic antiphospholipid syndrome: Addressing misinterpretations in subgroup analysis

Authors reply to direct oral anticoagulants in thrombotic anti‐phospholipid syndrome: Addressing misinterpretations in subgroup analysis

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