Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Multicenter Study
. 2022 Jan 25;24(1):33.
doi: 10.1186/s13075-022-02726-9.

Non-criteria manifestations in primary antiphospholipid syndrome: a French multicenter retrospective cohort study

Alexis F Guédon #  1 , Jennifer Catano #  1 , Laure Ricard  1 , Charlotte Laurent  1 , Claire de Moreuil  2 , Geoffrey Urbanski  3 , Sophie Deriaz  4 , Grigorios Gerotziafas  5 , Ismail Elalamy  5 , Alexandra Audemard  4 , Francois Chasset  6 , Sonia Alamowitch  7 , Jérémie Sellam  8 , François Maillot  4 , Jean Jacques Boffa  9 , Ariel Cohen  10 , Noémie Abisror  1 , Olivier Fain  1 , Arsène Mekinian  11
Affiliations
Multicenter Study

Non-criteria manifestations in primary antiphospholipid syndrome: a French multicenter retrospective cohort study

Alexis F Guédon et al. Arthritis Res Ther. .

Abstract

Background: From this retrospective study, we aimed to (1) describe the prevalence and characteristics of non-criteria features in primary antiphospholipid syndrome (p-APS) and (2) determine their prognostic value.

Methods: This retrospective French multicenter cohort study included all patients diagnosed with p-APS (Sydney criteria) between January 2012 and January 2019. We used Kaplan-Meier and adjusted Cox proportional hazards models to compare the incidence of relapse in p-APS with and without non-criteria manifestations.

Results: One hundred and seventy-nine patients with p-APS were included during the study time, with a median age of 52.50 years [39.0; 65.25] and mainly women (n = 112; 62.6%). Among them, forty-three patients (24.0%) presented at least one non-criteria manifestation during the follow-up: autoimmune cytopenias (n = 17; 39.5%), Libman Sachs endocarditis (n = 5; 11.6%), APS nephropathy (n = 4; 9.3%), livedo reticularis (n = 8; 18.6%), and neurological manifestations (n = 12; 27.9%). In comparison to p-APS without any non-criteria manifestations (n = 136), p-APS with non-criteria features had more arterial thrombosis (n = 24; 55.8% vs n = 48; 35.3%; p = 0.027) and more frequent pre-eclampsia (n = 6; 14.3% vs n = 4; 3.1%; p = 0.02). The prevalence of triple positivity was significantly increased in patients with non-criteria features (n = 20; 47.6% vs n = 25; 19.8%; p = 0.001). Patients with p-APS and non-criteria manifestations (n = 43) received significantly more additional therapies combined with vitamin K antagonists and/or antiaggregants. Catastrophic APS (CAPS) tended to be more frequent in p-APS with non-criteria features (n = 2; 5.1% vs none; p = 0.074). The p-APS with non-criteria manifestations had significantly increased rates of relapse (n = 20; 58.8% vs 33; 33.7%; p = 0.018) in bivariate analysis, but in survival analyses, the hazard ratio (HR) of relapse was not significantly different between the two groups (HR at 1.34 [0.67; 2.68]; p = 0.40).

Conclusions: The presence of non-criteria features is important to consider, as they are associated with particular clinical and laboratory profiles, increased risk of relapse, and need for additional therapies. Prospective studies are necessary to better stratify the prognosis and the management of p-APS.

Keywords: Antiphospholipid antibodies; Antiphospholipid syndrome; Non-criteria antiphospholipid syndrome.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Time to relapse according to non-criteria APS status
See this image and copyright information in PMC

References

    1. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS) J Thromb Haemost. 2006;4(2):295–306. doi: 10.1111/j.1538-7836.2006.01753.x. - DOI - PubMed
    1. Abreu MM, Danowski A, Wahl DG, Amigo M-C, Tektonidou M, Pacheco MS, et al. The relevance of “non-criteria” clinical manifestations of antiphospholipid syndrome: 14th International Congress on Antiphospholipid Antibodies Technical Task Force Report on Antiphospholipid Syndrome Clinical Features. Autoimmun Rev. 2015;14(5):401–414. doi: 10.1016/j.autrev.2015.01.002. - DOI - PubMed
    1. Sciascia S, Amigo M-C, Roccatello D, Khamashta M. Diagnosing antiphospholipid syndrome: “extra-criteria” manifestations and technical advances. Nat Rev Rheumatol. 2017;13(9):548–560. doi: 10.1038/nrrheum.2017.124. - DOI - PubMed
    1. Radin M, Ugolini-Lopes MR, Sciascia S, Andrade D. Extra-criteria manifestations of antiphospholipid syndrome: risk assessment and management. Semin Arthritis Rheum. 2018;48(1):117–120. doi: 10.1016/j.semarthrit.2017.12.006. - DOI - PubMed
    1. Pengo V, Ruffatti A, Legnani C, Gresele P, Barcellona D, Erba N, et al. Clinical course of high-risk patients diagnosed with antiphospholipid syndrome. J Thromb Haemost JTH. 2010;8(2):237–242. doi: 10.1111/j.1538-7836.2009.03674.x. - DOI - PubMed

Publication types

Substances