Learning Objectives

  • Review the major updates in the 2023 American College of Rheumatology and European Alliance of Associations for Rheumatology classification criteria for APS

  • Understand the distinction between classification criteria used for research purposes versus clinical care

A 43-year-old woman is evaluated for thrombocytopenia, with a nadir platelet count of 50 × 109/L. Three months prior, she was found to have mitral valve thickening on an echocardiogram performed for shortness of breath. A lupus anticoagulant is persistently positive at baseline and 12 weeks later. An alternative etiology for thrombocytopenia is not identified. Does this patient meet classification criteria for antiphospholipid syndrome?

Antiphospholipid syndrome (APS) is an acquired systemic autoimmune disorder characterized by macrovascular arterial or venous thrombosis, pregnancy morbidity, microvascular phenomena, and other nonthrombotic manifestations in the context of compatible laboratory testing for antiphospholipid antibodies (aPL). An APS...

1.
Wilson
WA
,
Gharavi
AE
,
Koike
T
, et al.
International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop
.
Arthritis Rheum
.
1999
;
42
(
7
):
1309
-
1311
.
2.
Miyakis
S
,
Lockshin
MD
,
Atsumi
T
, 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
.
3.
Barbhaiya
M
,
Zuily
S
,
Naden
R
, et al
;
ACR/EULAR APS Classification Criteria Collaborators
.
2023 ACR/EULAR antiphospholipid syndrome classification criteria
.
Ann Rheum Dis
.
2023
;
82
(
10
):
1258
-
1270
.
4.
Erkan
D
,
Yazici
Y
,
Peterson
MG
,
Sammaritano
L
,
Lockshin
MD
.
A cross- sectional study of clinical thrombotic risk factors and preventive treatments in antiphospholipid syndrome
.
Rheumatology (Oxford)
.
2002
;
41
(
8
):
924
-
929
.
5.
Foddai
SG
,
Radin
M
,
Cecchi
I
, et al.
2023 ACR/EULAR classification criteria in existing research cohorts: an international study
.
Rheumatology (Oxford)
.
2024
;
63
(
10
):
2770
-
2775
.
6.
Branch
DW
,
Lim
MY
.
How I diagnose and treat antiphospholipid syndrome in pregnancy
.
Blood
.
2024
;
143
(
9
):
757
-
768
.
7.
Erton
ZB
,
K Leaf
R
,
de Andrade
D
, et al.
Immunosuppression use in primary antiphospholipid antibody-positive patients: descriptive analysis of the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (“Registry”)
.
Lupus
.
2022
;
31
(
14
):
1770
-
1776
.
8.
Knight
JS
,
Erkan
D
.
Rethinking antiphospholipid syndrome to guide future management and research
.
Nat Rev Rheumatol
.
2024
;
20
(
6
):
377
-
388
.
9.
Kelchtermans
H
,
Pelkmans
L
,
de Laat
B
,
Devreese
KM
.
IgG/IgM antiphospholipid antibodies present in the classification criteria for the antiphospholipid syndrome: a critical review of their association with thrombosis
.
J Thromb Haemost
.
2016
;
14
(
8
):
1530
-
1548
.
10.
Urbanski
G
,
Yelnik
CM
,
Maillard
H
, et al.
Antiphospholipid syndrome with isolated isotype M anticardiolipin and/or anti-B2GPI antibody is associated with stroke
.
Stroke
.
2018
;
49
(
11
):
2770
-
2772
.
11.
Chayoua
W
,
Kelchtermans
H
,
Gris
J-C
, et al.
The (non-)sense of detecting anti-cardiolipin and anti-β2glycoprotein I IgM antibodies in the antiphospholipid syndrome
.
J Thromb Haemost
.
2020
;
18
(
1
):
169
-
179
.
12.
Pengo
V
,
Ruffatti
A
,
Legnani
C
, et al.
Clinical course of high-risk patients diagnosed with antiphospholipid syndrome
.
J Thromb Haemost
.
2010
;
8
(
2
):
237
-
242
.
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