Revised classification criteria for antiphospholipid syndrome

Antiphospholipid syndrome is present if at least 1 of the clinical criteria and 1 of the laboratory criteria that follow are met*

Clinical criteria

Vascular thrombosis^¶
1 or more clinical episodes^Δ of arterial, venous, or small vessel thrombosis^◊, in any tissue or organ. Thrombosis must be confirmed by objective validated criteria (ie, unequivocal findings of appropriate imaging studies or histopathology). For histopathologic confirmation, thrombosis should be present without significant evidence of inflammation in the vessel wall.

Pregnancy morbidity
1. 1 or more unexplained deaths of a morphologically normal fetus at or beyond the 10^th week of gestation, with normal fetal morphology documented by ultrasound or by direct examination of the fetus; or
2. 1 or more premature births of a morphologically normal neonate before the 34^th week of gestation because of: (i) eclampsia or severe preeclampsia defined according to standard definitions, or (ii) recognized features of placental insufficiency^§; or
3. 3 or more unexplained consecutive spontaneous abortions before the 10^th week of gestation, without maternal or hormonal abnormalities, and paternal and maternal chromosomal causes excluded.
In studies of populations of patients who have more than 1 type of pregnancy morbidity, investigators are strongly encouraged to stratify groups of subjects according to a, b, or c above.

Laboratory criteria^¥

LA present in plasma, on 2 or more occasions at least 12 weeks apart, detected according to the guidelines of the International Society on Thrombosis and Haemostasis (Scientific Subcommittee on LAs/phospholipid-dependent antibodies).

aCL of IgG and/or IgM isotype in serum or plasma, present in medium or high titer (ie, >40 GPL or MPL, or >the 99th percentile), on 2 or more occasions, at least 12 weeks apart, measured by a standardized ELISA.

Anti-beta2 glycoprotein I antibody of IgG and/or IgM isotype in serum or plasma (in titer >the 99th percentile), present on 2 or more occasions, at least 12 weeks apart, measured by a standardized ELISA, according to recommended procedures.

LA: lupus anticoagulant; aCL: anticardiolipin antibody; Ig: immunoglobulin; ELISA: enzyme-linked immunosorbent assay; APS: antiphospholipid syndrome; aPL: antiphospholipid antibodies; LDL: low-density lipoprotein; HDL: high-density lipoprotein; GFR: glomerular filtration rate.

* Classification of APS should be avoided if less than 12 weeks or more than 5 years separate the positive aPL test and the clinical manifestation.

¶ Coexisting inherited or acquired factors for thrombosis are not reasons for excluding patients from APS trials. However, 2 subgroups of APS patients should be recognized, according to: (a) the presence; and (b) the absence of additional risk factors for thrombosis. Indicative (but not exhaustive) cases include: age (>55 in men and >65 in women) and the presence of any of the established risk factors for cardiovascular disease (hypertension, diabetes mellitus, elevated LDL or low HDL cholesterol, cigarette smoking, family history of premature cardiovascular disease, body mass index ≥30 kg m^–2, microalbuminuria, estimated GFR <60 mL minute^–1), inherited thrombophilias, oral contraceptives, nephrotic syndrome, malignancy, immobilization, and surgery. Thus, patients who fulfill criteria should be stratified according to contributing causes of thrombosis.

Δ A thrombotic episode in the past could be considered as a clinical criterion, provided that thrombosis is proved by appropriate diagnostic means and that no alternative diagnosis or cause of thrombosis is found.

◊ Superficial venous thrombosis is not included in the clinical criteria.

§ Generally accepted features of placental insufficiency include: (i) abnormal or non-reassuring fetal surveillance test(s), eg, a non-reactive non-stress test, suggestive of fetal hypoxemia; (ii) abnormal Doppler flow velocimetry waveform analysis suggestive of fetal hypoxemia, eg, absent end-diastolic flow in the umbilical artery; (iii) oligohydramnios, eg, an amniotic fluid index of 5 cm or less; or (iv) a postnatal birth weight less than the 10th percentile for the gestational age.

¥ Investigators are strongly advised to classify APS patients in studies into one of the following categories: I, more than 1 laboratory criteria present (any combination); IIa, LA present alone; IIb, aCL antibody present alone; IIc, anti-beta2 glycoprotein I antibody present alone.

From: 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; 4:295. http://onlinelibrary.wiley.com/doi/10.1111/j.1538-7836.2006.01753.x/abstract. Copyright © 2006 International Society on Thrombosis and Haemostasis. Reproduced with permission of John Wiley & Sons, Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared, or emailed. Please contact Wiley's Permissions Department either via email: [email protected] or use the RightsLink service by clicking on the Request Permission link accompanying this article on Wiley Online Library (www.onlinelibrary.wiley.com).

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Revised classification criteria for antiphospholipid syndrome