Sydney Classification Criteria Research Articles

Unveiling the clinical spectrum: Exploring the role of anti-β2glycoprotein-1 antibodies (anti-β2GPI) in antiphospholipid syndrome suspects.

The objectives of this study were to determine the prevalence of anti-β2glycoprotein-1 antibodies (anti-β2GPI) in Pakistani patients clinically suspected to have antiphospholipid syndrome (APS) and assess their association with clinical manifestations. The antiphospholipid syndrome (APS) is a complex disorder characterized by recurrent thrombotic and obstetric complications. An analytical cross-sectional study was conducted at Aga Khan University Hospital from January to June 2022, after obtaining ethical approval (ERC ID: 2021-6404-19580). A total of 133 patients aged 18-60 years, clinically suspected of having APS based on the updated international consensus (Sydney) classification criteria, were recruited. Anti-β2GPI antibodies were tested using the same blood samples provided for aCL testing, with verbal consent. Demographic, clinical, and biochemical data were collected via a structured questionnaire, while information on lupus anticoagulant testing was retrospectively obtained from prior records. The study included 120 females (90.2%) and 13 males (9.8%) with a mean age of 31.3 ± 8.8 years. Predominant clinical manifestations included unexplained miscarriages at >10 weeks of gestation (n = 77/120 female, 64.2%), while deep venous thrombosis (DVT) was a common non-obstetric clinical feature (n = 18/133, 13.5%). The median level of anti-β2GPI was 2.12 U/ml (1.34-7.04) and 7.5% (n = 10) were positive. Of the 10 positive patients, 2 displayed positive anti-β2GPI while concurrently testing negative for other aPL antibodies. A significant association was identified between the presence of anti-β2GPI and the occurrence of DVT and other venous thromboembolic events (VTE). This study highlights the prevalence and diagnostic utility of anti-β2GPI in Pakistani patients suspected of APS, identifying cases missed by other aPL tests and showing significant associations with thrombotic manifestations like DVT and VTE. However, the cross-sectional design, lack of confirmatory testing, and absence of locally derived cut-offs limit causal inferences.

Impact of the 2023 ACR/EULAR Classification Criteria in Women with Primary Antiphospholipid Syndrome during Pregnancy.

Background/Objectives: ACR/EULAR has recently developed new classification criteria for antiphospholipid syndrome (APS). The present study aims to analyze the impact of these new 2023 ACR/EULAR classification criteria in a cohort of pregnant women with primary APS. Methods: Retrospective cohort study of 93 consecutive pregnant women attending the Autoimmune Diseases Pregnancy Clinic, a multidisciplinary unit of a tertiary care teaching hospital, between 2005 and 2023. All of them fulfilled the Sydney classification criteria for APS. Women diagnosed with rheumatic autoimmune diseases other than APS were excluded. Results: Twenty-four out of ninety-three patients (25.8%) met the 2023 ACR/EULAR criteria for APS. Patients who met the new classification criteria were very similar to those who did not, except for being younger (p < 0.001), and had a lower number of clinical pregnancies (p = 0.004). The obstetric domain was clearly underrepresented in women who fulfilled the 2023 ACR/EULAR criteria (p < 0.001). Patients meeting the new classification criteria were primarily characterized by preterm births before 34 weeks due to severe placentation disorders (p = 0.004). Women with early and late fetal loss were significantly underrepresented (p < 0.0001 and 0.03, respectively). Nearly half of these patients had thrombocytopenia (p < 0.001). Serologically, these patients showed a higher frequency of persistent lupus anticoagulant (p = 0.02) and a lower frequency of IgM isotype antiphospholipid antibodies (p = 0.05). Conclusions: Almost three-quarters of the patients included in the study did not meet the 2023 ACR/EULAR criteria. Most patients who could not be classified according to these new classification criteria were those with early and/or late fetal deaths, as well as patients carrying only IgM aCL/AB2GPI antibodies. The high specificity of the 2023 ACR/EULAR criteria, restricted to severe placentation disorders, may leave the majority of patients with obstetric APS out of the new classification criteria.

Recurrent thrombotic events in pediatric antiphospholipid syndrome: A systematic review and meta-analysis

Recurrent thrombotic events in pediatric antiphospholipid syndrome: A systematic review and meta-analysis

Identifying high-risk profile in primary antiphospholipid syndrome through cluster analysis: French multicentric cohort study

IntroductionAntiphospholipid syndrome (APS) is an autoimmune disease characterised by thrombosis (arterial, venous or small vessel) or obstetrical events and persistent antiphospholipid antibodies (aPL), according to the Sydney classification criteria. Many...

Determination of four homogeneous subgroups of patients with antiphospholipid syndrome: a cluster analysis based on 509 cases.

APS is a heterogeneous disease with different phenotypes. Using an unsupervised hierarchical cluster analysis, we aimed to determine distinct homogeneous phenotypes among APS patients. We performed an observational, retrospective study of APS patients enrolled in the French multicentre 'APS and SLE' registry who met the Sydney classification criteria. The clustering process involved an unsupervised multiple correspondence analysis followed by a hierarchical ascendant clustering analysis; it used 27 variables selected to cover a broad range of APS clinical and laboratory manifestations. These analyses included 509 patients, mainly women (77.8%). Mean (s.d.) age at APS diagnosis was 36.2 (14.6) years, and mean follow-up since diagnosis 10.3 (8.5) years. This hierarchical classification cluster analysis yielded four homogeneous groups of patients: cluster 1, mostly with venous thromboembolism without any associated autoimmune disease; cluster 2, older, lowest proportion of women, history of arterial events, and/or with migraines, arterial hypertension, diabetes mellitus, or dyslipidaemia; cluster 3, younger, highest proportion of women, associated SLE or other autoimmune diseases, and a history of venous thromboembolism or pregnancy morbidity; and cluster 4, mainly with a history of catastrophic antiphospholipid syndrome, aPL-associated nephropathy, and pregnancy morbidity, with frequent triple positivity and more deaths (16.7%). Our study applied an unsupervised clustering method to distinguish four homogeneous APS patient subgroups that were predominantly venous; arterial; associated with SLE or another autoimmune disease; and arterial microthrombotic. Heterogeneous pathophysiological mechanisms may explain these findings.

Thrombosis and thrombocytopenia in antiphospholipid syndrome: their association with mean platelet volume and hematological ratios

To assess the mean platelet volume (MPV), platelet-to-lymphocyte ratio (PLR), the neutrophil-to-lymphocyte ratio (NLR) and the MPV-to-lymphocyte ratio, and to test them according to the clinical/serological status, shift through time and other comorbidities in APS. We included 96 primary APS patients according to the Sydney classification criteria and/or patients with thrombocytopenia and/or autoimmune hemolytic anemia who also fulfilled the serological criteria. We tested aCL, anti-β2GP-I and aPS/PT antibodies and LA. We first registered the MPV and the aforementioned ratios within at least 6months after an event of thrombosis or thrombocytopenia/AIHA (baseline determination), and during thrombosis/thrombocytopenia/AIHA onset when available (acute event). A lower baseline MPV and a higher PLR characterized the thrombotic group (n=74). The AUC for baseline PLR was 0.82 (p<0.001): SE of 69%, SP 91%, PPV 96%, NPV 74%, LR+ 13.67 and LR- 0.19. During the acute event, both variables increased. The thrombocytopenic group (n=66) had a higher baseline MPV and a lower PLR, and during an acute event the PLR decreased more deeply. The AUC for MPV was 0.64 (p=0.02): SE 44%, SP 92%, PPV 86%, NPV 40%, LR+ 3.3 and LR- 0.85. These findings were not related with the aPL antibody profile status, titers or comorbidities. Basal MPV and PLR might help to identify APS patients according to their thrombotic or thrombocytopenic phenotype. These variables change during the acute events and might be the reflex of physiopathological or compensatory mechanisms in APS.

Non-Criteria Manifestations of Juvenile Antiphospholipid Syndrome.

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder mainly characterised by increased risks of thrombosis and pregnancy morbidity and persistent positive test results for antiphospholipid antibodies (aPLs). The criteria for diagnosing juvenile APS have yet to be validated, while the Sydney classification criteria do not contain several non-thrombotic clinical manifestations associated with the presence of aPLs. As such, difficulties have been encountered in the diagnosis of patients who have no certain thrombotic occlusions. Moreover, extra-criteria manifestations (i.e., clinical manifestations not listed in the classification criteria), including neurologic manifestations (chorea, myelitis and migraine), haematologic manifestations (thrombocytopenia and haemolytic anaemia), livedo reticularis, nephropathy and valvular heart disease have been reported, which suggests that the clinical spectrum of aPL-related manifestations extends beyond that indicated in the classification criteria. Studies have demonstrated that more than 40% of children with aPLs demonstrated non-thrombotic aPL-related clinical manifestations alone. Moreover, our results showed that the pathogenesis of non-criteria manifestations is characterised by “APS vasculopathy”. The present review introduces the characteristics and findings of non-criteria manifestations observed in juvenile APS.

Clinical and autoantibody profile in male and female patients with systemic lupus erythematosus: A retrospective study in 603 Brazilian patients.

Sex and ethnic background may influence the clinical and autoantibody profile in systemic lupus erythematosus (SLE). This retrospective study aimed to compare the clinical and autoantibody profiles of male and female patients with SLE in a sample of Brazilian patients. This was a retrospective study of 603 patients (48 males and 555 females) from a single rheumatology center. Collected clinical data included clinical findings according to the definition of 2012 Systemic Lupus International Collaborating Clinics classification criteria, the presence of antiphospholipid antibody syndrome according to the Sydney classification criteria and autoantibody profile (anti-dsDNA, anti-Ro, anti-La, anti-Sm, anti-RNP, rheumatoid factor, anticardiolipin [aCl] IgG, aCl IgM, lupus anticoagulant, and direct antiglobulin test), and histological results of kidney biopsies. It was found that females had higher age at disease onset (p=0.02), more oral ulcers (p=0.001), and presented more often with alopecia (p<0.0001) than males. Males had a higher prevalence of glomerulonephritis (OR=6.5; 95%CI=3.0-13.7) and anti-dsDNA (OR=2.59; 95%CI=1.38-4.85) than females, but no differences were found in the pattern of renal biopsies (p=0.46). In this sample of Brazilian patients, the males had more renal involvement, fewer oral ulcers, and presented fewer times with alopecia than females.

Beyond current concepts in anti-phospholipid syndrome: The 16th International Congress on Anti-phospholipid Antibodies (ICAPA) in Manchester

To assess the mean platelet volume (MPV), platelet-to-lymphocyte ratio (PLR), the neutrophil-to-lymphocyte ratio (NLR) and the MPV-to-lymphocyte ratio, and to test them according to the clinical/serological status, shift through time and other comorbidities in APS.We included 96 primary APS patients according to the Sydney classification criteria and/or patients with thrombocytopenia and/or autoimmune hemolytic anemia who also fulfilled the serological criteria. We tested aCL, anti-β2GP-I and aPS/PT antibodies and LA. We first registered the MPV and the aforementioned ratios within at least 6 months after an event of thrombosis or thrombocytopenia/AIHA (baseline determination), and during thrombosis/thrombocytopenia/AIHA onset when available (acute event).A lower baseline MPV and a higher PLR characterized the thrombotic group (n = 74). The AUC for baseline PLR was 0.82 (p < 0.001): SE of 69%, SP 91%, PPV 96%, NPV 74%, LR+ 13.67 and LR- 0.19. During the acute event, both variables increased. The thrombocytopenic group (n = 66) had a higher baseline MPV and a lower PLR, and during an acute event the PLR decreased more deeply. The AUC for MPV was 0.64 (p = 0.02): SE 44%, SP 92%, PPV 86%, NPV 40%, LR+ 3.3 and LR- 0.85. These findings were not related with the aPL antibody profile status, titers or comorbidities.Basal MPV and PLR might help to identify APS patients according to their thrombotic or thrombocytopenic phenotype. These variables change during the acute events and might be the reflex of physiopathological or compensatory mechanisms in APS.

THU0523 CLINICAL UTILITY OF TESTING CONVENTIONAL AND NON-CONVENTIONAL ANTI-PHOSPHOLIPID ANTIBODIES IN SUSPECTED OBSTETRIC ANTI-PHOSPHOLIPID SYNDROME

Background:Anti-phospholipid syndrome (APS) is an important cause for recurrent pregnancy losses (RPL). Conventional APS antibodies (aPLs) like lupus anti-coagulant (LA), anti-cardiolipin(ACL) and anti-beta 2 glycoprotein I (anti-β2 GP I) are not present in significant number of obstetric APS(OAPS) patients, leading to a state described as “ sero-negative” OAPS (SNOAPS). Recent literature shows non-conventional aPLs like Anti phosphatidylserine-prothrombin complex (Anti-PSPT) and Anti-Annexin V (Anti-Ann V) can be positive in up to 50% of SNOAPS patientsObjectives:Testing the performance of conventional and non-conventional aPLs in suspected OAPS patients (obstetric events as defined in the Sydney classification criteria for APS)Methods:We performed a retrospective chart review of 101 patients who underwent combined testing for non-conventional aPLs for suspected OAPS from May 2016 to November 2019 at our department. Patients were categorized into OAPS cases (n=50, median age 31 years) and controls (n=51, median age 30 years) based on their fulfillment of clinical definition of OAPS events defined by Sydney criteria. Conventional aPLs were tested by methods adapted in Sydney criteria and Anti PSPT /Anti Ann V were tested by commercial ELISA. The sample size(n=101) has 95% confidence interval with a margin of error of 10% for the objective of the study.Results:36 cases (72%) were ‘sero-positive’ &amp; 14 cases (28%) were truly ‘sero-negative’ for conventional aPLs. 5 (35.7%) of the SNOAPS patients were positive for Ant-PSPT and/or Anti AnnV antibodies. Performance of the various aPLs in suspected OAPS is displayed in Table 1 &amp; Figure 1.Table 1showing the performance of the various conventional and non-conventional APLs in suspected obstetric APS casesAntibodySensitivitySpecificityLikelihood Ratio(+)Likelihood Ratio (-)Positive Predictive ValueNegative Predictive ValueAccuracyYouden’s IndexLA50%94.1 %8.50.589.3%65.7%72.3%44.1%ACL32%98%16.30.794.1%59.5 %65.3%30 %anti β2 GP I IgM38.4%91.4 %4.50.783.3%57.1 %63.5%29.8%anti β2 GP I IgG24%96.1 %6.10.885.7%56.3%60.4%20.1%Anti PSPT28%96.1 %7.10.787.5%57.6 %62.4%24.1%Anti AnnV28%98 %14.30.793.3%58.1%63.4%26%Conventional APLs72%88.2%6.10.385.7%76.3 %79.8%60.2%Non-conventional APLS38%94.1%6.40.786.4%60.7 %66.3%32.1%All APLs82%86.3%6.000.2085.4%83 %84.2%68.3%Figure 1showing the comparative diagnostic performance of Conventional aPL testing vs Combined testing along with non-conventional aPLs in suspected obstetric APS scenarioConclusion:In a delicate situation like RPL, performance of non-conventional aPLs on their own, though not as sensitive as conventional aPLs, still demonstrate better specificity. Non-conventional APLs can newly identify 1/3rd of SNOAPS as APS. The real value of testing Anti PSPT &amp; Anti Ann V in RPL, is combined testing with conventional aPLs wherein they improve the sensitivity and accuracy of diagnosis of OAPS by 10% &amp; 4.4 % respectively, with only 1.9% drop in specificity. Non-conventional aPLs should be tested in SNOAPS.Disclosure of Interests:None declared

Antiphospholipid Antibodies and Fertility: No Impact on Ovarian Reserve in Premenopausal Women

Objective: To investigate possible differences in levels of ovarian reserve between antiphospholipid antibodies (aPL) asymptomatic carriers and antiphospholipid syndrome (APS) patients, by measuring the levels of anti-Müllerian hormone (AMH). Methods: We enrolled 69 premenopausal women divided in 2 groups: a) patients with APS, either primary (PAPS) or secondary (SAPS), according to the Sydney classification criteria; b) asymptomatic aPL carriers. Aged-matched premenopausal healthy donors (HDs) were also recruited. Complete aPL testing was performed and AMH levels were measured using enzyme-linked immunosorbent assay. Results: Among the 69 patients included in the study, 22 were diagnosed with PAPS, 13 with SAPS, and 14 patients were asymptomatic aPL carriers. No differences in AMH levels were observed among the three groups [mean AMH: PAPS 3.09 ng/ml ± 1.9 (range 1.02 − 7.1); SAPS 3.1 ng/ml ± 2.2 (range 1.1 − 7.6); aPL carriers 2.2 ng/ml ± 5.4 (range 1 − 6.3)] and between patients/aPL carriers and HDs [mean AMH 2.82 ng/ml ± 2.9 (range 1 − 6.9)]. Any correlation between the global APS score (GAPSS) and AMH levels failed to be found (rho = 0.31; p = 0.073). Conclusion: With the limitations of the current study, as observed in women with APS, we confirm that ovarian reserve, assessed with AMH levels, is not reduced in premenopausal women with isolated aPL positivity. Moreover, when granulating the aPL profile in terms of risk assessment, using the GAPSS, no impact on fertility was observed.

Prevalence of Helicobacter pylori among children in a training and research hospital clinic in Istanbul and comparison with Updated Sydney Classification Criteria.

OBJECTIVE:Helicobacter pylori (H. pylori) is a gram-negative bacterium and one of the reasons for gastritis, peptic and duodenal ulcers. It is a crucial public health problem for both children and adults, especially in developing countries. This study aims to investigate the prevalence of Helicobacter pylori positivity in children and to compare with updated Sydney classification criteria.METHODS:This study was conducted from January 2015 to June 2017. This study included 885 children aged 0-17 year(s). Endoscopic biopsies were evaluated for the diagnosis of infection due to H. pylori.RESULTS:The findings showed that 418 (47.2%) of 885 children were positive for H. pylori, and this positivity had a significantly increasing correlation with the presence of chronic inflammation, neutrophilic activity, lymphoid aggregates, and follicles. Erythematous pangastritis and antral nodularity on endoscopic findings had a correlation with H. pylori positivity.CONCLUSION:In this hospital-based study, the findings suggest that H. pylori infection is a problem for children and more extensive studies are needed to determine the prevalence of H. pylori positivity among children.

Comparative study of obstetric antiphospholipid syndrome (OAPS) and non-criteria obstetric APS (NC-OAPS): report of 1640 cases from the EUROAPS registry

To compare clinical features, laboratory data and fetal-maternal outcomes between 1000 women with obstetric APS (OAPS) and 640 with aPL-related obstetric complications not fulfilling Sydney criteria (non-criteria OAPS, NC-OAPS). This was a retrospective and prospective multicentre study from the European Registry on Obstetric Antiphospholipid Syndrome. A total of 1650 women with 5251 episodes, 3601 of which were historical and 1650 latest episodes, were included. Altogether, 1000 cases (OAPS group) fulfilled the Sydney classification criteria and 650 (NC-OAPS group) did not. Ten NC-OAPS cases were excluded for presenting thrombosis during follow-up. All cases were classified as category I (triple positivity or double positivity for aPL) or category II (simple positivity). Overall, aPL laboratory categories showed significant differences: 29.20% in OAPS vs 17.96% in NC-OAPS (P < 0.0001) for category I, and 70.8% in OAPS vs 82% in NC-OAPS (P < 0.0001) for category II. Significant differences were observed when current obstetric complications were compared (P < 0.001). However, major differences between groups were not observed in treatment rates, livebirths and thrombotic complications. In the NC-OAPS group, 176/640 (27.5%) did not fulfil Sydney clinical criteria (subgroup A), 175/640 (27.34%) had a low titre and/or non-persistent aPL positivity but did meet the clinical criteria (subgroup B) and 289/640 (45.15%) had a high aPL titre but did not fulfil Sydney clinical criteria (subgroup C). Significant clinical and laboratory differences were found between groups. Fetal-maternal outcomes were similar in both groups when treated. These results suggest that we could improve our clinical practice with better understanding of NC-OAPS patients.

Prevalence and associations of anti-phosphatidylserine/prothrombin antibodies with clinical phenotypes in patients with primary antiphospholipid syndrome: aPS/PT antibodies in primary antiphospholipid syndrome

The clinical significance of anti-phosphatidylserine/prothrombin (aPS/PT) in antiphospholipid syndrome (APS) is still controversial. We assessed the prevalence of aPS/PT antibodies, their association with other anti-phospholipid antibodies (aPL) and with different APS clinical phenotypes. We included 95 primary APS patients according to the Sydney classification criteria, and patients with thrombocytopenia and/or hemolytic anemia who also fulfilled the serological APS criteria. We tested aCL, anti-β2GP-I and aPS/PT antibodies (both IgG and IgM isotypes) and lupus anticoagulant (LA). We used χ2 test, Spearman's correlation coefficient, Mann-Whitney U test and logistic regression. Seventy-seven percent of patients had thrombosis, 50% hematologic involvement and 25% obstetric events (non-exclusive groups). Twenty patients had only hematologic features. The prevalence of IgG and IgM aPS/PT antibodies was 61% and 60%, respectively. Patients with LA+ had a higher prevalence and higher titers of IgG and IgM aPS/PT antibodies. aPS/PT antibodies correlated with aPL antibodies including LA. IgG aPS/PT antibodies were associated with thrombosis (OR 8.6 [95% CI 2.13-33.8, p = 0.002]) and pure hematologic features (OR 0.2, CI 95% 0.05-0.97, p = 0.004). IgM anti-β2GP-I antibodies conferred high risk for both hematologic (OR 7.9, 95% CI 1.88-34.61, p = 0.006) and thrombotic involvement (OR 7.4, 95% CI 1.76-31.12, p = 0.006). aPS/PT antibodies were highly prevalent and correlated with other aPL antibodies. IgG aPTS/PT conferred a high risk for thrombosis, but not for pure hematologic involvement. aPS/PT antibodies may be a useful serological tool in the diagnosis and phenotypic characterization of APS patients.

Antiplatelet and anticoagulant agents for primary prevention of thrombosis in individuals with antiphospholipid antibodies.

Antiplatelet and anticoagulant agents for primary prevention of thrombosis in individuals with antiphospholipid antibodies.

Closing the Serological Gap in the Antiphospholipid Syndrome: The Value of "Non-criteria" Antiphospholipid Antibodies.

Most clinicians use the 2006 Sydney classification criteria to evaluate patients suspected of having antiphospholipid syndrome (APS). Although sensitive and specific for APS, many patients fulfilling clinical criteria for the syndrome are persistently negative for the specific serological tests ("laboratory criteria"). These "seronegative APS" (SN-APS) patients can go undiagnosed and untreated until they experience serious clinical events. This study's objective was to describe antibody profiles of SN-APS patients using non-criteria markers, assess the clinical utility of these markers separately and in combination, and suggest incorporation into guidelines for patients suspected of APS. We categorized 175 consecutive patients suspected of APS into 2 subgroups: 107 fulfilling Sydney APS classification for seropositive APS (SP-APS) and 68 with clinical manifestations suggestive of APS but having negative serology, on 2 occasions, for criteria markers (SN-APS). On study inclusion, samples were retested for criteria and 11 non-criteria markers, including antiphosphatidylserine/prothrombin antibodies. Using 4 of 11 non-criteria tests, a cumulative 30.9% of SN-APS patients were detected. Combining results of all 11 non-criteria tests, 25 SN-APS (36.8%) and 89 SP-APS (83.2%) were positive for 1 or more non-criteria antibodies. Failure to diagnose APS can result in severe clinical consequences. Patients displaying clinical features of APS, but negative for conventional criteria markers, should undergo additional testing for non-criteria biomarkers. In our cohort, around one-third of SN-APS patients showed reactivity to 1 or more non-criteria markers. An update to the current classification criteria incorporating new serological markers should be considered to identify and stratify patients with APS for more effective treatment and management.

Assessment of the independent associations of IgG, IgM and IgA isotypes of anticardiolipin with thrombosis in SLE

ObjectiveThe Sydney classification criteria for antiphospholipid syndrome include lupus anticoagulant or moderate-to-high titre anticardiolipin IgG or IgM. We explored the association of all anticardiolipin isotypes, lupus anticoagulant and the combination...

The European Registry on Obstetric Antiphospholipid Syndrome (EUROAPS): A survey of 1000 consecutive cases.

To analyse the clinical features, laboratory data and foetal-maternal outcomes, and follow them up on a cohort of 1000 women with obstetric antiphospholipid syndrome (OAPS). The European Registry of OAPS became a registry within the framework of the European Forum on Antiphospholipid Antibody projects and was placed on a website in June 2010. Thirty hospitals throughout Europe have collaborated to carry out this registry. Cases with obstetric complaints related to antiphospholipid antibodies (aPL) who tested positive for aPL at least twice were included prospectively and retrospectively. The seven-year survey results are reported. 1000 women with 3553 episodes were included of which 2553 were historical and 1000 were latest episodes. All cases fulfilled the Sydney classification criteria. According to the laboratory categories, 292 (29.2%) were in category I, 357 (35.7%) in IIa, 224 (22.4%) in IIb and 127 (12.7%) in IIc. Miscarriages were the most prevalent clinical manifestation in 386 cases (38.6%). Moreover, the presence of early preeclampsia (PE) and early foetal growth restriction (FGR) appeared in 181 (18.1%) and 161 (16.1%), respectively. In this series, 448 (44.8%) women received the recommended OAPS treatment. Patients with recommended treatment had a good live-birth rate (85%), but worse results (72.4%) were obtained in patients with any treatment (low-dose aspirin (LDA) or low-molecular-weight heparin (LMWH) not on recommended schedule, while patients with no treatment showed a poor birth rate (49.6%). In this series, recurrent miscarriage is the most frequent poor outcome. To avoid false-negative diagnoses, all laboratory category subsets were needed. OAPS cases have very good foetal-maternal outcomes when treated. Results suggest that we were able to improve our clinical practice to offer better treatment and outcomes to OAPS patients.

Clinical advances of interest in the diagnosis and treatment of patients with antiphospholipid syndrome

In this paper we review recent studies and consensus documents that we consider relevant to the diagnosis and treatment of patients with antiphospholipid syndrome (APS). The diagnosis of APS is based on the Sydney classification criteria (2006), in which positive laboratory tests (anticardiolipin antibodies, anti-β2-glycoprotein I antibodies or lupus anticoagulant) are mandatory. However, it is not uncommon to see patients with clinical features highly suggestive of the syndrome in whom these antibodies are persistently negative. Therefore, we summarize the principal clinical and serological findings in a subgroup of patients with seronegative APS in the first series published up to date. In addition, a recent study draws attention to the safety and efficacy of the long-term use of low-molecular-weight heparins in patients with APS not susceptible to warfarin treatment. There is also a subgroup of women with APS and recurrent fetal loss with no response to the standard antithrombotic therapy; in this group the materno-fetal prognosis could be improved by the addition of low-dose prednisolone during the first trimester of pregnancy. Finally, we list the principal recommendations regarding thromboprophylaxis in APS drawn from the expert consensus document elaborated at the meeting held in Galvestone (2010).

Treatment of 139 Pregnancies in Antiphospholipid-positive Women Not Fulfilling Criteria for Antiphospholipid Syndrome: A Retrospective Study

The effect of low-dose aspirin (LDA) on pregnancy outcome in antiphospholipid (aPL)-positive women not fulfilling the criteria for antiphospholipid antibody syndrome (APS) was evaluated retrospectively. We evaluated 139 pregnancies of 114 aPL-positive women not fulfilling the Sydney classification criteria for definite APS (104 treated with LDA, 35 untreated). Inclusion criteria consisted of (1) any titer of aPL and no previous pregnancy or no pregnancy losses (defined as aPL carriers); (2) any titer of aPL and 1 or 2 pregnancy losses before the 10th gestational week. No women had previous thrombosis. The rate of pregnancy loss, gestational age at delivery, and birth weight percentile were compared in the treated and untreated patients. Associations between clinical and laboratory characteristics and pregnancy outcomes were investigated. The rate of pregnancy loss was low in both treated and untreated groups (7.7% vs 2.9%, respectively). There were no statistically significant differences in the rate of pregnancy loss, gestational age at birth, or birth weight percentile in the treated and untreated groups. There were significant associations between gestational age at birth ≤ 34th week and positivity for lupus anticoagulant (p = 0.025) and anti-ß2-glycoprotein I IgG antibodies at titers > 99th (p = 0.016). LDA treatment does not appear to improve pregnancy outcome in low-risk women not fulfilling the criteria for APS. Because antibody profile seems to influence pregnancy outcome, further studies of patients stratified according to their antibody profile are warranted.