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Antiphospholipid syndrome (APS) is a systemic autoimmune disorder with vascular, obstetric, and hematological manifestations associated with thrombotic and inflammatory mechanisms orchestrated by antiphospholipid (aPLs) antibodies. Current clinical practice in APS is highly variable duo to lack of high quality of evidence. Here, Chinese Rheumatology Association developed recommendations for management of APS in China. The recommendations cover the early diagnosis, disease evaluation, thrombotic risk assessment, and treatment.
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Objective:To study clinical characteristics and pregnancy outcomes under anti-coagulation therapy of non-criteria obstetric antiphospholipid syndrome.Methods:Patients suspected of obstetric antiphospholipid syndrome(OAPS) were recruited through Chinese Rheumatism Data Center from 2015 to 2019 consecutively. Patients fulfilling 2006 Sydney revised antiphospholipid syndrome criteria were classified as OAPS. Patients fulfilling definition of non-criteria OAPS(NCOAPS) by expert consensus on diagnosis and management of obstetric antiphospholipid syndrome of China were classified as NCOAPS. Clinical characteristics and laboratory results of two groups were compared. Live birth rates and pregnancy outcomes under anti-coagulation therapy were studied.Results:A total of 88 patients were enrolled, including 56 patients (63.6%) as OAPS, 32(36.4%) as NCOAPS. Live births were only reached in 16.1% (9/56) in OAPS patients and 12.5%(4/32) in NCOAPS. Fetal losses after 10 weeks of gestation and pre-eclampsia before 34 weeks were more common in OAPS group compared to NCOAPS group [78.6%(44/56) vs. 18.8%(6/32), P<0.001; 25.0%(14/56) vs. 3.1%(1/32), P=0.020, respectively]. After enrollment, 15 pregnancies were recorded in OAPS, 10 in NCOAPS, all of whom were treated with low-dose aspirin (LDA) combined with low-molecular weight heparin (LMWH). Live birth rates saw dramatic improvements compared to baseline levels in OAPS [16.1% (9/56) vs. 11/15] along with NCOAPS [12.5% (4/32) vs. 7/10]. Conclusion:Though NCOAPS and OAPS patients differ in antiphospholipid antibody spectrum and pattern of pregnancy morbidities, both groups benefit from LDA combined with LWMH treatment, as live birth rates improve. Non-criteria OAPS patients are recommended to receive anti-coagulation therapy during pregnancy.
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Background: Antiphospholipid syndrome (APS) is an autoimmune disorder characterised by thrombosis and/or pregnancy morbidity in the presence of antiphospholipid antibodies (aPLs) based on the Sydney criteria. We aimed to explore the clinico-laboratory features and treatment strategies of APS patients retrospectively. Methodology: The medical records of APS patients registered under Hospital Universiti Sains Malaysia (Kelantan state) between 2000 and 2015 were reviewed. Results: A total of 17 APS subjects (age 40.7 ± 12.8 years) including 11 primary (64.7%) and six secondary APS (35.3%) patients were identified. The follow-up period was 9.5 ± 6.7 years with male:female ratio of 1.0:4.7. Pregnancy morbidity was the most common clinical manifestation (11/14; 78.6%) followed by recurrent venous thrombosis (10/17; 58.8%). For other clinical features, menorrhagia was the most frequently observed manifestation (4/14; 28.6%) followed by aPLs-associated thrombocytopenia (4/17; 23.5%) and ovarian cyst (3/14; 21.4%). LA and aCL were positive in 94.1% (16/17) and 81.8% (9/11) of the patients, respectively. APTT value (76.7 ± 17.0 sec) was significantly high (p < 0.05). Low intensity warfarin alone was successful to maintain target INR (2.0 - 3.0) and prevent recurrence of thrombosis. Conclusion: The tendency of pregnancy morbidity in this cohort of Malaysian Kelantanese APS patients was high compared to other previously reported APS cohorts. Low intensity warfarin was successful in preventing recurrence of thrombosis, however, APS women receiving long-term anticoagulants should be monitored for possible occurrence of menorrhagia and ovarian cysts.
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Antecedentes: El síndrome antifosfolípido (SAF) se caracteriza por manifestaciones clínicas de trombosis o morbilidad gestacional y la presencia de anticuerpos antifosfolípidos (aAFL), que se pueden unir al trofoblasto o al endotelio materno, alterando la placentación normal. Objetivo: Evaluar el efecto del suero de mujeres con SAF en un modelo tridimensional de remodelación vascular in vitro. Métodos: Cuantificación sistematizada con el programa Image J y el complemento Angiogenesis Analyzer de la interacción de trofoblasto y endotelio en un modelo tridimensional de remodelación vascular y detección por ELISA del factor de crecimiento del endotelio vascular (VEGF). Se incluyeron 25 mujeres: con morbilidad gestacional y trombosis vascular (MG/TV, n=7) y con morbilidad gestacional únicamente (MG, n=8), ambos grupos con presencia de aAFL; con morbilidad gestacional sin aAFL (MG/aFL-, n=10), y un grupo control de mujeres sanas (SHN, n=7). Resultados: El suero de mujeres con morbilidad gestacional, MG/aAFL-, MG y MG/TV indujo disminución de la angiogénesis endotelial, pero en trofoblasto, únicamente el suero de los dos grupos de mujeres con aAFL tuvo este mismo efecto en concordancia con la reducción del VEGF. El suero de mujeres con MG/aAFL- y MG/TV redujo elementos angiogénicos en el co-cutivo de trofoblasto y endotelio, comparado con el grupo control de SHN. En contraste con el grupo de MG/aAFL-, el suero de mujeres con aAFL, redujo la angiogénesis en células trofoblásticas y endoteliales. Conclusión: El efecto anti-angiogénico de los aAFL se observó focalizado en trofoblasto y el suero del grupo de mujeres con MG/TV indujo mayores efectos deletéreos.
Background: Antiphospholipid syndrome (APS) is characterized by manifestations of thrombosis or pregnancy morbidity and antiphospholipid antibodies (aAPL) which can bind to trophoblast or to maternal endothelium, altering normal placentation. Aims: To evaluate the effect of sera from patients with APS on a three-dimensional in vitro model of vascular remodeling. Methods: Systematic quantification of the interaction between trophoblast and endothelium with the software Image J and the plug-in Angiogenesis Analyzer in a three-dimensional model of vascular remodeling and the detection of vascular endothelial growth factor (VEGF) by ELISA. 25 women divided in three groups were included as follows: with pregnancy morbidity and thrombosis (PM/VT, n=7), pregnancy morbidity (PM, n=8), both groups with aAPL; pregnancy morbidity without aAPL (PM/aAPL-, n=10) and healthy women was used as control (NHS, n=7). Results: Sera from women with gestational morbidity: PM/VT, PM, PM/aAPL-, decreased endothelial angiogenesis, but on trophoblast, only the sera of two groups of women with aAPL had this effect along with reduction of VEGF. Sera from women with PM/aAPL- and PM/VT reduced angiogenesis in the co-culture of trophoblast and endotelial cells compared to NHS. In contrast to PM/aAPL-, sera from women with APS decreased angiogenesis in trophoblastic cells. Conclusions: The anti-angiognic effect of aAPL was focused on trophoblast and sera from PM/VT induced higher deleterious effects.
Subject(s)
Humans , Female , Adult , Antiphospholipid Syndrome , Neovascularization, Physiologic , Pregnancy Complications , Vascular RemodelingABSTRACT
El síndrome antifosfolípido es un trastorno multisistémico adquirido y una importante causa de trombosis venosas o arteriales, así como también de morbilidad en el embarazo. Puede ser primario o secundario, este último sobre todo en pacientes con lupus eritematoso sistémico, infecciones y consumo de algunas drogas. Se exponen determinados elementos sobre sus manifestaciones clínicas y los criterios de clasificación actualizados para el diagnóstico. El tratamiento se basa en medidas de profilaxis antitrombóticas y control de los factores de riesgo asociados; pero aún muchos aspectos clínicos y de laboratorio concernientes a esta hipercoagulabilidad por la presencia de anticuerpos contra los fosfolípidos, se hallan sujetos a discusión e investigación.
The antiphospholipid syndrome is an acquired multisystemic disorder and an important cause of venous or arterial thrombosis, as well as of morbidity in pregnancy. It can be primary or secondary, the last one mainly in patients with systemic lupus erythematosus, infections and consumption of some drugs. Certain elements on its clinical manifestations and the updated classification criteria for the diagnosis are exposed. The treatment is based on antithrombotic prevention measures and control of the associated risk factors; but many clinical and laboratory aspects concerning this hypercoagulability due to the presence of antibodies against phospholipids, are still under discussion and research.
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BACKGROUND: The presence of lupus anticoagulants (LA) is a strong risk factor for thrombosis in antiphospholipid syndrome. We investigated the usefulness of addition of silica clotting time (SCT) to the pre-existing dilute Russell's viper venom test (dRVVT) for detection of LA. Also, we analyzed differences in the thrombotic features and the characteristics of antiphospholipid antibodies between dRVVT and SCT. METHODS: A total of 167 patients positive for LA or anti-cardiolipin (anti-CL) antibody and 76 healthy controls were enrolled. The dRVVT and SCT were used for detection of LA. Anti-CL, anti-beta2-glycoprotein I (anti-beta2 GPI) and anti-prothrombin (anti-PT) antibodies were measured using commercial ELISA kits. RESULTS: In detection of thrombosis, the sensitivity of the combined test of SCT and dRVVT was 56.4%, which was higher than that of dRVVT alone (46.2%) or SCT alone (23.1%). The specificity of the combined test (80.9%) was comparable to that of dRVVT (81.9%). Also, odds ratio for predicting thrombosis was higher in the combined test than in dRVVT or SCT alone. When normalized LA ratio of the two tests was compared, the group of patients with higher ratio of SCT showed significantly higher prevalence of recurrent abortion and higher positivity of IgG types of anti-CL, anti-beta2 GPI and anti-PT than the group with higher ratio of dRVVT. CONCLUSIONS: Addition of SCT to dRVVT can improve the detection sensitivity of thrombosis in LA test. And the high normalized LA ratio of SCT may be a useful parameter for detection of recurrent abortion.