scholarly journals Does Adjusted Global Antiphospholipid Syndrome Score (aGAPSS) Predict the Obstetric Outcome in Antiphospholipid Antibody Carriers? A Single-Center Study

2021 ◽  
Author(s):  
Sara Del Barrio-Longarela ◽  
Víctor M. Martínez-Taboada ◽  
Pedro Blanco-Olavarri ◽  
Ana Merino ◽  
Leyre Riancho-Zarrabeitia ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Antiphospholipid Syndrome ◽  
Adverse Pregnancy Outcome ◽  
Clinical Manifestations ◽  
Live Birth Rate ◽  
Response To Treatment ◽  
Antiphospholipid Antibody ◽  
Single Center Study ◽  
Patients At Risk ◽  
Adverse Pregnancy

AbstractThe adjusted Global Antiphospholipid Syndrome (APS) Score (aGAPSS) is a tool proposed to quantify the risk for antiphospholipid antibody (aPL)-related clinical manifestations. However, aGAPSS has been validated mainly for thrombotic events and studies on APS-related obstetric manifestations are scarce. Furthermore, the majority of them included patients with positive aPL and different autoimmune diseases. Here, we assess the utility of aGAPSS to predict the response to treatment in aPL carriers without other autoimmune disorders. One-hundred and thirty-seven women with aPL ever pregnant were included. Sixty-five meet the APS classification criteria, 61 had APS-related obstetric manifestations, and 11 were asymptomatic carriers. The patients’ aGAPSS risk was grouped as low (< 6, N = 73), medium (6–11, N = 40), and high risk (≥ 12, N = 24). Since vascular risk factors included in the aGAPSS were infrequent in this population (< 10%), the aGAPSS score was mainly determined by the aPL profile. Overall, the live birth rate was 75%, and 37.2% of the patients had at least one adverse pregnancy outcome (APO). When considering patients according to the aGAPSS (high, medium, and low risk), no significant differences were found for pregnancy loss (29.2%, 25%, and 21.9%) or APO (33.3%, 47.5%, and 32.9%). In the present study, including aPL carriers without other autoimmune diseases, aGAPSS is not a valuable tool to identify patients at risk for obstetric complications despite treatment. In these patients with gestational desire, in addition to the aPL profile, other pregnancy-specific factors, such as age or previous obstetric history, should be considered.

scholarly journals Prevention of Pregnancy Complications in Antiphospholipid Syndrome

2020 ◽  
Vol 40 (02) ◽  
pp. 174-183
Author(s):  
Andreas Czwalinna ◽  
Frauke Bergmann
Keyword(s):  
Antiphospholipid Syndrome ◽  
Pregnancy Complications ◽  
Antiphospholipid Antibodies ◽  
Birth Rate ◽  
Clinical Symptoms ◽  
Adverse Pregnancy Outcome ◽  
Live Birth Rate ◽  
Obstetric Antiphospholipid Syndrome ◽  
Adverse Pregnancy ◽  
Definition Of

AbstractDespite a lot of research on antiphospholipid antibodies (aPL), standardization of test systems, and better definition of its clinical symptoms, the pathomechanism of this acquired autoimmune disease is not yet fully explained. Progress in treatment increased the live birth rate in 70 to 80% of women suffering from obstetric antiphospholipid syndrome (OAPS). However, still 20 to 30% will develop adverse pregnancy outcome. Lack of awareness of this disorder as the cause for pregnancy complications is very harmful to mothers and to their newborns. Complications can be avoided or minimized by proper treatment. The aim of this article is to increase the awareness of gynecologists and medical personal for OAPS.

scholarly journals Case of an unusual diagnosis of primary antiphospholipid syndrome with multiple clinical complications

2020 ◽  
Vol 2020 (12) ◽  
Author(s):  
Stathis Tsiakas ◽  
Chrysanthi Skalioti ◽  
Paraskevi Kotsi ◽  
Ioannis Boletis ◽  
Smaragdi Marinaki
Keyword(s):  
Antiphospholipid Syndrome ◽  
Thrombotic Event ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Young Male ◽  
Multiple Organ ◽  
Systemic Autoimmune Disease ◽  
Antiphospholipid Antibody ◽  
Primary Antiphospholipid Syndrome ◽  
Wide Range

ABSTRACT Antiphospholipid syndrome (APS) is a systemic autoimmune disease defined by the presence of antiphospholipid antibodies in association with thrombotic events and/or obstetric complications. Renal involvement is not infrequent in both primary and secondary APS. Kidney manifestations comprise a wide range of clinical features, including hypertension, major renal vessel thrombosis or microvascular endothelial injury, also described as APS nephropathy. In the absence of a thrombotic event, clinical manifestations of APS are often non-specific. We recently encountered a case of primary APS in a young male with newly diagnosed hypertension and renal impairment. The diagnosis of APS was initially suspected by his kidney biopsy findings, when electron microscopy examination showed the features of chronic microangiopathy, and was later confirmed by a triple positive antiphospholipid antibody profile and multiple organ involvement.

The antiphospholipid (antibody) syndrome

2011 ◽  
pp. 343-352
Author(s):  
Alan J. Hakim ◽  
Gavin P.R. Clunie ◽  
Inam Haq
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Autoimmune Diseases ◽  
Venous Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Lupus Anticoagulant ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Pregnancy Morbidity

Introduction 344 Epidemiology and pathology 345 Clinical features of antiphospholipid syndrome 346 Treatment of antiphospholipid syndrome 348 Catastrophic antiphospholipid syndrome 350 The antiphospholipid syndrome (APS) was first described in the 1980s and comprises arterial and venous thrombosis with or without pregnancy morbidity in the presence of anticardiolipin (ACL) antibodies or the lupus anticoagulant (LAC). It can be primary, or secondary to other autoimmune diseases, most commonly systemic lupus erythematosus (SLE) (...

scholarly journals Evaluation of Anticoagulant Use during Pregnancy in Mothers with Antiphospholipid Syndrome: An Observational Cross-Sectional Study

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5062-5062 ◽  
Author(s):  
Inolyn Pandjaitan ◽  
Karmel L Tambunan ◽  
Tubagus Djumhana Atmakusuma
Keyword(s):  
Birth Weight ◽  
Pregnant Women ◽  
Antiphospholipid Syndrome ◽  
Conflicts Of Interest ◽  
Adverse Pregnancy Outcome ◽  
Antiplatelet Agent ◽  
Elevated Concentration ◽  
Antiphospholipid Antibody ◽  
Reduced Rate ◽  
D Dimer

Abstract Introduction: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by vascular thrombosis and/or pregnancy morbidity with persistent elevated concentration of antiphospholipid antibody. The use of anticoagulant in pregnant women with APS has reduced implantation failure, reduced rate of placental insufficiency, increased rate of delivery of live infant, and reduced rate of abortion. However, compared to its beneficial effects, anticoagulant therapy during pregnancy can be challenging because of the burden of the treatment (self-injecting anticoagulant for 9 months, cost of the treatment) and potential anticoagulant side effects. The purpose of this study was to evaluate the outcome of pregnant women with APS with anticoagulant. Method: We searched through medical records of pregnant women who visited hematology oncology clinic of Cikini CCI Hospital from year 2000 to 2017. Pregnant women with positive antiphospholipid antibodies who were treated with anticoagulant and followed up until presence of outcome were included in the study. Main outcome was any delivery of live infant above 22 weeks gestational age. Activated partial thromboplastin time (aPTT), d dimer level, birth weight and length were also documented. Results: We identified 92 pregnancies in 88 mothers diagnosed with antiphospholipid syndrome who were prescribed anticoagulant. From 92 pregnancies, 67 (73%) and 25 (27%) patients were prescribed low molecular weight heparin (LMWH) and unfractionated heparin (UFH), respectively, with combination of low dose antiplatelet agent. APTT and d dimer level of every patients were monitored monthly. 80% of the pregnancies successfully resulted in live births. The proportion of successful delivery of healthy infant in patients treated with UFH (21 out of 25; 84%) was greater than LMWH (53 out of 67; 79%) but not statistically different (p = 0,708) (Figure 1). Birth weight and length between groups were similar. Conclusion: UFH may be used as safe and cost-effective alternative treatment to LMWH in pregnant women with APS. Despite the use of anticoagulant and antiplatelet agents, 20% of patients still had adverse pregnancy outcome. Disclosures No relevant conflicts of interest to declare.

High positive antibody titers and adverse pregnancy outcome in women with antiphospholipid syndrome

2011 ◽  
Vol 90 (12) ◽  
pp. 1428-1433 ◽  
Author(s):  
MICHAL J. SIMCHEN ◽  
MORDECHAI DULITZKI ◽  
GUY ROFE ◽  
HAGIT SHANI ◽  
PNINA LANGEVITZ ◽  
...  
Keyword(s):  
Pregnancy Outcome ◽  
Antiphospholipid Syndrome ◽  
Adverse Pregnancy Outcome ◽  
Positive Antibody ◽  
Antibody Titers ◽  
Adverse Pregnancy

Diagnosis of the antiphospholipid syndrome: A proposal for use of laboratory tests

Lupus ◽  
1998 ◽  
Vol 7 (2_suppl) ◽  
pp. 144-148 ◽  
Author(s):  
EN Harris ◽  
SS Pierangeli ◽  
AE Gharavi
Keyword(s):  
Autoimmune Diseases ◽  
Antiphospholipid Syndrome ◽  
Lupus Anticoagulant ◽  
Laboratory Tests ◽  
Pregnancy Loss ◽  
Clinical Manifestations ◽  
Sensitive Test ◽  
Microtiter Plates ◽  
Specific Determination

The presence of antiphospholipid (aPL) antibodies has been associated with thrombosis, pregnancy loss and thrombocytopenia in the antiphospholipid syndrome (APS). The anticardiolipin and the lupus anticoagulant tests are frequently used to detect aPL antibodies. The anticardiolipin ELISA utilizes cardiolipin coated on polystyrene plates as antigen and is a very sensitive test but lacks specificity, since it can be positive in a number of infectious (such as syphilis, HIV) and autoimmune diseases other than APS. In an effort to improve specificity, new ELISA techniques that employ alternative antigens (such as β2-glycoprotein 1, particularly when coated onto oxidized microtiter plates or mixture of phospholipids) have been developed. Several investigators have reported that these new assays enable more specific determination of aPL antibodies and thus can be used more reliably for the diagnosis and confirmation of APS. This article examines the results of those studies, including data that shows correlations of these assays with clinical manifestations of APS, and proposes a new protocol for the use of laboratory tests in the diagnosis of APS.

scholarly journals AB1051 KIKUCHI FUJIMOTO DISEASE, IS IT SLE?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1815.3-1816
Author(s):  
J. Camins-Fàbregas ◽  
V. Ortiz-Santamaria ◽  
N. Busquets-Pérez ◽  
A. Cuervo ◽  
I. Cañas Alcántara ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Autoimmune Diseases ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Response To Treatment ◽  
Systemic Autoimmune Disease ◽  
Histiocytic Necrotizing Lymphadenitis ◽  
Systemic Autoimmune Diseases ◽  
Necrotizing Lymphadenitis

Background:Kikuchi-Fujimoto disease (KFD) is a rare entity characterized by adenopathies and fever. It raises a broad differential diagnosis that includes lymphoproliferative disorders, infections and systemic autoimmune diseases, and diagnostic confirmation is always by histology, which shows histiocytic necrotizing lymphadenitis. Although its course is generally benign and self-limited, it can be associated both at the time of diagnosis and during follow-up with systemic autoimmune diseases, the most frequent of which is systemic lupus erythematosus (SLE).Objectives:To describre the clinical and analytical characteristics of patients diagnosed with KFD and the development of systemic autoimmune disease.Methods:Patients diagnosed with KFD during the 1990s and 2020s are collected in a regional hospital (Granollers General Hospital). The clinic is documented at the diagnosis of EKF, the appearance of systemic autoimmune disease during follow-up and its clinical and analytical characteristics.Results:A total of 7 patients with EKF were diagnosed. All of them women with a mean age at diagnosis of 30 years. Diagnosis was made in all cases with compatible clinical symptoms, fever and lymphadenopathy, and lymph node biopsy confirming histiocytic necrotizing lymphadenitis. At the time of diagnosis, a patient was also diagnosed with SLE. During the follow-up, 4 of the 6 remaining patients developed clinical manifestations compatible with SLE (3 of them with systemic manifestations and a case of subacute cutaneous lupus. The mean time of onset of SLE was 34 months (between 6 months and 5 years). All of them received treatment with hydroxychloroquine, with good response to treatment.The clinical and analytical characteristics are presented in Table 1 below.Conclusion:In our center, 5 of the 7 patients (71%) diagnosed with EKF developed manifestations compatible with SLE. The importance of the diagnosis of EKF lies precisely in the possible association with systemic autoimmune disease, the most common being SLE, so it is recommended that patients be monitored to identify those who develop associated autoimmune disease.Disclosure of Interests:None declared

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