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2.
Lupus ; 21(7): 761-3, 2012 Jun.
Article in English | MEDLINE | ID: mdl-22635225

ABSTRACT

In this review preliminary data on the follow-up of 141 babies born to mothers with antiphospholipid syndrome are reported. In spite of maternal treatment, the rate of both preterm delivery and low birth weight were 16 and 17%, respectively. At birth, no clinical evidence of perinatal thrombosis was observed. Placental transfer of antiphospholipid antibodies occurred in 20, 25 and 43% of cases for lupus anticoagulant, anticardiolipin and anti-ß2-glycoprotein I antibodies, respectively. At 24 months of follow-up, four children showed behaviour abnormalities suggesting the possible need for long-term neurological evaluation in this clinical setting.


Subject(s)
Antiphospholipid Syndrome/epidemiology , Infant, Newborn , Pregnancy Complications/epidemiology , Registries , Europe/epidemiology , Female , Follow-Up Studies , Humans , Longitudinal Studies , Pregnancy , Prospective Studies
3.
Minerva Pediatr ; 62(3 Suppl 1): 25-7, 2010 Jun.
Article in English | MEDLINE | ID: mdl-21089714

ABSTRACT

The registry is an European, multicentre, prospective and longitudinal study which follows a cohort of children born to mothers with antiphospholipid syndrome (APS). In this article we report preliminary results obtained from 138 mothers and 141 babies (three twin pregnancies). At birth, 16.3% of neonates were less than 37 weeks of gestation and 17% were low birth weight; in addition, 11.3% of neonates were small for gestational age. No cases of neonatal thrombosis were observed. During follow-up period five children showed behavioral abnormalities. A long term clinical follow-up will be necessary to evaluate the neuropsychological development of these children.


Subject(s)
Antiphospholipid Syndrome/epidemiology , Pregnancy Complications/epidemiology , Pregnancy Outcome , Registries , Antibodies, Antiphospholipid/blood , Autistic Disorder/epidemiology , Autistic Disorder/etiology , Child, Preschool , Europe , Female , Follow-Up Studies , Humans , Immunity, Maternally-Acquired , Infant , Infant, Low Birth Weight , Infant, Newborn , Infant, Small for Gestational Age , Infant, Very Low Birth Weight , Learning Disabilities/epidemiology , Learning Disabilities/etiology , Pregnancy , Pregnancy, Multiple , Premature Birth/epidemiology , Prospective Studies , Psychomotor Disorders/epidemiology , Psychomotor Disorders/etiology , Thrombosis/congenital , Thrombosis/epidemiology , Twins
4.
Lupus ; 18(10): 889-93, 2009 Sep.
Article in English | MEDLINE | ID: mdl-19671788

ABSTRACT

The Euro-Phospholipid project started in 1999 with a multicentre, consecutive and prospective design. A total cohort of 1000 patients with antiphospholipid syndrome (APS), derived from 13 countries (Belgium, Bulgaria, Denmark, France, Germany, Greece, Hungary, Israel, Italy, the Netherlands, Portugal, Spain and United Kingdom), has been followed since then. This project allowed the identification of the prevalence and characteristics of the main clinical and immunological manifestations at the onset and during the evolution of APS and demonstrated that it is possible to recognize more homogeneous subsets of clinical significance. Patients with APS associated with systemic lupus erythematosus (SLE) had more episodes of arthritis, livedo reticularis and more frequently exhibited thrombocytopenia and leucopenia. Female patients had more episodes of arthritis and livedo reticularis - both connected with the higher prevalence of migraine and SLE-related APS in women, while male patients had more myocardial infarction, epilepsy and lower limb arterial thrombosis. Childhood onset patients presented more episodes of chorea and jugular vein thrombosis, whereas older onset patients were more frequently male and had more strokes and angina pectoris, but less frequently livedo reticularis.


Subject(s)
Antiphospholipid Syndrome/epidemiology , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/mortality , Cohort Studies , Europe/epidemiology , Follow-Up Studies , Humans , Morbidity , Prospective Studies
5.
Lupus ; 18(10): 900-4, 2009 Sep.
Article in English | MEDLINE | ID: mdl-19671790

ABSTRACT

The registry is a prospective, European, multicentric, longitudinal study, which follows a cohort of children born to mothers with antiphospholipid syndrome (APS). It was started in 2003. In this report, we update the results obtained from the study of 110 mothers and 112 children (two twin births). Eighty per cent of the mothers (n = 86) had primary APS. Purely obstetrical, thrombotic and mixed (obstetrical and thrombotic) APS represent 65.5 %, 21.8 % and 12.7 % of the whole cohort respectively. Isolated antiphospholipid antibodies and isolated anticardiolipin antibodies positivity were present in 50 of 109 (46%) and in 34 of 109 (31%) of the pregnant women, respectively. In the babies, in spite of a high rate of prematurity (14.3%) with four (3.6%) of the premature babies born before 33 weeks of gestation and an increased number of newborns small for gestational age (17%), the large majority of the neonates were healthy. Thirty-one infants are now older than 24 months. Among them, three displayed behavioural abnormalities before 3 years of age. After completing data, there will be the possibility to evaluate the newborn status in relation to the mothers' diseases, treatments and antibodies and to follow the neuropsychological development and immunological evolution of the babies during the next 5 years.


Subject(s)
Antiphospholipid Syndrome/epidemiology , Pregnancy Complications/epidemiology , Registries , Antiphospholipid Syndrome/immunology , Europe/epidemiology , Female , Humans , Infant, Newborn , Longitudinal Studies , Pregnancy , Pregnancy Complications/immunology , Prospective Studies
6.
Lupus ; 18(10): 920-3, 2009 Sep.
Article in English | MEDLINE | ID: mdl-19671793

ABSTRACT

The antiphospholipid antibodies included as laboratory criteria of the antiphospholipid syndrome (APS) are antibodies reacting with anionic phospholipids - anticardiolipin antibodies and lupus anticoagulant - and with beta(2)-glycoprotein I. However, antibodies reacting with phosphatidylethanolamine (aPE), a zwitterionic phospholipid, have also been described to be associated with the main features of APS. The objectives of this review are to describe the characteristics of aPE and to bring attention to recent evidence that aPE are correlated with the main clinical features of APS, notably, in the absence of the laboratory criteria of this syndrome.


Subject(s)
Antibodies, Antiphospholipid/blood , Antiphospholipid Syndrome/immunology , Phosphatidylethanolamines/immunology , Abortion, Habitual/etiology , Antiphospholipid Syndrome/complications , Enzyme-Linked Immunosorbent Assay , Humans , Thrombosis/etiology
7.
Ann Rheum Dis ; 68(9): 1428-32, 2009 Sep.
Article in English | MEDLINE | ID: mdl-18801761

ABSTRACT

OBJECTIVES: To identify the main causes of morbidity and mortality in patients with antiphospholipid syndrome (APS) during a 5-year period and to determine clinical and immunological parameters with prognostic significance. METHODS: The clinical and immunological features of a cohort of 1000 patients with APS from 13 European countries who had been followed up from 1999 to 2004 were analysed. RESULTS: 200 (20%) patients developed APS-related manifestations during the 5-year study period. Recurrent thrombotic events appeared in 166 (16.6%) patients and the most common were strokes (2.4% of the total cohort), transient ischaemic attacks (2.3%), deep vein thromboses (2.1%) and pulmonary embolism (2.1%). When the thrombotic events occurred, 90 patients were receiving oral anticoagulants and 49 were using aspirin. 31/420 (7.4%) patients receiving oral anticoagulants presented with haemorrhage. 3/121 (2.5%) women with only obstetric APS manifestations at the start of the study developed a new thrombotic event. A total of 77 women (9.4% of the female patients) had one or more pregnancies and 63 (81.8% of pregnant patients) had one or more live births. The most common fetal complications were early pregnancy loss (17.1% of pregnancies) and premature birth (35% of live births). 53 (5.3% of the total cohort) patients died. The most common causes of death were bacterial infection (21% of deaths), myocardial infarction (19%) and stroke (13%). No clinical or immunological predictor of thrombotic events, pregnancy morbidity or mortality was detected. CONCLUSION: Patients with APS still develop significant morbidity and mortality despite current treatment (oral anticoagulants or antiaggregants, or both).


Subject(s)
Antiphospholipid Syndrome/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Anticoagulants/administration & dosage , Antiphospholipid Syndrome/drug therapy , Antiphospholipid Syndrome/immunology , Child , Child, Preschool , Drug Utilization/statistics & numerical data , Epidemiologic Methods , Europe/epidemiology , Female , Humans , Infant , Infant, Newborn , Male , Middle Aged , Prognosis , Thrombosis/epidemiology , Young Adult
9.
Lupus ; 16(8): 634-41, 2007.
Article in English | MEDLINE | ID: mdl-17711900

ABSTRACT

Perinatal thrombosis in infants born to mothers with antiphospholipid antibodies (aPL) is a rare event, but with risk of death or severe sequelae. We analysed 16 infants with such perinatal thrombosis reported in the literature in the last 20 years. Thromboses were arterial (13/16), mostly strokes (8/16). Hydrops fetalis with left renal vein thrombosis was associated to a lupus anticoagulant (LA) present only in the child. Risk factors additional to aPL: either prenatal (preeclampsia and/or intra-uterine growth retardation) or perinatal (asphyxia, sepsis, arterial or venous catheter and congenital thrombophilia) were present (one to four of them) in nine out of the 14 evaluable babies. aPL were the only risk factor found in five full term babies who suffered from stroke in four cases and from renal thrombosis in another. Eleven of these infants with aPL in their serum presented a neonatal APS with the same antibody (LA or aCL IgG) found in neonates and their mothers, while the other infants had thrombosis with aPL only in their mother's blood. aCL IgM was only found in one neonate who suffered from sepsis. Thrombosis treatments were diverse. This analysis suggests that women with aPL should be investigated for other thrombophilic risk factors and that aPL should be detected systematically at birth in the offspring of mothers with APS.


Subject(s)
Antibodies, Antiphospholipid/blood , Thrombosis/epidemiology , Antiphospholipid Syndrome/blood , Antiphospholipid Syndrome/complications , Birth Weight , Female , Humans , Infant, Newborn , Male , Pregnancy , Risk Factors , Thrombosis/prevention & control
10.
Lupus ; 13(9): 713-7, 2004.
Article in English | MEDLINE | ID: mdl-15485110

ABSTRACT

This prospective multicentric register was initiated by the European Forum of Antiphospholipid Antibodies (APL) in 2003 after approval by local ethic committees. This register allows the investigation of infants after written informed parental consent. It collects mothers' clinical pattern of antiphospholipid syndrome (APS), course and outcome of pregnancy, treatment and immunological status. For the babies, clinical and immunological examinations are performed at birth; neurodevelopmental conditions followed up to five years. A re-evaluation of lupus anticoagulant (LA), anticardiolipin (ACL) or other antibodies will be done if they are positive at birth to follow their kinetics. A descriptive and a case control study of babies with versus without APL at birth will be possible after the inclusion of 300 cases.


Subject(s)
Antiphospholipid Syndrome , Infant, Newborn, Diseases/etiology , Pregnancy Complications , Registries , Antiphospholipid Syndrome/complications , Autoantibodies/analysis , Europe , Female , Follow-Up Studies , Humans , Infant, Newborn , Multicenter Studies as Topic , Pregnancy , Pregnancy Outcome
12.
J Clin Immunol ; 23(5): 377-83, 2003 Sep.
Article in English | MEDLINE | ID: mdl-14601646

ABSTRACT

Two-hundred ninety five patients with the antiphospholipid syndrome (APS) were studied for the presence of antibodies against six anti-beta2GPI-related peptides Abs. The prevalence of a wide spectrum of clinical and laboratory parameters of APS was evaluated in all patients, and correlated with the presence of each anti-beta2GPI peptide antibody. The rates of the various antipeptides Abs ranged from 18.0 to 63.7%. Altogether, 87.1% of the patients had antibody reactivity against at least one of the six beta2GPI-related peptides. A high degree of simultaneous reactivity against several beta2GPI-peptides was found. Positive and negative correlations were found between several antipeptides Abs and the rates of thrombosis and fetal loss. Our results point to a heterogeneous activity of antiphospholipid Abs in APS patients, directed, often concurrently, against various epitopes of the beta2GPI molecule. Evaluation of APS patients for the presence of specific antipeptides Abs may be of a value in predicting the risk for future thrombotic and obstetrical complication, as well as for specific therapeutic purposes.


Subject(s)
Antibodies/immunology , Antiphospholipid Syndrome/immunology , Glycoproteins/immunology , Peptides/chemistry , Peptides/immunology , Adolescent , Adult , Amino Acid Sequence , Antiphospholipid Syndrome/etiology , Glycoproteins/chemistry , Humans , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/immunology , Middle Aged , Molecular Sequence Data , beta 2-Glycoprotein I
13.
Lupus ; 12(7): 499-503, 2003.
Article in English | MEDLINE | ID: mdl-12892387

ABSTRACT

Hypothetical circumstances that may require prophylaxis for a potential antiphospholipid syndrome (primary prophylaxis), or in some instances when there already had been some manifestations ofthe syndrome (secondary prophylaxis), were presented to a panel of experts for their consideration on potential prophylactic intervention. These were subsequently presented to the participants in the First International Consensus on Treatment of the Antiphospholipid Syndrome. In most instances there was consensus in adding low dose aspirin, an exception being aspirin allergy when other antiaggregants could be used in nonpregnant subjects. General measures to prevent thrombosis and other vasoprotective actions should also be provided. Higher risk of fetal loss or thrombosis called for anticoagulation with coumadin in nonpregnant subjects or subcutaneous low molecular weight heparin in pregnant ones. When indicated, prophylaxis of the antiphospholipid syndrome should be provided in systemic lupus erythematosus patients who are being treated for their disease. In no instance should corticosteroids or immunosuppresants be given as prophylactic of an antiphospholipid syndrome.


Subject(s)
Anticoagulants/therapeutic use , Antiphospholipid Syndrome/complications , Thrombosis/prevention & control , Antiphospholipid Syndrome/prevention & control , Aspirin/therapeutic use , Female , Humans , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Platelet Aggregation Inhibitors/therapeutic use , Pregnancy , Pregnancy Complications, Hematologic/prevention & control , Thrombosis/etiology
14.
Lupus ; 12(7): 530-4, 2003.
Article in English | MEDLINE | ID: mdl-12892393

ABSTRACT

The term 'catastrophic' antiphospholipid syndrome (APS) is used to define an accelerated form of APS resulting in multiorgan failure. Although catastrophic APS patients represent less than 1% of all patients with APS, they are usually in a life-threatening medical situation that requires high clinical awareness. The careful and open discussion of several proposals by all participants in the presymposium workshop on APS consensus, held in Taormina on occasion of the 10th International Congress on aPL and chaired by Munther A Khamashta and Yehuda Shoenfeld (29 September 2002), has allowed the acceptation of a preliminary set of classification criteria. On the other hand, the optimal management of catastrophic APS must have three clear aims: to treat any precipitating factors (prompt use of antibiotics if infection is suspected, amputation for any necrotic organ, high awareness in patients with APS who undergo an operation or an invasive procedure), to prevent and to treat the ongoing thrombotic events and to suppress the excessive cytokine 'storm'. Anticoagulation (usually intravenous heparin followed by oral anticoagulants), corticosteroids, plasma exchange, intravenous gammaglobulins and, if associated with lupus flare, cyclophosphamide, are the most commonly used treatments for catastrophic APS patients.


Subject(s)
Antiphospholipid Syndrome/classification , Antiphospholipid Syndrome/therapy , Multiple Organ Failure/etiology , Antiphospholipid Syndrome/complications , Humans , Multiple Organ Failure/therapy
15.
Thromb Haemost ; 86(2): 575-83, 2001 Aug.
Article in English | MEDLINE | ID: mdl-11522006

ABSTRACT

Despite the widely recognized practical importance of anticardiolipin (aCL) ELISA, the reliability of this test has been recently discussed. In order to investigate this area on European scale, we sent to 30 experienced centers a questionnaire focusing on the diagnostic procedures applied to patients with antiphospholipid syndrome (APS) and on the detailed protocols used to perform aCL. Anticardiolipin ELISA was found to be the most frequently performed test in patients with suspected APS, but significant difference was shown among the various protocols. The cross-laboratory multiple examination of ten serum samples evaluated independently by the 24 centers pointed out the difficulty in getting comparable results. Therefore a "consensus" protocol was derived from the aCL methods giving the best performance. The materials and reagents necessary to perform the "consensus" method, including, as putative standards, one IgG and one IgM monoclonal antibody (HCAL and EY2C9) were distributed to 19 Centers. The results of one IgG and one IgM aCL high positive sera measured in serial dilutions were compared. A progressive decrease in the variability of the values obtained for a given sample appeared evident when all the laboratories used the same standard, in their own in-house ELISA and even more in the "consensus" ELISA. Our data show that aCL ELISA standardization is necessary in order to obtain comparable results in different laboratories.


Subject(s)
Antibodies, Anticardiolipin/blood , Adult , Antibodies, Monoclonal , Antiphospholipid Syndrome/diagnosis , Data Collection , Decision Making , Enzyme-Linked Immunosorbent Assay/methods , Enzyme-Linked Immunosorbent Assay/standards , Female , Humans , Immunoglobulin G , Immunoglobulin M , Male , Middle Aged , Observer Variation , Reference Standards , Reproducibility of Results
16.
Int Immunol ; 13(2): 203-10, 2001 Feb.
Article in English | MEDLINE | ID: mdl-11157853

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) is an uncommon disease of an unknown etiology, characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia, fever and acute thrombotic complications, especially within the cerebral circulation. Although anti-endothelial cell antibodies (AECA) have occasionally been shown to be present in TTP, their role in the pathogenesis of the disease has never been ascertained. In the current study we demonstrated the pathogenic activity of affinity-purified anti-endothelial cell F(ab)2 antibodies (AECA/TTP) from four consecutive patients with active TTP. These AECA/TTP bound to and activated only microvascular endothelial cells (EC) and not large vessel EC. The specificity of AECA/TTP binding to microvascular EC was confirmed by competition assay employing membranes derived from small and large vessels EC. Activation included enhanced IL-6 and von Willebrand factor release from the EC followed by increased expression of adhesion molecules P-selectin, E-selectin and vascular cell adhesion molecule-1 on the EC, as evaluated by ELISA. Increased expression of adhesion molecules was followed by an increase in monocyte adhesion to EC. The level of soluble thrombomodulin (TM) also increased in the culture medium of activated microvascular EC upon exposure to AECA/TTP antibodies and was directly correlated to a decrease in cell-associated TM. Our data suggest that AECA/TTP directed against microvascular EC could play a pathogenic role in the development of endothelial injury in TTP that leads to thrombosis.


Subject(s)
Autoantibodies/physiology , Endothelium, Vascular/immunology , Endothelium, Vascular/metabolism , Purpura, Thrombotic Thrombocytopenic/immunology , Antibody Specificity , Autoantibodies/blood , Binding Sites, Antibody , Biomarkers/blood , Bone Marrow Cells/immunology , Cell Adhesion/immunology , Cell Line , Cell Line, Transformed , Endothelium, Vascular/pathology , Humans , Interleukin-6/metabolism , Microcirculation/immunology , Microcirculation/metabolism , Monocytes/immunology , Monocytes/metabolism , Purpura, Thrombotic Thrombocytopenic/blood , Thrombomodulin/biosynthesis , von Willebrand Factor/metabolism
19.
Rev Med Interne ; 21(3): 266-77, 2000 Mar.
Article in French | MEDLINE | ID: mdl-10763188

ABSTRACT

INTRODUCTION: Thromboembolic venous disease, which includes both peripheral venous thrombosis and pulmonary embolism, is a frequent disorder in patients with cancer. Although thromboembolic manifestations may precede the diagnosis of cancer, the value of extensive clinical search for potential underlying cancer when faced with venous thromboembolic manifestations has not been demonstrated. CURRENT KNOWLEDGE AND KEY POINTS: Clinical and biological studies have demonstrated that acquired abnormalities in blood hemostasis, especially procoagulant factors, account for the onset of thromboembolic manifestations in patients with cancer. Classical anticoagulant therapy is associated with low efficacy and tolerance in patience with cancer who are at high risk for hemorrhagic complications and recurrence of thromboembolic disease. FUTURE PROSPECTS AND PROJECTS: Recent data suggest the value of anticoagulant therapy using either low molecular weight heparin or warfarin at low doses (INR < 2) according to the specific surgical or medical context.


Subject(s)
Neoplasms/complications , Thromboembolism/etiology , Anticoagulants/therapeutic use , Blood Coagulation/physiology , Blood Platelets/physiology , Humans , Neoplasms/therapy , Primary Prevention/methods , Risk Factors , Thromboembolism/blood , Thromboembolism/diagnosis , Thromboembolism/epidemiology , Thromboembolism/therapy
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