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The authors reflect on the new development of anticoagulant for the treatment of patient with antiphospholipid syndrome (APS). They note that the warfarin has been introduced as an anticoagulant therapy but is considered unsafe due to its anticoangulant effect in APS patient. They mentions that several novel anticoagulants have been developed such as rivaroxaban which can offer more predictable and effective anticoagulation than warfarin.

The 8th edition of the International Congress on Autoimmunity took place in the beautiful and historical city of Granada, Spain, from 9 to 13 March 2012. It gathered more than 2350 participants from 71 different countries, including internists, immunologists, rheumatologists, basic researchers and many other clinicians and laboratory people interested in autoimmune diseases who were updated with the latest available diagnostic tools and new therapeutic avenues. This top international autoimmunity meeting put together an international faculty of more than 100 experts to present a high-level scientific program under the leadership of Professor Yehuda Shoenfeld (Zabludowicz Center for Autoimmune Diseases, Tel-Hashomer, Israel) who chaired the congress together with Ricard Cervera (Hospital Clínic, Barcelona, Catalonia, Spain), Angela Tincani (University of Brescia, Italy) and Carlos Vasconcelos (Hospital S. António, Porto, Portugal) as co-chairpersons. [ABSTRACT FROM AUTHOR]

Abstract: Background: To evaluate the clinical significance of IgG/IgM antibodies directed against prothrombin (PT) in a homogeneous cohort of patients with primary APS (PAPS). Methods: IgG/IgM anti-prothrombin (aPT) antibodies were measured using a commercial ELISA kit in 158 PAPS patients and in 214 control subjects (100 healthy blood donors and 114 patients with autoimmune diseases). Results: IgG/IgM aPT antibodies were significantly associated with PAPS (OR, 95% CI: 52.0, 7.0–385.5; 9.8, 1.2–80.8, respectively). They were found to have a high specificity (IgG 99.50%, IgM 99.54%) but a low sensitivity (IgG 19.60%, IgM 3.80%) for PAPS. IgG aPT antibodies were significantly higher in the PAPS patients with thrombosis (OR, 95% CI: 69.2, 9.2–519.1) as well as in those with pregnancy morbidity alone (OR, 95% CI: 20.5, 2.4–174.5). The prevalence of IgG aPT was not significantly different in the thrombotic and obstetric patients, and the presence of IgM aPT antibodies was significant only in patients with thrombosis (OR, 95% CI: 2.6, 1.6–110.8). Conclusions: The study''s findings confirm that IgG/IgM aPT antibodies are significantly associated with PAPS and indicate that IgG aPT antibodies are associated with clinical subsets of the disease. For the time being, however, the lower sensitivity of IgG/IgM antibodies with respect to conventional aPL antibodies precludes their inclusion in the recommendations for the diagnosis of PAPS. [Copyright &y& Elsevier]

A letter to the editor is presented which discusses the clinical study on the anticardiolipin antibodies (aCL) downregulation effect of rituximab (RTX) in treating antiphospholipid syndrome (APS) patients.

Antiphospholipid syndrome (APS) often presents as a multisystem disorder characterized by recurrent arterial and venous thrombosis and pregnancy loss. We present a 46-year-old female who was admitted with oliguric acute renal failure. APS was suspected due to prolongation of activated partial thromboplastin time and history of miscarriages. Investigations for secondary causes were normal. Renal biopsy findings revealed thrombotic microangiopathy. A complete recovery of renal function was attained with a combination of plasma exchange and pulse methylprednisolone followed by oral steroids and warfarin. The case is presented for its rarity and therapeutic implications. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Heparin Treatment in Antiphospholipid Syndrome With Recurrent Pregnancy Loss: A Systematic Review and Meta-analysis," by P. D. Ziakas and colleagues in a 2010 issue, along with a response from the authors of the article.

A 32-year old woman with antiphospholipid antibody syndrome (APS) developed severe thrombocytopenia, elevated liver enzymes and progressive cerebral thrombosis a few days after preterm delivery by caesarean section. Her condition deteriorated despite treatment with low dose aspirin, anticoagulation by heparin and iv glucocorticoid administration. Intravenous immunoglobulin (IVIG) on three consecutive days was followed by rapid resolution of her neurological impairment and increasing platelets counts. The temporal association between IVIG and reversal of both neurological impairment and platelet number strongly indicates a specific effect of IVIG administration in this condition. It is proposed that IVIG therapy is considered as a therapeutic option in APS patients with progressive cerebral infarction despite optimal use of anticoagulant and immunomodulating agents. [ABSTRACT FROM AUTHOR]

Antiphospholipid syndrome (APS) is a disorder characterized by antiphospholipid antibody positivity, arterial or venous thrombosis, and fetal loss. In APS, renal as well as vascular and glomerular involvement is observed. Systemic lupus erythematosus and other connective tissue diseases should be excluded to diagnose primary APS. Immunoglobulin M (IgM) nephropathy is characterized by single or dominant IgM deposition in glomerular mesangium. It often presents with hematuria and proteinuria. In a 45-year-old female patient admitted to our clinic with diabetes mellitus and proteinuria, fundus examination did not reveal diabetic retinopathy but a high anticardiolipin IgM and venous thrombosis in the upper extremity were observed. Renal biopsy revealed IgM nephropathy. The patient was diagnosed with primary APS and IgM nephropathy. Cyclophosphamide and steroid treatment was started. Her proteinuria decreased as a result of the treatment. Although, it is reported in the literature that primary APS coexists with other glomerulonephritis, we did not detect coexistence of primary APS and IgM nephropathy. [ABSTRACT FROM AUTHOR]

IgA anti-beta2-glycoprotein I (IgA-aB2GPI) antibodies are currently not included as a laboratory criterion of antiphospholipid syndrome (APS). In the 13th International Congress on Antiphospholipid Antibodies, Galveston, TX, (USA) in 2010, these antibodies were accepted as an APS laboratory criterion in patients who had clinical manifestations of APS but were negative for ''consensus'' antiphospholipid antibodies (aPL) (IgG and IgM isotypes). Consequently, individuals with thrombotic events who are negative for consensus aPL may be undiagnosed for APS. The most recent publications have confirmed that IgA-aB2GPI antibodies are a risk factor for thrombotic events. In this viewpoint, we propose that IgA-aB2GPI antibodies should be included as an APS consensus criterion and that we have to help Cinderella become a princess. [ABSTRACT FROM AUTHOR]

We describe a patient with systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APLS) who developed a plaque-like lesion around the mouth and lost all body hair. Biopsies of the circumoral lesion and scalp, were originally reported as containing extensive basal cell carcinoma, but on review, both showed the typical appearance of a benign malformation of the hair follicle known as basaloid follicular hamartoma. Regrowth of hair and partial resolution of the peri-oral plaque occurred with more aggressive treatment of her SLE, but the basaloid follicular hamartomas in her scalp skin persisted. There is a known, but rare, association between this pattern of basaloid follicular hamartoma, alopecia and myasthenia gravis, but only two cases have been described in association with SLE and none with APLS. [ABSTRACT FROM AUTHOR]

The article discusses the use of drug belimumab in antiphospholipid syndrome (APS). Topics include autoimmune disease characterized by thrombosis or pregnancy morbidity with persistently positive antiphospholipid antibodies (aPL); patients with aPL experience thrombocytopenia, cardiac valve disease, and nephropathy; and uses of anticoagulation for the management of APS patients.

Background: Antiphospholipid syndrome is an autoimmune disorder characterized by the presence of antiphospholipid antibodies and commonly presents with vascular thromboembolic phenomena, thrombocytopenia, and obstetric complications. Antiphospholipid syndrome can be classified as either primary or secondary to other connective tissue diseases. Dermatologic manifestations are common; however, non-vasculitic skin ulceration is an uncommon manifestation of antiphospholipid syndrome with limited treatment options.Case Presentation: In this paper we report the case of a 58-year-old white woman who developed necrotic abdominal wall ulcers 27 years after a diagnosis of secondary antiphospholipid syndrome associated with systemic lupus erythematosus. The ulcers developed despite our patient being on therapeutic anticoagulation with warfarin and were resistant to further increases in the intensity of anticoagulation. Management was further complicated due to reluctance on the part of our patient to switch over to injectable heparin.Conclusions: This case highlights a rare late dermatologic presentation of antiphospholipid syndrome, which responded poorly to conventional anticoagulation with warfarin. Current management is limited to experimental therapies and the role of newer anticoagulants is still unknown. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 51-year-old man with a 2-day history of cognitive decline. Following Magnetic resonance imaging (MRI), Computed tomography (CT) brain scan and cranio-cervical CT angiogram he was diagnosed with antiphospholipid syndrome and was treated with low-molecular-weight heparin and a vitamin K antagonist. After recovery a further cranio-cervical CT angiogram showed partial revascularisation of both internal carotid arteries.

Purpose: Anti-phospholipid antibodies (aPL) analyzed by line immunoassay (LIA) can recognize beta2-glycoprotein I (β2GPI) domain 1 (D1) epitopes depending on β2GPI binding to distinct phospholipids. The aPL LIA was compared with consensus ELISA to investigate whether both techniques can discriminate anti-phospholipid syndrome (APS) patients from aPL-positive, systemic autoimmune rheumatic diseases (SARD) patients without clinical symptoms of APS and controls.Methods: Thirty-four APS patients (14 arterial/venous thrombosis, 16 pregnancy morbidity, and 4 both), 41 patients with SARD lacking clinical APS criteria but demonstrating positivity for anti-β2GPI (aβ2GPI) IgG, and 20 healthy subjects (HS) were tested for aPL to cardiolipin (aCL), phosphatidic acid, phosphatidylcholine, phosphatidylethanolamine, phosphatidylglycerol (aPG), phosphatidylinositol, phosphatidylserine, β2GPI, prothrombin, and annexin V by LIA. Samples were also tested for aCL, aβ2GPI, aβ2GPI-domain 1 (aD1), and aβ2GPI-domains 4-5 (aD4-5) by ELISA and for lupus anti-coagulant.Results: Comparison of LIA with ELISA revealed a good agreement for the consensus criteria aPL aβ2GPI and aCL IgG (kappa = 0.69, 0.68, respectively) and a moderate agreement for IgM (kappa = 0.52, 0.49, respectively). Regarding ELISA, aD1/aD4-5 demonstrated the best performance of differentiating APS from asymptomatic SARD [area under the curve (AUC): 0.76]. aPG IgG had the best performance by LIA (AUC: 0.72) not significantly different from aD1/aD4-5. There was a good agreement for aPG IgG with aD1/aD4-5 (kappa = 0.71).Conclusions: aD1/aD4-5 (ELISA) and aPG IgG (LIA) differentiate APS from SARD patients. PG appears to interact with β2GPI of APS patients and exposes D1 thereof for disease-specific aPL binding in LIA. [ABSTRACT FROM AUTHOR]

Primary Antiphospholipid antibody syndrome is a rare disease associated with thromboembolic events which may affect either the arterial or the venous vasculature. It presents with an increased risk of thrombosis in pregnant woman leading to repeated fetal losses. We present here a case of primary antiphospholipid antibody syndrome in young women who had previous event of gangrene of toes leading to their amputation and repeated fetal losses. [ABSTRACT FROM AUTHOR]

The article presents the author's views on a research conducted by researcher J. Gebhart and colleagues in which they analyzed treatment of patients suffering from lupus anticoagulant (LA). Topics discussed includes symptoms of antiphospholipid syndrome (APS), its pathogenesis, pathophysiology of thrombosis in APS patients.

The antiphospholipid syndrome (APS) is defined by the development of venous and/or arterial thromboses, often multiple, and pregnancy morbidity (mainly, recurrent fetal losses), in the presence of antiphospholipid antibodies (aPL). Some estimates indicate that the incidence of the APS is around 5 new cases per 100,000 persons per year and the prevalence around 40–50 cases per 100,000 persons. The aPL are positive in approximately 13% of patients with stroke, 11% with myocardial infarction, 9.5% of patients with deep vein thrombosis and 6% of patients with pregnancy morbidity. Currently, there is consensus in treating APS patients with thrombosis with long-term oral anticoagulation and to prevent obstetric manifestations with the use of aspirin and heparin. This review summarizes the main knowledge on the clinical and therapeutic aspects of this syndrome. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Treatment of refractory obstetric antiphospholipid syndrome: The state of the art and new trends in the therapeutic management," by J. Alijotas-Reig in the 2013 issue.

The article presents a case study of a 22 month-old boy who was admitted to the clinic with cyanosis and was later diagnosed with recurrent modified Blalock-Taussig shunt thrombosis. Topics include transthoracic echocardiography of the patient which showed transposition of great arteries and pulmonary stenosis, administeration of heparin infusion and oral aspirin to prevent graft thrombosis in the patient, and finding that antiphospholipid syndrome levels should be measured in such patients.

Pathogenesis, treatment options and outcomes for antiphospholipid syndrome (APS) are poorly defined. A new observational collaborative study has analysed morbidity and mortality in APS, but, ultimately, the data that will guide us to optimally treat patients with APS are lacking. Here we provide a construct for future large-scale collaborative studies. [ABSTRACT FROM AUTHOR]

The article discusses two case studies including one of a 40-year-old man with epileptic crises and other of a 43-year-old man with clinical symptoms of livedo reticularis, systemic hypertension and ulcers on legs. The report of arteriography and ulcer biopsy revealed presence of antiphospholipid syndrome (APS) and polyarteris nudosa in both the patients. The article discusses the association between APS and systemic vasculitis along with the required therapeutic intervention.

A letter to the editor is presented in response to the article "Treatment of refractory obstetric antiphospholipid syndrome: The state of the art and new trends in the therapeutic management," by J. Alijotas-Reig, and discusses two alternative treatments for the disease.

The author reflects on the association between antiphospholipid antibodies and the brain and low molecular weight (LMW) heparin treatment. The author states that brain involvement has been recognized as a common and central feature of antiphospholipid syndrome (APS), which involve headache, balance problems and memory loss. The author discusses the heparin therapeutic trial for patients who failed anti-platelet therapy.

Information about the 8th Meeting of the European Forum on Antiphospholipid Antibodies which was held in 2012 is presented. The meeting focused on antiphospholipid syndrome (APS) and enabled European researchers to share information with those from the U.S. Physician Piero Marson discussed role of the University of Padua Medical School in development of modern medicine. It also threw light on forum's ongoing studies and projects related to APS.

The article discusses a study on the efficacy and safety of long-term low molecular weight heparin (LMWH) in the treatment of patients with antiphospholipid syndrome (APS). It aims to describe the outcomes of patients with APS who were treated with LMWH for different reasons including warfarin failure, no neurological improvement, and major bleeding with warfarin. It also shows that osteoporosis was the only side-effect present in a few patients.

A 25-year-old Bangladeshi lady presented to neurology with a three-month history of involuntary movements of her right arm, associated with loss of power. There was progression to the right leg, and she subsequently developed episodes of slurred speech and blurred vision. At the time of presentation, she was 12 weeks pregnant and the symptoms were reported to have started at conception. Past medical history was unremarkable apart from one first trimester miscarriage and there was no significant family history suggestive of a hereditary neurological condition. MRI of the head revealed no abnormalities but serology showed positive antinuclear antibodies (ANAs) at a titre of 1/400. Further investigations revealed strongly positive anticardiolipin antibodies (>120) and positive lupus anticoagulant antibodies. The patient had a second miscarriage at 19 weeks gestation strengthening the possibility that the chorea was related to antiphospholipid antibody syndrome and she was started on a reducing dose of Prednisolone 40 mg daily and aspirin 300 mg daily. Sixmonths later, she had complete resolution of neurological symptoms. There are several reports of chorea as a feature of antiphospholipid syndrome, but no clear consensus on underlying pathophysiology. [ABSTRACT FROM AUTHOR]

With the exception of the catastrophic antiphospholipid syndrome (APS), the management of patients with APS has been largely supportive aiming at avoiding a recurrent thrombotic event; it is noteworthy that data concerning therapy targeting the triggering factor (the antiphopsholipid antibodies) are scarce. We report a case of APS manifested as recurrent fetal losses, ischemic stroke and renal dysfunction with concomitant nephrotic syndrome successfully treated with the combination of plasmapheresis and anti-CD20 antibody. [ABSTRACT FROM AUTHOR]