Antibodies, Antiphospholipid ; immunology ; therapeutic use ; Antiphospholipid Syndrome ; diagnosis ; immunology ; therapy ; Child ; Humans

Antibodies, Antiphospholipid ; blood ; immunology ; Antiphospholipid Syndrome ; diagnosis ; etiology ; immunology ; Child, Preschool ; Diagnosis, Differential ; Humans ; Male ; Partial Thromboplastin Time

Antiphospholipid syndrome (APS) is primarily considered to be an autoimmune pathological condition that is also referred to as "Hughes syndrome". It is characterized by arterial and/or venous thrombosis and pregnancy pathologies in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disease or secondary to a connective tissue disorder, most frequently systemic lupus erythematosus (SLE). Damage to the nervous system is one of the most prominent clinical constellations of sequelae in APS and includes (i) arterial/ venous thrombotic events, (ii) psychiatric features and (iii) other non- thrombotic neurological syndromes. In this overview we compare the most important vascular ischemic (occlusive) disturbances (VIOD) with neuro-psychiatric symptomatics, together with complete, updated classifications and hypotheses for the etio-pathogenesis of APS with underlying clinical and laboratory criteria for optimal diagnosis and disease management.
Antibodies, Antiphospholipid/immunology ; Antiphospholipid Syndrome/diagnosis/*immunology/therapy ; Arterial Occlusive Diseases/diagnosis/*immunology/therapy ; Cerebrovascular Disorders/diagnosis/immunology/therapy ; Humans ; Lupus Erythematosus, Systemic/diagnosis/immunology/therapy

Adult ; Antibodies, Anticardiolipin ; blood ; Antibodies, Antiphospholipid ; blood ; Autoantibodies ; blood ; Female ; Humans ; Male ; Middle Aged ; Phosphatidic Acids ; immunology ; Phosphatidylcholines ; immunology ; Phosphatidylethanolamines ; immunology ; Phosphatidylinositols ; immunology ; Phosphatidylserines ; immunology ; Reference Values

Systemic lupus erythematosus (SLE) is a prototype for multi-system, autoimmune diseases of unknown etiology, characterized by the production of autoantibodies. SLE can involve any organ system of the body with constitutional symptoms, including musculoskeletal, skin, renal, neuropsychiatric, cardiovascular, respiratory and gastrointestinal systems. These wide spectra of disease manifestations have made disease classification difficult. American College of Rheumatology (ACR) proposed classification criteria for SLE for research purpose in 1982, which had been widely used for research purpose and not for diagnosis. In 1997, these criteria were updated with further recognition of antiphospholipid antibodies, but not validated. But ACR criteria didn't still meet the necessity for earlier diagnosis of SLE. In order to improve clinical relevance and incorporate new knowledge to the field of lupus immunology, the Systemic Lupus Erythematosus International Collaborating Clinics (SLICC), an international lupus expert group dedicated to clinical research on lupus, revised the ACR systemic lupus classification criteria in 2012. The new 2012 SLICC criteria were validated using a large set of patient scenarios rated by experts. The history and diagnostic utility of SLE criteria are covered in this review.
Allergy and Immunology ; Antibodies, Antiphospholipid ; Autoantibodies ; Autoimmune Diseases ; Classification* ; Diagnosis ; Humans ; Lupus Erythematosus, Systemic* ; Rheumatology ; Skin

Primary antiphopholipid syndrome (APS) is a disease producing vascular thrombus with antiphospholipid antibody without association with autoimmune diseases as systemic lupus erythematosus. Retinal vein occlusion is a rare vascular manifestation in primary APS. We describe 2 cases of primary APS presenting with developing blurred vision. Each had central retinal vein occlusion and high titer of IgG anticardiolipin antibody.
Adult ; Antibodies, Anticardiolipin/analysis ; Antiphospholipid Syndrome/*complications/immunology ; Case Report ; Human ; Male ; Middle Age ; Retinal Vein Occlusion/*etiology/immunology

BACKGROUNDThe management of patients with recurrent miscarriage (RM) and antiphospholipid antibody syndrome (APS) includes prolonged treatment with heparin and aspirin, starting from the confirmation of pregnancy and continuing until 6 weeks after birth. This study was conducted to determine the relationship between changes in antiphospholipid antibody titers and clinical outcomes. The effect of a shortened treatment regimen was also evaluated.

METHODSA prospective study of 123 patients with RM and APS between March 2012 and May 2014 was conducted. Patients were pretreated with a low dose of prednisone plus aspirin before pregnancy, and heparin was added after conception. The levels of antiphospholipid antibodies and pregnancy outcomes were evaluated.

RESULTSAll patients were positive for anti-β2-glycoprotein 1 (anti-β2-GP1) IgM. After prepregnancy treatment with low-dose prednisone plus aspirin, 99 of 123 patients became pregnant, and 87 of those pregnancies resulted in successful live births, while 12 resulted in miscarriage, showing a success rate of 87.9%. In the live birth group, levels of anti-β2-GP1 were 56.8 ± 49.0 RU/ml before the pretreatment regimen, 32.1 ± 26.0 RU/ml after 2 months of pretreatment, and 24.1 ± 23.1 RU/ml during early pregnancy (P < 0.05). In the miscarriage group, antiphospholipid antibody titers were 52.8 ± 30.7 RU/ml before pretreatment, 38.5 ± 34.2 RU/ml after pretreatment, and 33.9 ± 24.7 RU/ml during early pregnancy; the decrease in antiphospholipid antibodies was lower in the miscarriage group than in the live birth group (P < 0.05). Of the 24 infertile patients, the average antibody titer did not decline after pretreatment (P = 0.802).

CONCLUSIONSAnti-β2-GP1 IgM was the predominant form of antibody in patients with RM and APS. The decreases in antiphospholipid antibody titers correlated with better pregnancy outcomes. The shorter treatment regimen was effective and economical.

Abortion, Habitual ; immunology ; prevention & control ; Adult ; Antibodies, Antiphospholipid ; immunology ; Anticoagulants ; therapeutic use ; Antiphospholipid Syndrome ; drug therapy ; immunology ; Aspirin ; therapeutic use ; Female ; Heparin ; therapeutic use ; Humans ; Live Birth ; Prednisone ; therapeutic use ; Pregnancy ; Pregnancy Complications ; prevention & control ; Pregnancy Outcome ; Prospective Studies

PURPOSE: The effects of maternal systemic lupus erythematosus (SLE) on neonatal prognosis were examined by comparing clinical features of full-term babies born to lupus mothers and age- and parity-matched controls. PATIENTS and METHODS: From January 2000 to December 2005, 39 singletons were born to 37 SLE women. Excluding 11 cases of prematurity and preeclampsia, 28 full-term neonates formed the lupus group. The control group included 66 full-term babies. The retrospective study examined medical records and compared gestational age, birth weight, days of hospital stay, small for gestational age (SGA) frequency, Apgar scores < 7, and parity. Lupus neonates were tested for anti-nuclear antibody (ANA) and platelet count, and electrocardiogram was performed. RESULTS: Average gestational age (38 vs. 39 weeks, p < 0.05) and birth weight (2,775 vs. 3,263g, p < 0.05) were significantly different between the SLE and control groups. SGA frequency was higher in the SLE group (25% vs. 4.5%, p < 0.05). No significant difference was observed in Apgar score, birth weight, gestational age, SGA frequency, and platelet count between lupus subgroups formed based on anti-dsDNA antibody levels and antiphospholipid antibody status. CONCLUSION: The association of maternal ANAs, antiphospholipid antibodies, and drug history with neonatal prognosis could not be elucidated. However, even in uncomplicated pregnancies, maternal lupus is disadvantageous for gestational age, birth weight, and SGA frequency.
Adult ; Antibodies, Antinuclear/immunology ; Antibodies, Antiphospholipid/immunology ; Disease Susceptibility/immunology/pathology ; Female ; Humans ; Infant, Newborn ; Lupus Erythematosus, Systemic/*complications/immunology ; Male ; Platelet Count ; Pregnancy ; *Pregnancy Complications ; *Pregnancy Outcome ; Prognosis ; Risk Factors

OBJECTIVEAntiphospholipid antibody (APL) is a particularly important laboratory diagnostic criterion for antiphospholipid syndrome (APS). The significances of positive APL in childhood are seldom reported nor fully understood. The purpose of this study was to analyze 13 cases with positive APL seen in our hospital and to study the relationship between the positive rates of APL and various clinical diseases especially systemic lupus erythematosus (SLE) in order to improve the clinical diagnoses and treatment level of APS in children.

METHODSThe clinical data collected from 2000 to 2002 of 13 hospitalized children with positive APL were retrospectively evaluated. Enzyme linked immunosorbent assay (ELISA) and indirect immunofluorescence technique were used respectively to detect APL and antineutrophil cytoplasmic autoantibodies (ANCA) of sera from those children. Other various indexes were also detected according to different characteristics of different diseases.

RESULTSEight cases had SLE; 2 had acute post-streptococcal infections. The other 3 cases did not show any evidences of primary diseases; they probably had primary APS. SLE was the most common primary diseases to cause development of APL and the cases with SLE showed more severe cutaneous vasculitis than SLE patients who were negative for APL. There was no significant relationship between the positive rates of APL and that of ANCA. Eight APL positive cases complicated with thrombocytopenia and bleeding were treated with high dosage of immunoglobulin [400 mg/(kg.d), for 3 - 5 d] intravenously; the clinical conditions of these cases were ameliorated soon. While the 5 cases who had thrombotic vasculitis and thromboembolism were treated with anticoagulant and antithrombotic therapy with low molecular weight heparin [50 - 100 U/(kg.d)], which led to good clinical effects.

CONCLUSIONSThe clinical manifestations of children positive for APL were somehow different from those of adults. Positive APL itself may be nonspecific, it can occur from different causes of diseases. APL detection may be useful to suggest anticoagulant and/or antithrombosis therapy. Treatments for APS should be variable according to different causes and severity of diseases, in the cases of thrombocytopenia and bleeding, high dose intravenous immunoglobulin should be given as soon as possible, while in the cases of thrombotic vasculitis and thromboembolism, anticoagulant and antithrombotic therapy should be given soon.

Adult ; Antibodies, Antineutrophil Cytoplasmic ; blood ; Antibodies, Antiphospholipid ; blood ; immunology ; Anticoagulants ; therapeutic use ; Antiphospholipid Syndrome ; blood ; complications ; diagnosis ; therapy ; Child ; Fibrinolytic Agents ; therapeutic use ; Hemorrhage ; etiology ; therapy ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Lupus Erythematosus, Systemic ; immunology ; Streptococcal Infections ; immunology ; Thrombocytopenia ; etiology ; therapy ; Thromboembolism ; drug therapy ; etiology ; Thrombosis ; drug therapy ; etiology ; Vasculitis ; drug therapy ; etiology

BACKGROUND: The presence of antiphospholipid antibodies (aPLs) is associated with the clinical features of antiphospholipid syndrome (APS), which comprises venous and arterial thrombosis and pregnancy loss, and systemic lupus erythematosus (SLE). The prevalence of aPLs has been reported to be different in patient populations affected by either of these conditions. We performed a retrospective study to evaluate the prevalence and clinical associations of aPLs, including lupus anticoagulant (LAC), anticardiolipin (aCL), and anti-beta2-glycoprotein I antibodies (anti-beta2-GPI) in a cohort of Korean patients with SLE. METHODS: This study included samples from 88 SLE patients for whom aPL testing had been advised between June 2006 and July 2009 at the Dong-A University Hospital. Serum and plasma samples were tested for LAC, aCL (IgG, IgM), and anti-beta2-GPI (IgG, IgM) antibodies. Clinical data from patients were obtained from a review of medical records. RESULTS: LAC was the most common (34.1% of total patients, 30/88) antibody, followed by IgM aCL (31.8%, 28/88), IgG aCL (18.2%, 16/88), and IgM and IgG anti-beta2-GPI (both 5.7%, 5/88 each). Positivity for LAC was strongly associated with venous/arterial thrombosis (P=0.002). CONCLUSIONS: LAC was the most common antibody detected in Korean SLE patients and is shown to have a significant association with the presence of venous/arterial thrombosis. The measurement of LAC may be clinically useful in identifying patients with SLE who are at a high risk for venous/arterial thrombosis.
Adolescent ; Adult ; Antibodies, Anticardiolipin/*blood ; Antibodies, Antiphospholipid/*blood ; Cohort Studies ; Female ; Humans ; Immunoglobulin G/blood ; Immunoglobulin M/blood ; Lupus Coagulation Inhibitor/*blood ; Lupus Erythematosus, Systemic/epidemiology/*immunology ; Male ; Middle Aged ; Pregnancy ; Prevalence ; Retrospective Studies ; Risk Factors ; Venous Thrombosis/epidemiology/immunology