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

OBJECTIVE: To explore the predictive value of four items of new thrombus markers combined with conventional coagulation tests for thrombosis in antiphospholipid syndrome. METHODS: A total of 121 antiphospholipid syndrome (APS) patients who hospitalized at Peking University People's Hospital from March 2022 to January 2023 were selected and divided into thrombus group (50 cases) and nonthrombus group (71 cases) according to whether thrombosis occurred. The differences of laboratory characteristics including antiphospholipid antibodies were compared between the thrombotic and non-thrombotic groups. Chemiluminescent immunoassay was used to detect thrombomodulin (TM), thrombin-antithrombin complex (TAT), Plasmin-α2 plasmin inhibitor complex (PIC), and tissue plasminogen activator inhibitor complex (t-PAIC) in plasma from venous. The independent risk factors of thrombosis in patients with APS were determined using binary Logistic regression. Receiver operating characteristic (ROC) curve analysis was applied to evaluate the efficacy of each index on the prediction of thrombosis. RESULTS: Compared with the patients without thrombosis, the patients with thrombosis were older [49 (32, 64) years vs. 36 (32, 39) years, P < 0.05]. The percentages of male, smoking, hypertension, and global antiphospholipid syndrome score (GAPSS)≥10 in the patients with thrombosis were significantly higher than those in the patients without thrombosis (P < 0.05). The positive rates of anticardiolipin antibody (aCL) and lupus anticoagulant (LA) in the thrombotic group were significantly higher than those in the non-thrombotic group (P < 0.05), and the levels of prothrombin time, activated partial thromboplastin time, fibrinogen, fibrin degradation product in the thrombotic group were significantly higher than those in the non-thrombotic group (P < 0.05).Among the thrombosis group, venous thrombosis accounted for 19 (38.00%), including deep vein thrombosis (16, 84.21%) and pulmonary embolism accounted (5, 26.32%); Arterial thrombosis accounted for 35 (70.00%), including myocardial infarction (6, 17.14%) cerebral infarction (30, 85.71%). The patients in the thrombotic group had significantly greater TM levels than those in the non-thrombotic group (P < 0.05).There were no significant dif-ferences between the two groups in TAT (Z=-1.420, P=0.156), PIC (Z=-0.064, P=0.949), and t-PAIC (Z=-1.487, P=0.137). Univariate and binary Logistic regression analysis of relevant variables showed that advanced age [OR=1.126, P=0.002], elevated TM [OR=1.325, P=0.048], prolonged prothrombin time (PT) [OR=4.127, P=0.008] were independent risk factors for thrombosis in the patients with APS. ROC curve analysis of the above three independent risk factors showed that the combined detection of age, PT and TM had the highest Yoden index (0.727) and sensitivity (83.0%), with a specificity of 89.7%. CONCLUSION TAT, PIC, TM, and t-PAIC may reflect thrombus formation from the coagulation system, fibrinolysis system, and endothelial system. The combined of age TM and PT is superior to the application of a single marker, which has diagnostic value for the early identification of APS thrombosis.
Humans ; Male ; Antiphospholipid Syndrome/diagnosis* ; Tissue Plasminogen Activator ; Thrombosis/etiology* ; Antibodies, Antiphospholipid/analysis* ; Blood Coagulation Tests/adverse effects*

OBJECTIVE: To detect the serum levels of soluble endothelial glycoprotein endoglin (s-Eng) in patients with antiphospholipid syndrome (APS) and to evaluate the correlation between s-Eng levels and clinical features and laboratory parameters. METHODS: The levels of serum s-Eng were measured by enzyme linked immunosorbent assay (ELISA) in 139 patients with APS, 44 patients with SLE but no APS, 37 patients with primary Sjögren's syndrome (pSS), 23 patients with Bechet's disease (BD), 22 patients with systemic sclerosis (SSc) and 22 persistent anticardiolipin antibody (aCL) positive individuals without SLE or APS (simply aCL positive group) and 87 health controls (HC) without any auto-immune diseases. These APS patients included 64 primary APS patients and 75 APS patients secondary to SLE.The correlation between the clinical data, laboratory parameters, and serum s-Eng levels were analyzed.Independent samples t test, paired t test, Chi-square Test, Mann-Whitney U test, Pearson's χ² test were used for statistical analyses. RESULTS: (1) The serum levels of s-Eng were significantly higher in the patients with APS whether primary or secondary to SLE than in the health controls and simply aCL positive group and the patients with other autoimmune diseases, including SLE, pSS, BD and SSc (P<0.001). There was no significant difference in the serum s-Eng levels between simply aCL positive group and health controls [(5.17±2.00) mg/L vs. (5.04±1.11) mg/L, P>0.05]. (2) The best cut-off value for the diagnosis of APS was no less than 8.37 mg/L as mean ± 3SD value, with the sensitivity at 0.772 and the specificity at 0.928. The Youden index was 0.700. These results indicated good validity of s-Eng as a diagnostic marker for APS. (3) The proportions of artery thrombosis and pathological pregnancy were higher in the group of s-Eng-positive APS patients than that in s-Eng-negative group (46/81 vs. 19/58, 29/65 vs. 10/44, respectively, all P<0.05). The levels of PLT were lower in the group of s-Eng-positive APS patients (72.00×10⁹/L vs. 119.00×10⁹/L, P<0.001). (4) The proportions of the presence (93.83% vs. 37.93%, P<0.001) and titer (61.70 U/mL vs. 15.45 U/mL, P<0.001) of aCL were both higher in the group of s-Eng-positive APS patients than in s-Eng-negative group. The proportions of the presence (61.73% vs. 43.10%, P<0.05) and titer (33.48 U/mL vs.17.40 U/mL, P<0.05) of anti-β2-glycoprotein I antibody were both higher in the group of s-Eng-positive APS patients than in s-Eng-negative group too. CONCLUSION s-Eng serum levels were significantly increased in the patients with APS, and it may play a role as acomplementary serological marker for the diagnosis and risk prediction of APS.
Antibodies, Anticardiolipin ; Antiphospholipid Syndrome/diagnosis* ; Autoantibodies ; Endoglin/blood* ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Pregnancy

Abortion, Habitual ; complications ; Antibodies, Antiphospholipid ; blood ; Antiphospholipid Syndrome ; complications ; pathology ; therapy ; Erythema ; complications ; Heart Valve Diseases ; complications ; Humans ; Kidney Diseases ; complications ; Skin Diseases ; complications ; Thrombosis ; complications

The antiphospholipid antibody syndrome is cha- racterized by antibodies directed against either phos-pholipids or plasma proteins bound to anionic phos- pholipids. These antibodies have been characterized by lupus anticoagulants and anticardiolipin antibodies. Patients with the antiphospholipid antibody syndrome may display a constellation of clinical features including venous and arterial thrombosis, recurrent fetal losses, and thrombocytopenia. Although the majority of patients reported have a thrombotic microangiopathy, some have also seen membranous nephopathy as well as IgA nephropathy in a patient with anti-phospholipid antibody syndrome accompanying glo-merulonephritis. Authors experienced a 37-year-old male patient who presented with generalized edema at the moment of follow-up for primary antiphos-pholipid syndrome accampanying systemic thrombotic events. Anticardiolipin antibody-IgM positivity was detected by seroligic test but no evidence for systemic lupus erythematosus was found. Kidney biopsy showed mesangial IgA deposition without th throm-botic microangiopathy of gomerular capillaries and was diagnosed finally as primary antiphospholipid syndrome with IgA nephropathy. Patient's symptom was relieved with steroids and anti platelete agents and now he is being follow-up to out patient department. This case suggests some possibility that anticardiolipin antibody may induce the IgA nephropathy. Therefore clinician should have concern about the relationship between antiphospholipid antibody and immune mediate glomerulonephritis.
Adult ; Antibodies ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid* ; Anticoagulants ; Antiphospholipid Syndrome* ; Biopsy ; Blood Platelets ; Blood Proteins ; Capillaries ; Edema ; Follow-Up Studies ; Glomerulonephritis ; Glomerulonephritis, IGA ; Humans ; Immunoglobulin A ; Kidney ; Lupus Erythematosus, Systemic ; Male ; Steroids ; Thrombocytopenia ; Thrombosis ; Thrombotic Microangiopathies

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

Antiphospholipid antibody syndrome (APS) is defined as the presence of lupus anticoagulant antibody or anticardiolipin antibody with vascular thrombosis or pregnancy complications. APS can be associated with autoimmune disease or infectious disease. APS has also been reported in conjunction with variety of solid and hematologic malignancies. There were some reports on APS which were accompanied by hematologic malignancy, but there was no report with solid malignancy in Korea. We experienced one case of secondary APS, which was diagnosed during pre-operative evaluation of thyroid cancer. This patient had prolonged aPTT (activate partial thromboplastin time) and decreased coagulation factors which were regarded as hemophilia at first. Although the precise mechanism of the relationship between APS and cancer has not been proven thoroughly, APS can be accompanied by various malignancies. So proper screening and early detection of malignancies in APS patients are recommended.
Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome ; Autoimmune Diseases ; Blood Coagulation Factors ; Communicable Diseases ; Hematologic Neoplasms ; Hemophilia A ; Humans ; Korea ; Lupus Coagulation Inhibitor ; Mass Screening ; Pregnancy Complications ; Thromboplastin ; Thrombosis ; Thyroid Gland ; Thyroid Neoplasms

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

PURPOSE: We performed this study to evaluate the incidence and clinical significance of antiphospholipid antibodies (aPL Ab) in Korean children with Henoch-Schonlein purpura (HSP). METHODS: The medical records of 62 patients (31 boys and 31 girls) aged 6.0+/-3.1 (1-16) years with a clinical diagnosis of HSP based on the EULAR/PReS criteria were reviewed retrospectively. From the years 2007 to 2009, the sera from children with acute HSP were tested for aPL Ab such as LA, anti-cardiolipin antibody and anti-beta2 glycoprotein I antibody. RESULTS: LA was positive in 18 (29%) of the 62 patients with HSP and We divided the patients into the two groups LA positive group (N=18) and LA negative group (N=44). There were no significant differences between the two groups with regard to abdominal pain, arthralgia and renal involvement, but LA positive group had significantly higher C-reactive protein (4.3+/-7.2 mg/dL vs. 1.3+/-1.8 mg/dL, P=0.035), erythrocyte sedimentation rate (37.5+/-26.2 mm/hr vs. 25.1+/-22.6 mm/hr, P= 0.039), IgM (148.1+/-48.4 mg/dL vs. 114.9+/-41.5 mg/dL, P=0.024), C3 (143.1+/-21.9 mg/dL vs. 129.7+/-24.5 mg/dL, P=0.048) and C4 levels (30.9+/-6.3 mg/dL vs. 24.9+/-7.8 mg/dL, P=0.002) compared with LA negative group. CONCLUSION: We found that the incidence of positive aPL Ab tests was relatively higher in Korean children with HSP and the presence of aPL Ab was associated with acute inflammatory process of HSP. These results suggest that the aPL Ab are involved in the pathogenesis of HSP in children.
Abdominal Pain ; Aged ; Antibodies, Antiphospholipid ; Arthralgia ; Blood Sedimentation ; C-Reactive Protein ; Child ; Glycoproteins ; Humans ; Immunoglobulin M ; Incidence ; Lupus Coagulation Inhibitor ; Medical Records ; Purpura, Schoenlein-Henoch ; Retrospective Studies

Antiphospholipid syndrome is a disease that has continuous high titer of antibodies directed against either phospholipids or plasma proteins bound to anionic phospholipids in serum and shows a variety of clinical manifestations including recurrent venous and arterial thrombosis, recurrent fetal losses, livedo reticularis and thrombocytopenia. Because thrombosis may develop in any vessel, clinical manifestations are variable. Renal microangiopathy has been reported in antiphospholipid syndrome associated with systemic lupus erythematosus and rarely reported in primary antiophospholipid syndrome. But there was no case report of antiphospholipid syndrome accompanied by renal microangiopathy in Korea. Recently, we experienced a 25 years old male patient who had primary antiphospholipid syndrome with intrarenal thrombotic microangiopathy and IgA nephropathy. So, we report this case with review of relevant literature.
Adult ; Antibodies ; Antiphospholipid Syndrome* ; Blood Proteins ; Glomerulonephritis, IGA ; Humans ; Korea ; Livedo Reticularis ; Lupus Erythematosus, Systemic ; Male ; Phospholipids ; Thrombocytopenia ; Thrombosis ; Thrombotic Microangiopathies