ABSTRACT
AIM: To study the clinical and immunological features of primary antiphospholipid syndrome (APS), and to analyze the differences between primary APS and APS associated with autoimmune rheumatic disease (ARD/APS). METHODS: This prospective, longitudinal study, carried out from December 2004 to July 2011 included 179 patients with primary APS and 52 patients of ARD/APS diagnosed as per modified 2006 Sapporo's Criteria. RESULTS: Out of 179 patients of primary APS, 12 were male and 167 were female. The mean age at the time of study entry was 27 ± 4.33 years. Venous thrombosis was noted in 33 (18.43%) patients. Seventeen patients had deep vein thrombosis and 11 (7.19%) had cortical vein and/or cortical sinus thrombosis. Arterial thrombosis was noted in 19 (10.61%) patients, out of which nine had intracranial arterial thrombosis. Thirty-two (17.85%) had recurrent early fetal losses (< 10 weeks) and 97 (54.18%) had late fetal loss (> 10 weeks). Immunoglobulin G (IgG) and IgM aCLA were present in 141 (78.77%) and 32 (17.87%) patients respectively, whereas lupus anticoagulant was present in 99 (55.3%) patients. In patients with bad obstetric outcome, lupus anticoagulant positivity was significantly more prevalent (P < 0.05) than aCLA positivity. Both venous and arterial thrombosis were significantly more common (P < 0.05) in ARD/APS. However, late fetal loss was significantly more prevalent (P < 0.001) in primary APS. CONCLUSION: Primary APS may lead to a variety of clinical manifestations due to venous and/or arterial thrombosis, which at times may be lethal. It is also an important cause of early and late pregnancy loss(es) and other pregnancy morbidities.
Subject(s)
Antiphospholipid Syndrome/immunology , Autoantibodies/blood , Autoimmunity , Hospitals, University , Rheumatic Diseases/immunology , Abortion, Spontaneous/epidemiology , Abortion, Spontaneous/immunology , Adolescent , Adult , Antiphospholipid Syndrome/blood , Antiphospholipid Syndrome/diagnosis , Antiphospholipid Syndrome/epidemiology , Arterial Occlusive Diseases/epidemiology , Arterial Occlusive Diseases/immunology , Biomarkers/blood , Child , Female , Humans , India/epidemiology , Longitudinal Studies , Male , Middle Aged , Predictive Value of Tests , Pregnancy , Prevalence , Prognosis , Prospective Studies , Rheumatic Diseases/blood , Rheumatic Diseases/diagnosis , Rheumatic Diseases/epidemiology , Risk Factors , Time Factors , Venous Thrombosis/epidemiology , Venous Thrombosis/immunology , Young AdultABSTRACT
The aim of this study is to explore the role of plasminogen activator inhibitor type 1 (PAI-1) in primary and secondary antiphospholipid syndrome (APS). Thirty patients of APS (24 primary and 6 secondary) were recruited in the study who fulfilled the revised Sapporo criteria. Control groups comprised of age- and sex-matched 10 healthy volunteers and 10 patients each of systemic lupus erythematosus and rheumatoid arthritis without any antecedent thrombotic event and/or APS-related pregnancy morbidity. Serum samples were tested for PAI-1 antigen levels measured by quantitative ELISA. Positivity rate of PAI-1 in patients of primary, secondary as well as total APS patients was significantly higher in relation to age- and sex-matched healthy volunteers (p = 0.010, p = 0.003 and p < 0.001, respectively). Mean ± SEM levels of PAI-1 in primary and secondary as well as total APS patients were significantly higher (p = 0.006, p < 0.001 and p < 0.001) in relation to healthy controls. Correlation of PAI-1 levels (mean ± SEM) with clinical characteristics, that is, thrombosis and pregnancy morbidity, revealed significantly higher levels of PAI-1 (p < 0.001) in patients having thrombosis and APS-related pregnancy morbidity. Elevated PAI-1 level leading to impaired fibrinolysis plays a significant role in producing hypercoagulable state in primary and secondary APS.