engleski

Antiphospholipid syndrome - case report

Introduction: The antiphospholipid syndrome (APS) is defined by the persistent presence of antiphospholipid antibodies (aPLS) with a variety of clinical phenotypes, including venous or arterial thrombosis, recurrent pregnancy loss and thrombocytopenia. It can be a primary disease or secondary when associated with other autoimmune diseases. Laboratory diagnosis of aPLS is based on the detection of lupus anticoagulant (aLA), and/or anticardiolipin (aCL) and anti-ß2-glycoprotein-1 (aß2G-1) antibodies. Pathophysiologic mechanisms in APS include aPLS induced cellular activation (predominantly with aß2G-1), inhibition of natural anticoagulant and fibrinolitic systems, and complement activation. Subjects and methods: The aim of the study was to review the clinical manifestations of the APS and the antibody titers of aPLS in a 45-year-old women with diagnosed primary APS at the age of 30, according to classification criteria of International Consensus for diagnosis and therapeutic strategies in patients affected by APS. The patient was diagnosed with secondary APS only 8 years after the onset of the disease. Results: Through this case report, we describe the evolution of its complex pathology over a 15-year period of follow-up: obstetric complications - spontaneus abortion, psoriasis, psoriatic arthritis, cerebral demyelinization, APL nephropathy - trombotic microangiopathy, hypertension, hereditary thrombophilia, colagenosis mixta, anaemia, thrombocytopenia, hemiparesis. We had triple positive antibodies: aLA was always positive, aCL and especially aß2G-1 antibodies concentrations were highly elevated. Concentrations of C3 and C4 component of complement were permanently reduced, titers of antinuclear antibodies (ANA) were negative. Biochemia Medica 2018 ; 28(Suppl 1):S1–S223 S55 Conclusion: We conclude that high values of aPLS in APS, in particular anti-ß2-glycoprotein-1 antibodies, can be the cause of numerous pathological clinical manifestations. Long-term monitoring is important for identifying laboratory parameters that may portend the development of further autoimmune symptoms associated with APS.

antiphospholipid syndrome, antiphospholipid antibody

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