Syndrome antiphospholipid

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Syndrome antiphospholipid

Antiphospholipid syndrome (APS) is a symptom complex that occurs against the background of hyperproduction of AT to phospholipids (lupus anticoagulant, AT to cardiolipin), characterized by thrombosis of various localization. It develops in rheumatic diseases (most often – SLE). Frequency – 7% in the general population, 30-60% of patients with SLE. The predominant age is 20-40 years. The predominant gender is female.

Clinical picture

    • Recurrent thrombosis
    • Deep vein thrombophlebitis
    • Cerebrovascular disorders (Sneddon’s syndrome) caused by cerebral thrombosis
    • Acute or transient cerebrovascular accident
    • Transverse myelitis (not common)
    • convulsive syndrome
    • Chorea
    • Migraine
    • Reticulate livedo, a tree-like pattern on the skin of the lower extremities associated with thrombosis of small skin vessels
    • Pulmonary hypertension
    • Budd-Ksh-ri syndrome
    • THEM
    • Reith Syndrome.
    • Obstetric pathology due to thrombosis of placental vessels
    • Recurrent spontaneous abortions
    • Intrauterine fetal death
    • Eclampsia.
    • Hematological disorders
    • Hemolytic anemia
    • thrombocytopenia.
    • Thrombotic non-infectious endocarditis (often with damage to the mitral valve).
    • Aseptic necrosis of the femoral heads (probably associated with thrombosis of the femoral artery).

Laboratory research

    • The presence in the blood plasma of AT to cardiolipin
    • Detection in the blood of lupus anticoagulant (AT, blocking in vitro phospholipid-dependent coagulation factors)
    • False positive von Wassermann reaction
    • Presence of antinuclear factor (50% of cases)
    • Cryoglobulinemia (not common)
    • RF detection (not common)
    • Thrombocytopenia (50% of cases)
    • Prolongation of blood clotting time.

Special Studies

    • X-ray examination
    • Angiography reveals signs of parietal thrombosis
    • Examination of the joints does not reveal any pathology
    • Echocardiography – thickening of the valve leaflets (often mitral), vegetation. Diagnostic criteria (Alarcon-Segovia D. et a/., 1992)
    • Recurrent spontaneous abortions
    • Venous thrombosis
    • Arterial thrombosis
    • Ulcers on the skin of the legs
    • Mesh livedo
    • Hemolytic anemia
    • Thrombocytopenia
    • AT to cardiolipin>5 S. Note. Diagnosis is carried out taking into account the presence of the clinical manifestations listed above, in combination with the quantitative determination of AT to cardiolipin
    • Definite API: 2 or more clinical criteria
    • AT to cardiolipin >5 S
    • Probable APS: one clinical criterion
    • AT to cardiolipin >5 S
    • Doubtful APS: no clinical manifestations
    • AT to cardiolipin >5 S or one clinical criterion
    • AT to cardiolipin in the range from 3 S to 5 S.

Tactics of conducting

    • Pathogenetic treatment of the underlying disease
    • Avoid oral contraceptives, smoking
    • As basic therapy – antiplatelet agents: aspirin (acetylsalicylic acid) 320 mg / day, dipyridamole 150-200 mg / day
    • Plasmapheresis
    • Immunosuppressive therapy is not always effective
    • Chloroquine 0.25 g/day, Plaquenil (hydroxychloroquine) 0.2 g/day
    • Glucocorticoids 15-20 mg/day.

Reduction. APS – antiphospholipid syndrome KSD. D89.9 Disorder involving immune mechanism, unspecified S is the sedimentation coefficient