Antiphospholipid syndrome - Symptoms

Symptoms of antiphospholipid syndrome allow diagnosis of the disease based on the diagnostic criteria adopted at the 8th Congress on Antiphospholipid Antibodies in Sapporo (Japan) in 1998.

They include clinical and laboratory criteria.

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Clinical criteria for antiphospholipid syndrome

• Vascular thrombosis: one or more clinical episodes of venous, arterial thrombosis or thrombosis of small vessels of any localization, confirmed by Doppler data or histological examination, and in histological examination, thrombosis should not be accompanied by signs of inflammation of the vascular wall (vasculitis).
• Pathology of pregnancy:
  • one or more unexplained antenatal deaths of a morphologically normal fetus (confirmed by ultrasound or pathomorphological examination) after 10 weeks of pregnancy;
  • one or more premature births of a morphologically normal fetus before 34 weeks of pregnancy due to severe preeclampsia or severe placental insufficiency;
  • three or more unexplained spontaneous miscarriages before 10 weeks of pregnancy after excluding anatomical abnormalities, hormonal disorders in the mother, and chromosomal pathology in the parents.

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Laboratory criteria for antiphospholipid syndrome

• Detection of anticardiolipin antibodies of class G immunoglobulins (IgG) and/or class M immunoglobulins (IgM) in the blood in medium or high titers (at least a two-fold increase) at intervals of 6 weeks using a standardized enzyme immunoassay method for measuring (32-glycoprotein-dependent antibodies to cardiolipin (ACL).
• Determination of lupus anticoagulant in plasma (at least a two-fold increase) at intervals of at least 6 weeks using methods recommended by the International Society of Thrombosis and Hemostasis, including the following steps:
  • establishing the fact of prolongation of the phospholipid-dependent phase of plasma coagulation based on the results of screening tests such as activated partial thromboplastin time (APTT), kaolin time, Russell test with dilution, prothrombin time with dilution;
  • inability to correct for prolonged screening test times by mixing with normal platelet-free plasma;
  • shortening the screening test time or normalizing it after adding excess phospholipids to the plasma being tested and excluding other coagulopathies, such as the presence of a factor VIII inhibitor or heparin. The diagnosis of antiphospholipid syndrome is reliable if at least 1 clinical and 1 laboratory criterion are present.

Antiphospholipid syndrome can be suspected in the presence of autoimmune diseases, habitual miscarriage, early development of gestosis, especially its severe forms, placental insufficiency, fetal malnutrition during previous pregnancies, thrombocytopenia of unknown etiology, false-positive Wasserman reactions.

Other symptoms of antiphospholipid syndrome include:

• cutaneous manifestations (livedo reticularis, acrocyanosis, chronic leg ulcers, photosensitivity, discoid rash, capillary disease);
• neurological manifestations (migraine, chorea, epilepsy);
• endocarditis;
• serositis (pleurisy, pericarditis);
• aseptic necrosis of the femoral head;
• renal dysfunction (proteinuria, cylindruria);
• autoimmune thrombocytopenia (petechial rash).