CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME: MODERN VIEWS ON PATHOGENESIS, CLINICS, DIAGNOSIS, TREATMENT
Delva Yu.V., Drogomeretska O.I., Fedorovych H.M., Yatsyshyn R.I.
Summary. Catastrophic antiphospholipid syndrome (CAPS) is the most severe, rapidly progressive and aggressive clinical form of antiphospholipid syndrome (APS), which is accompanied by numerous thromboembolisms and is characterized by generalized visceral lesions, the development of multiple organ failure, and high mortality. The purpose of the study: using the materials of the publications, to summarize information about the origin, mechanisms of development, clinical manifestations, methods of diagnosis, prognosis, recommendations for the treatment of CAFS. The results. APS is a systemic autoimmune disease, most often manifested by thrombosis or obstetric problems, observed in individuals with persistence of antiphospholipid antibodies (APL). CAFS develops infrequently, but is an extremely life-threatening pathology. Mortality in persons with CAFS reaches 50%. The cause of death in such patients are infections, multiple organ failure, strokes, heart attacks. For the treatment of patients with CAFS, a complex of drugs including anticoagulants, corticosteroids, intravenous immunoglobulin (IVIG), and plasmapheresis sessions are recommended. Biological agents, such as anti-TNF-α monoclonal antibodies (adalimumab, certolizumab), anti-CD38 monoclonal antibodies (daratumumab), BAFF/Blys inhibitors and BTK inhibitors (belimumab and zanubrutinib), have been proven to be therapeutically effective in CAFS. Conclusions. CAFS is characterized by the rapid onset of thrombosis in many blood vessels at once, which leads to multiple organ failure and a high risk of mortality. Such a complication is considered a systemic thrombosed state. CAFS should be distinguished from other systemic thrombotic disorders such as thrombotic thrombocytopenic purpura, hemolytic uremic syndromes, and heparin-induced thrombocytopenia. It is necessary to develop additional potential therapeutic strategies aimed at components of the innate immune system, in particular, the complement system, the possibilities of immunomodulatory therapy.
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