2023
DOI: 10.3390/immuno4010001
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Catastrophic Antiphospholipid Syndrome: A Review

Carmine Siniscalchi,
Manuela Basaglia,
Michele Riva
et al.

Abstract: Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombotic or obstetric events occurring in individuals who have persistent antiphospholipid antibodies. Catastrophic antiphospholipid syndrome (CAPS) is a rare and potentially fatal form of APS characterized by severe thrombotic complications occurring in multiple organs over a short period of time or simultaneously. CAPS is associated with a high (50%) death rate. Infections, multi-organ failure, and cerebral and heart thrombos… Show more

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Cited by 6 publications
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“…A severe dysregulation of complement activity has been described in CAPS, and treatment with eculizumab has been associated with a reduced risk of death in the acute phase of disease and a reduced VTE recurrence in the follow-up [120]. Eculizumab seems more effective in subjects with CAPS showing thrombocytopenia with TMA and in those who are refractory to first-line treatments, represented by anticoagulation, corticosteroids, intravenous immunoglobulins, or plasma exchange [120,121].…”
Section: Inhibition Of Complement Activationmentioning
confidence: 99%
“…A severe dysregulation of complement activity has been described in CAPS, and treatment with eculizumab has been associated with a reduced risk of death in the acute phase of disease and a reduced VTE recurrence in the follow-up [120]. Eculizumab seems more effective in subjects with CAPS showing thrombocytopenia with TMA and in those who are refractory to first-line treatments, represented by anticoagulation, corticosteroids, intravenous immunoglobulins, or plasma exchange [120,121].…”
Section: Inhibition Of Complement Activationmentioning
confidence: 99%