Article ; Online: Antiphospholipid Patients Admitted in the Intensive Care Unit: What Must The Rheumatologist Know?
2024
Abstract: Purpose of the review: Antiphospholipid syndrome (APS) is a rare systemic autoimmune disorder that can escalate into a 'thrombotic storm' called the catastrophic antiphospholipid syndrome (CAPS), frequently requiring ICU admission for multiple organ ... ...
Abstract | Purpose of the review: Antiphospholipid syndrome (APS) is a rare systemic autoimmune disorder that can escalate into a 'thrombotic storm' called the catastrophic antiphospholipid syndrome (CAPS), frequently requiring ICU admission for multiple organ failure. This review aims to offer insight and recent evidence on critically-ill APS patients. Recent findings: The CAPS classification criteria define this condition as the involvement of at least three organs/systems/tissues within less than a week, caused by small vessel thrombosis, in patients with elevated antiphospholipid antibodies levels. These criteria do not encompass the full spectrum of critically-ill thrombotic APS patients and they need to be cautiously used for the bedside diagnosis of CAPS. Thrombocytopenia is the laboratory hallmark of CAPS, sometimes dropping below 20G/L, but a complete thrombotic microangiopathy pattern is infrequent. Anticoagulation is the pivotal treatment for APS and CAPS, associated with improved outcome. Triple therapy - the combination of anticoagulation, high-dose corticosteroids, and either plasma exchange or intravenous immunoglobulins - remains the standard treatment for CAPS patients. Eculizumab, an anti-C5 monoclonal antibody, may be useful in refractory patients. Despite significant progress, CAPS mortality rate remains high. Its diagnosis and management are complex, requiring a close multidisciplinary cross talk between APS specialists and intensivists. |
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Language | English |
Publishing date | 2024-04-23 |
Publishing country | United States |
Document type | Journal Article ; Review |
ZDB-ID | 2057357-1 |
ISSN | 1534-6307 ; 1523-3774 |
ISSN (online) | 1534-6307 |
ISSN | 1523-3774 |
DOI | 10.1007/s11926-024-01148-7 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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