Antiphospholipid Patients Admitted in the Intensive Care Unit: What Must The Rheumatologist Know?
Curr Rheumatol Rep
; 26(7): 269-277, 2024 Jul.
Article
in En
| MEDLINE
| ID: mdl-38652403
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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Antiphospholipid Syndrome
/
Intensive Care Units
Limits:
Humans
Language:
En
Journal:
Curr Rheumatol Rep
Journal subject:
REUMATOLOGIA
Year:
2024
Document type:
Article
Affiliation country:
Francia