Francisco Chacón-Lozsán: Catastrophic Antiphospholipid Syndrome A Rapidly Progressive Multiorgan Thrombotic Emergency
Francisco Chacón-Lozsán, Fellow at World Extreme Medicine, Member of European Society of Intensive Care Medicine (ESICM) and American College of Cardiology, shared a post on LinkedIn about recent articles:
“Critical Physiology Series 93
Catastrophic Antiphospholipid Syndrome
A patient develops acute kidney injury, respiratory failure, neurological dysfunction and thrombocytopenia within days.
Sepsis? DIC? TTP?
Consider also catastrophic antiphospholipid syndrome (CAPS).
Pathophysiology
CAPS is a rare, life threatening manifestation of antiphospholipid syndrome characterized by rapidly progressive thrombosis, predominantly affecting the microcirculation.
Antiphospholipid antibodies, particularly those targeting β2 glycoprotein I, activate endothelial cells, monocytes and platelets.
Antibody binding lead to complement activation lead to endothelial dysfunction lead to tissue factor expression lead to thrombin generation
Neutrophil extracellular traps and inflammatory mediators amplify thrombosis.
The result:
microvascular occlusion lead to tissue ischemia lead to organ dysfunction
Unlike isolated venous thromboembolism, CAPS may simultaneously damage kidneys, lungs, brain, heart and other organs.
Risk factors and triggers
CAPS may develop in established APS or be its first manifestation.
Important associations include:
systemic lupus erythematosus, previous thrombosis and high risk antiphospholipid antibody profiles.
Common triggers:
infection, surgery, trauma, pregnancy complications and interruption of anticoagulation.
Diagnosis
Suspect CAPS when thrombosis and organ dysfunction develop rapidly across multiple systems.
Classical classification criteria include:
higher 3 organs involved
manifestations within approximately one week
small vessel thrombosis on histology
positive antiphospholipid antibodies
However, incomplete criteria do not exclude probable CAPS.
Request:
lupus anticoagulant
anticardiolipin IgG or IgM
anti-β2-glycoprotein I IgG or IgM
Interpret testing cautiously during acute illness and anticoagulation.
Differentiate CAPS from DIC, TTP or HUS, HIT and sepsis associated microangiopathy.
Monitoring
Follow:
platelets and hemolysis markers
PT aPTT, fibrinogen
creatinine, urine output
lactate and organ perfusion
ECG, troponin, echocardiography
neurological and respiratory function
Management
CAPS requires urgent multidisciplinary treatment, often before complete diagnostic confirmation.
The conventional approach combines:
Therapeutic heparin lead to suppress ongoing thrombosis
High dose corticosteroids lead to suppress inflammation
Plasma exchange and or IVIG lead to modulate pathogenic immune activity
Treat the precipitating infection or other trigger.
In SLE associated CAPS, cyclophosphamide may be indicated.
Refractory cases may require rituximab or complement inhibition with eculizumab in selected circumstances.
Evidence remains largely observational because randomized trials are lacking.
Recommended lectures.”
Title 1: Diagnosis and Management of Catastrophic Antiphospholipid Syndrome and the Potential Impact of the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria
Authors: Lucas Jacobs, Nader Wauters, Yahya Lablad, Johann Morelle, Maxime Taghavi
Read Full Article on MDPI.

Title 2: Antiphospholipid syndrome pathogenesis in 2023: an update of new mechanisms or just a reconsideration of the old ones?
Authors: Elena Raschi, Maria Orietta Borghi, Francesco Tedesco, Pier Luigi Meroni
Read Full Article on Rheumatology.

Title 3: McMaster RARE‐Bestpractices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome
Authors: K. Legault, H. Schunemann, C. Hillis, C. Yeung, E.A. Akl, M. Carrier, R. Cervera, M. Crowther, F. Dentali, D. Erkan, G. Espinosa, M. Khamashta, J.J. Meerpohl, K. Moffat, S. O’Brien, V. Pengo, J.H. Rand, I. Rodriguez Pinto, L. Thom, A. Iorio
Read Full Article on Ovid.

Title 4: Treatment of catastrophic antiphospholipid syndrome
Authors: Nayef M. Kazzaz, W. Joseph McCune, Jason S. Knight
Read Full Article on JTH.

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