II. Epidemiology
- Incidence: <1% of patients with Antiphospholipid Antibody Syndrome
- Mortality approaches 50%
III. Pathophysiology
- Multisystem thrombosis (cardiopulmonary, splenic) affecting small to medium sized vessels
- Complication of Antiphospholipid Antibody Syndrome
IV. Risk Factors: Triggers
- Infection (most common)
- Injury or surgery
- Malignancy (esp. Lymphoma, ALL, Lung Adenocarcinoma, colorectal adenocarcinoma)
- Discontinuation of Anticoagulation
- Pregnancy (e.g. HELLP Syndrome, Pelvic Vein Thrombophlebitis)
- Oral Contraceptives
V. Findings: Presentations
- Respiratory
- Cardiac
- Gastrointestinal
- Bowel infarction
- Neurologic
- Encephalopathy
- Large vessel CVA
- Seizure
- Renal
- Dermatologic
- Livedo Reticularis
- Gangrene of fingers or toes
VI. Labs
VII. Diagnosis (All 4 Criteria Required)
- Three or more organs or systems involved
- Thrombotic events occur within 1 weeks of each other
- Small vessel Occlusion identified on histopathology (for at least one organ)
- Antiphospholipid Antibody Positive (APLA) at adequate titers
VIII. Differential Diagnosis
- Thrombotic Thrombocytopenic Purpura
- Hemolytic Uremic Syndrome
- Heparin Induced Thrombocytopenia
- Septic Shock
- Endocarditis
- Hypertensive Emergency
- Metastatic cancer
IX. Management
- Anticoagulation (Heparin)
- Systemic Corticosteroids
- IV Immunoglobulins
- Plasmapheresis
- Biologic Agents (e.g. eculizumab, Rituximab)
X. Resources
- Internet Book of Critical Care (IBCC, Farkas)