Diffuse

Catastrophic Anti-Phospholipid Antibody Syndrome

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Catastrophic Anti-Phospholipid Antibody Syndrome

  • See Also
  1. Antiphospholipid Antibody Syndrome
  2. Systemic Lupus Erythematosus
  • Epidemiology
  1. Incidence: <1% of patients with Antiphospholipid Antibody Syndrome
  2. Mortality approaches 50%
  • Pathophysiology
  1. Multisystem thrombosis (cardiopulmonary, splenic) affecting small to medium sized vessels
  2. Complication of Antiphospholipid Antibody Syndrome
  • Risk Factors
  • Triggers
  1. Infection (most common)
  2. Injury or surgery
  3. Malignancy (esp. Lymphoma, ALL, Lung Adenocarcinoma, colorectal adenocarcinoma)
  4. Discontinuation of Anticoagulation
  5. Pregnancy (e.g. HELLP Syndrome, Pelvic Vein Thrombophlebitis)
  6. Oral Contraceptives
  • Findings
  • Presentations
  1. Respiratory
    1. Adult Respiratory Distress Syndrome (ARDS)
    2. Pulmonary Embolism
  2. Cardiac
    1. Myocardial Infarction
    2. Congestive Heart Failure
  3. Gastrointestinal
    1. Bowel infarction
  4. Neurologic
    1. Encephalopathy
    2. Large vessel CVA
    3. Seizure
  5. Renal
    1. Acute Kidney Injury
  6. Dermatologic
    1. Livedo Reticularis
    2. Gangrene of fingers or toes
  • Labs
  1. See Antiphospholipid Antibody Syndrome for APLA related tests
  2. Complete Blood Count
    1. Thrombocytopenia
    2. Microangiopathic Hemolytic Anemia
    3. Disseminated Intravascular Coagulation
  • Diagnosis (All 4 Criteria Required)
  1. Three or more organs or systems involved
  2. Thrombotic events occur within 1 weeks of each other
  3. Small vessel Occlusion identified on histopathology (for at least one organ)
  4. Antiphospholipid Antibody Positive (APLA) at adequate titers
  • Differential Diagnosis
  1. Thrombotic Thrombocytopenic Purpura
  2. Hemolytic Uremic Syndrome
  3. Heparin Induced Thrombocytopenia
  4. Septic Shock
  5. Endocarditis
  6. Hypertensive Emergency
  7. Metastatic cancer
  • Management
  1. Anticoagulation (Heparin)
  2. Systemic Corticosteroids
  3. IV Immunoglobulins
  4. Plasmapheresis
  5. Biologic Agents (e.g. eculizumab, Rituximab)
  • Resources
  1. Internet Book of Critical Care (IBCC, Farkas)
    1. https://emcrit.org/ibcc/caps/
  • References
  1. Bustamante (2023) Antiphospholipid Syndrome, StatPearls, Treasure Island, FL [PubMed]
    1. https://www.ncbi.nlm.nih.gov/books/NBK430980/