Diagnosis of anti-phospholipid ab. syndrome (APS)
- Clinical Criteria
- Vascular thrombosis: arterial, venous, or small-vessel thrombosis in any tissue
- Pregnancy morbidity: Early (3) or late (1) spontaneous abortions OR pre-mature birth due to pre-eclampsia, placental insufficiency
- Laboratory Criteria
- Presence of lupus anticoagulant (LA) in plasma
- Elevated levels of anticardiolipin (IgG or IgM)
- Elevated levels of anti–beta-2 glycoprotein I antibodies (IgG or IgM)
- Each must be present on 2+ occasions at least 12 weeks apart
Posted 09/08/16 09:04:39 AM by Matthew Cummings
Thrombotic microangiopathies -- often difficult to distinguish
- TTP: difficult to distinguish from CAPS, must send ADAMTS13 prior to initiation of PLEX
- HUS: atypical HUS --> excessive activation of complement often 2/2 anti-factor H autoantibodies --> eculizumab
- CAPS: prior hx APS
- DIC: hypofibrinogenemia, prolonged INR
- HELLP: pregnancy, abnormal LFTs
- HIT: Type 2 --> typically thrombocytopenia 4-10 days after exposure to heparin products w/ vascular thrombosis, screen w/ PF4 abs.
Posted 09/08/16 09:06:42 AM by Matthew Cummings
- Rare, life-threatening form of APS -- widespread intravascular thrombosis resulting in multi-organ ischemia and failure
- Occurs in <1% patients with APS, but may be initial presentation in up to 50%
- Multiple small vessel occlusions, acute microangiopathy
- Myocardial injury/ischemia
- Multi-organ dysfunction syndrome
Posted 09/08/16 09:07:39 AM by Matthew Cummings
Catastrophic APS Diagnosis
- Evidence of clinical disorder of 3+ organ systems
- Development of manifestations within 1 week
- Laboratory confirmation of presence of aPL ab.
- Tissue confirmation of small-vessel occlusion in 1+ organ or tissue
Typically will initiate therapy based on clinical diagnosis (1-3), but often will push for tissue biopsy
Posted 09/08/16 09:12:04 AM by Matthew Cummings
Principles of APS Management
- Manage thrombotic events, suppress cytokine cascade
- IV heparin
- High-dose corticosteroids
- Plasma exchange +/- IVIG (remove or mitigate effect of anti-cardiolipin ab.)
- Identify and treat trigger (often infection)
- Refractory CAPS: emerging role for ritxumab, eculizimab
Posted 09/08/16 09:13:37 AM by Matthew Cummings
For further reading...
Posted 09/08/16 09:41:47 AM by Matthew Cummings
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