6.9 Antiphospholipid Syndrome Flashcards
1
Q
Antiphospholipid Syndrome
- Definition / pathogenesis
- Clinical Manifestations
- Venous
- Arterial
- Neurologic
- Pulmonary
- Cardiac
- Hematologic
- GI
- Adrenal
- Skin
- Bone
- Eyes
A
- Definition / pathogenesis
- Systemic autoimmune d/o
- Results in venous/arterial thrombosis and/or pregnancy morbidity
- Signaled by presence of APS antibodies
- Directed against phospholipid binding proteins (cardiolpin and B2-glycoprotein) and lupus anticoagulant
- Antibodies cause cellular and vascular damage –> blood clots
- Can occur as primary or secondary dz
- SLE
- Clinical Manifestations
- Venous clots
- DVT, LE > UE
- Pelvic, renal, pulm, hepatic/portal, axillar/subclav, ocular, cerebral sinuses, IVC
- Arterial clots
- Cerebral vasculature > stroke/TIA
- Coronary, retinal, renal, mesenteric
- Neurologic
- Antiphospholipid antibodies directly attack neuronal tissue
- Consider in young stroke pt without ovious risk factors
- Can cause cognitive deficits, a/w white matter lesions on MRI (vasculopathy)
- Pulmonary
- PE
- P HTN
- ARDS
- Diffuse alveolar hemorrhage
- Cardiac
- CAD
- Valve dz
- Mitral > aortic
- Valvular thickening and nodules –> regurgitant valves
- Hematologic
- Thrombocytopenia (thrombotic events still happen)
- Hemolytic anemia
- Thrombotic microangiopathies
- Bone marrow necrosis
- GI
- Ischemia to esophagus, stomach, small bowel, large bowel
- Bleeding, abd pain, gastric/duodenal ulcers, splenic/panc infarc
- Hepatic/portal vein thrombosis –> cirrhosis, portal HTN, Budd Chiari
- Adrenal
- Adrenal insufficiency d/t hemorrhagic infarct
- Skin
- Livedo reticularis: red/purple lacey reticular patterned rash
- Cutaneous ulcers
- Bone
- Osteonecrosis, femoral heads
- Eyes
- Ocular venous/arterial occlusion
- Retinal / ischemic optic neuropathy
- Venous clots
2
Q
Your pregnant patient wants to know about signs of Antiphospholipid Syndrome. You explain you’d suspect APS if:
A
- 3 or more consecutive spontaneous abortions at < 10wks gestation
- Not otherwise explained by fetal chromosomal abnormality or maternal anatomy/hormonal cause
- One or ore unexplained abortion of morphologically normal fetus at > 10wks
- One or more premature births < 34wks of morphologically normal neonate
- D/t eclampsia, preeclampsia, placental insufficiency
3
Q
A patient is determined to have APS if they meet what Sapporo Classification Criteria?
A
- At least one clinical criteria of:
- Vascular thrombosis, or
- Pregnancy morbidity
- AND at least antiphospholipid antibody on 2 or more occasions at least 12 wks apart of:
- IgG and/or IgM anticardiolipin antibodies
- IgG and/or IgM anti-B2 glycoprotein I
- Lupus anticoagulant activity
4
Q
Describe treatment for antiphospholipid syndrome
A
- Positive aPL tests with NO evidence clinically of APS?
- Treatment NOT recommended
- First line treatment of clinical APS
- AC with heparin vs enoxaparin
- Bridge to warfarin
- DOAC not recommended currently
- Second line tx
- Thrombosis prevention is vital given high rates of recurrent thromboticevents
- Venous: warfarin
- Arterial: warfarin plus ASA for high CV risk patients
- Address reversible risk factors
- Thrombosis prevention is vital given high rates of recurrent thromboticevents