Next to Last Day Flashcards
Antiphospholipid Antibodies?
-family of autoantibodies directed against phospholipid binding plasma protein (beta-glycoprotein)
Clinical Manifestations of antiphospholipid syndrome?
- asymptomatic to catastrophic antiphospholipid syndrome
- have positive aPL
- to prevent secondary thrombosis its risk-stratified, give high intensity anticoaguation in APS patients
Most common presentation of antiphospholipid syndrome?
- arterial thrombosis-stroke
- vein manifestation-deep vein thrombosis
Side effects of Antiphospholipid Syndrome?
- pregnancy loss after 10 weeks gestation
- to prevent fetal loss give low-dose aspirin and heparin
Catastrophis APS
-multiple thromboses of small/medium arteries over days
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Pathogenesis of SLE
- ongoing autoimmune reactions-autoantibodies/other protein products
- complement deficiencies confer highest risk (mutation in TREX1)
- environmental: UV light
- Type I INF-immune activation (key player)
- platelets/neutrophils/complement
Organ Systems affected by SLE
RASH PAIN O MD
- renal
- arthritis
- serositis
- hematologic
- photosensitivity
- ANA positive
- immunologic
- neurologic
- oral rash
- discoid rash
Must have 4 of these
Clinical Features of Sjogren’s Syndrome?
-deratoconjunctivitis sicca (dry eyes)
-xerostomia (dry mouth)
-parotid gland swelling
44X increase for non-hodgkin’s lymphoma
Pulmonary,renal,neuromuscular, raynauds, arthralgias
How is Sjogren’s Syndrome Diagnosed?
- subjective/objective assessment of dry eyes, dry mouth
- test: anticuclear abs, anti-Ro/SSA, antiLa/SSB
- salivary gland biopsy
- Schirmer’s test
- ANA
Lupus symptoms
Jaccoud’s arthropathy - joint not eroded
Serositis-pleura, pericardium, peritoneal cavity
-chest pain, alveolar hemorrhage
-shrinking lung syndrome
Kidney Involvement-anti-DNA-ab
Raynodes - vascular loss to fingers
Mixed Connective Tissue Disease
- lupus, scleroderma, inflammatory myositis
- **Raynaud’s
- U1-RNP antibodies (lack of renal/CNS)
Major cause of death in Mixed Connective Tissue Disease?
pulmonary hypertension (give EKG)
Undifferentiated Connective Tissue Disease
- Raynodes
- nail-fold capillary microscopy
- U1-RNP - MCTD
- DNA ab - SLE
- nuclear-SSc