Next to Last Day

Question 1

Antiphospholipid Antibodies?

Answer 1

-family of autoantibodies directed against phospholipid binding plasma protein (beta-glycoprotein)

Question 2

Clinical Manifestations of antiphospholipid syndrome?

Answer 2

• asymptomatic to catastrophic antiphospholipid syndrome
• have positive aPL
• to prevent secondary thrombosis its risk-stratified, give high intensity anticoaguation in APS patients

Question 3

Most common presentation of antiphospholipid syndrome?

Answer 3

• arterial thrombosis-stroke

- vein manifestation-deep vein thrombosis

Question 4

Side effects of Antiphospholipid Syndrome?

Answer 4

• pregnancy loss after 10 weeks gestation

- to prevent fetal loss give low-dose aspirin and heparin

Question 5

Catastrophis APS

Answer 5

-multiple thromboses of small/medium arteries over days

Question 6

-

Answer 6

-

Question 7

Pathogenesis of SLE

Answer 7

• 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

Question 8

Organ Systems affected by SLE

Answer 8

RASH PAIN O MD

• renal
• arthritis
• serositis
• hematologic
• photosensitivity
• ANA positive
• immunologic
• neurologic
• oral rash
• discoid rash

Must have 4 of these

Question 9

Clinical Features of Sjogren’s Syndrome?

Answer 9

-deratoconjunctivitis sicca (dry eyes)
-xerostomia (dry mouth)
-parotid gland swelling
44X increase for non-hodgkin’s lymphoma
Pulmonary,renal,neuromuscular, raynauds, arthralgias

Question 10

How is Sjogren’s Syndrome Diagnosed?

Answer 10

• subjective/objective assessment of dry eyes, dry mouth
• test: anticuclear abs, anti-Ro/SSA, antiLa/SSB
• salivary gland biopsy
• Schirmer’s test
• ANA

Question 11

Lupus symptoms

Answer 11

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

Question 12

Mixed Connective Tissue Disease

Answer 12

• lupus, scleroderma, inflammatory myositis
• **Raynaud’s
• U1-RNP antibodies (lack of renal/CNS)

Question 13

Major cause of death in Mixed Connective Tissue Disease?

Answer 13

pulmonary hypertension (give EKG)

Question 14

Undifferentiated Connective Tissue Disease

Answer 14

• Raynodes
• nail-fold capillary microscopy
• U1-RNP - MCTD
• DNA ab - SLE
• nuclear-SSc