CME questions: antiphospholipid syndrome.

1. The international consensus statement on classification criteria for antiphospholipid syndrome (Sapporo criteria) includes laboratory and clinical criteria. The laboratory criteria include:

a. Any antiphospholipid antibody documented by ELISA 

b. Moderate to high titer IgG, IgM, or IgA anticardiolipin antibody only

c. Moderate to high titer IgG or IgM anticardiolipin antibody or positive lupus anticoagulant

d. Biologic false positive serologic test for syphilis and lupus anticoagulant

2. The international consensus statement on classification criteria for antiphospholipid syndrome ("Sapporo criteria") includes laboratory and clinical criteria. Clinical criteria include:

a. Vasculitis or pregnancy loss only

b. Arterial or venous thrombosis only

c. Thrombocytopenia, thrombosis, and pregnancy loss or pre-eclampsia

d. Vascular thrombosis or pregnancy morbidity, including recurrent early losses, late loss, or premature births due to severe pre-eclampsia or severe placental insufficiency

3. Autoimmune antiphospholipid antibodies:

a. Represent a spectrum of auto antibodies directed at phospholipid-binding plasma proteins

b. Represent a spectrum of antibodies directed specifically at cardiolipin only

c. Interfere with hemostasis by increasing plasma levels of prothrombin

d. Represent an autosomal recessive genetic disorder due to a single gene mutation

4. Which is thought to increase likelihood of thrombosis in an aPL(+) individual, in keeping with the "second hit" hypothesis?

a. Inherited thrombophilia such as factor V Leiden 

b. Oral contraceptives

c. Prolonged immobilization

d. All of the above

5. The most common presentation of arterial thrombosis in antiphospholipid syndrome (APS) patients is:

a. Aortic occlusion

b. Stroke and transient ischemic attack 

c. Digital gangrene

d. Renal artery thrombosis 

6. Hematologic manifestations associated with APS include:

a. Thrombocytopenia, Coombs positive hemolytic anemia, and thrombotic microangiopathic hemolytic anemia 

b. Thrombocythemia

c. Thrombocytopenia only

d. Thrombocytopenia with bone marrow fibrosis

7. Antiphosopholipid-related nephropathy is characterized by presence of renal biopsy findings of:

a. WHO class IV glomerulonephritis 

b. Necrotizing vasculitis

c. Thrombotic microangiopathy with glomerular thrombi and atrophy

d. Amyloid deposition

8. The most common cardiac manifestation of APS is:

a. Atrial thrombus

b. Valvular heart disease, especially aortic and mitral regurgitation

c. Thrombotic cardiomyopathy

d. Restrictive cardiomyopathy

9. Which of the following statements regarding obstetric complications of APS is false?

a. The two greatest risk factors for fetal loss are high titer IgG anticardiolipin (aCL) and a history of prior fetal loss.

b. Obstetric complications may include early severe pre-eclampsia and/or HELLP syndrome.

c. Screening of every pregnant woman for aCL and lupus anticoagulant (LA), regardless of obstetric history, is now recommended.

d. Concordance of aCL and LA tests are incomplete, so both must be checked if APS is suspected.

10. Which of the following statements regarding treatment of APS manifestations is false?

a. No prospective data exist to support prophylactic treatment of asymptomatic aPL(+) patients, but high titer patients are often treated with low-dose aspirin.

b. Standard treatment of a single arterial thrombosis is anticoagulation with aspirin 325 mg in combination with warfarin at an INR of 3.5-4.0.

c. Standard treatment of recurrent pregnancy loss associated with aPL is low-dose aspirin and a prophylactic dose of subcutaneous heparin.

d. Precise type, duration, and intensity of anticoagulation is still in question for many patients with aPL-related thromboses.

Answers to CME Questions

1. C, 2. D, 3. A, 4. D, 5. B, 6. A, 7. C, 8. B, 9. C, 10. B