Rheumatology - Antiphospholipid Syndrome (APS)

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Questions and Answers

Which of the following best describes the underlying mechanism of anti-phospholipid syndrome (APS)?

  • An inflammatory process causing damage to blood vessel walls.
  • A genetic mutation affecting platelet production and function.
  • A deficiency in clotting factors leading to increased bleeding risk.
  • An autoimmune reaction resulting in the presence of procoagulatory antibodies. (correct)

A patient diagnosed with secondary anti-phospholipid syndrome (APS) most likely has which of the following co-existing conditions?

  • Systemic Lupus Erythematosus (SLE) (correct)
  • Osteoarthritis
  • Type 1 Diabetes Mellitus
  • Chronic Obstructive Pulmonary Disease (COPD)

Which of the following is a typical site for thrombosis in patients with anti-phospholipid syndrome (APS)?

  • Aortic valve
  • Deep veins of the leg (correct)
  • Coronary artery
  • Pulmonary artery

A 30-year-old female with anti-phospholipid syndrome (APS) presents with a history of three unexplained first-trimester miscarriages. Which of the following APS-related complications does this patient likely have?

<p>Pregnancy Losses (D)</p>
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Which of the following is considered one of the 'other manifestations' of anti-phospholipid syndrome (APS)?

<p>Livedo reticularis (C)</p>
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A patient presents with thrombosis in multiple organs occurring within a week, along with microangiopathy. Which complication of anti-phospholipid syndrome (APS) is most likely?

<p>Catastrophic APS (B)</p>
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Which of the following laboratory findings is consistent with anti-phospholipid syndrome (APS)?

<p>Prolonged aPTT (A)</p>
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Which of the following antibodies is associated with anti-phospholipid syndrome (APS)?

<p>Anti-cardiolipin antibodies (C)</p>
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According to the diagnostic criteria for anti-phospholipid syndrome (APS), a patient must have at least how many positive laboratory criteria results, spaced how far apart?

<p>One positive result on two occasions, 12 weeks apart. (D)</p>
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Which of the following conditions should be considered in the differential diagnosis of anti-phospholipid syndrome (APS)?

<p>Vasculitis (C)</p>
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Which acquired condition can lead to a hypercoagulable state that should be considered in the differential diagnosis of APS?

<p>Hormonal contraception use (B)</p>
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What is the recommended long-term treatment for thrombosis in patients with anti-phospholipid syndrome (APS)?

<p>Warfarin (A)</p>
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Which of the following medications is typically used as anticoagulation for pregnant women with anti-phospholipid syndrome (APS)?

<p>Subcutaneous Heparin (C)</p>
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Which of the following treatment options is most appropriate for a patient experiencing catastrophic anti-phospholipid syndrome (CAPS)?

<p>Steroids, IV Heparin, IV Ig, and Plasma Exchange (B)</p>
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A patient with APS is being treated with warfarin. What is the target INR range for therapeutic anticoagulation in this patient?

<p>2.0-3.0 (D)</p>
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A 28-year-old pregnant woman with APS is being treated with low-molecular weight heparin. Which of the following complications is most important to monitor for?

<p>Heparin-induced thrombocytopenia (HIT) (C)</p>
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Which of the following clinical scenarios would raise suspicion for catastrophic anti-phospholipid syndrome (CAPS)?

<p>Thrombosis in multiple organs within a short period (D)</p>
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A patient with APS has a history of recurrent arterial and venous thromboses despite being on warfarin therapy with a therapeutic INR. Which of the following approaches might be considered?

<p>Adding aspirin to the warfarin regimen (D)</p>
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Which of the following statements about warfarin and pregnancy is most accurate in the context of managing APS?

<p>Warfarin is teratogenic and should be avoided, especially in the first trimester. (C)</p>
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Besides anticoagulation and antiplatelet medications, what other medication has been shown to decrease the risk of thrombosis and pregnancy loss in APS patients?

<p>Hydroxychloroquine (D)</p>
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A patient with anti-phospholipid syndrome (APS) experiences new-onset cardiac valvular dysfunction. Which specific cardiac manifestation is most commonly associated with APS?

<p>Mitral regurgitation due to Libman-Sacks endocarditis (D)</p>
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In the context of diagnosing anti-phospholipid syndrome (APS), which factor distinguishes primary APS from secondary APS?

<p>Association with other autoimmune diseases (C)</p>
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Which specific laboratory finding, beyond the standard antiphospholipid antibody panel, would raise strong suspicion for catastrophic anti-phospholipid syndrome (CAPS) in a patient presenting with multi-organ thrombosis?

<p>Evidence of microangiopathic hemolytic anemia (C)</p>
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A patient with APS is on long-term warfarin therapy. They develop a gastrointestinal bleed. After the bleed is controlled, what is the most appropriate long-term anticoagulation strategy, considering the need to balance thrombotic risk and bleeding risk?

<p>Resume warfarin at a lower target INR of 1.5-2.0 (C)</p>
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A 35-year-old woman with APS and a history of recurrent miscarriages is planning a pregnancy. She is currently managed with low-dose aspirin. What additional intervention is most crucial to improve her chances of a successful pregnancy?

<p>Add hydroxychloroquine to her treatment regimen (C)</p>
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Which of the following is the most specific diagnostic criterion for anti-phospholipid syndrome (APS) according to the revised Sapporo criteria?

<p>The presence of lupus anticoagulant (LA) (C)</p>
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A young female patient presents with livedo reticularis, migraine headaches, and recurrent transient ischemic attacks (TIAs). While evaluating for possible causes, which of the following co-existing conditions would most strongly suggest a diagnosis of secondary anti-phospholipid syndrome (APS)?

<p>Systemic Lupus Erythematosus (SLE) (D)</p>
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A patient is being evaluated for recurrent early miscarriages and possible anti-phospholipid syndrome (APS). If anti-cardiolipin antibodies are detected, how many weeks apart should the positive tests be to meet the laboratory criteria for APS?

<p>12 weeks (B)</p>
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Which of the following clinical findings is most suggestive of catastrophic anti-phospholipid syndrome (CAPS) rather than typical APS?

<p>Thrombosis in multiple organs within a week (B)</p>
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A 40-year-old male with a history of anti-phospholipid syndrome (APS) presents with acute onset of neurological symptoms, including confusion and focal deficits, along with thrombocytopenia. MRI of the brain reveals multiple small infarcts. What immediate treatment strategy is most appropriate?

<p>Administer high-dose corticosteroids, intravenous immunoglobulin (IVIG), and anticoagulation (D)</p>
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A patient with anti-phospholipid syndrome (APS) is being treated with warfarin for secondary prevention of thrombosis. They require an urgent surgical procedure with a high risk of bleeding. Which of the following is the most appropriate strategy for managing their anticoagulation?

<p>Stop warfarin 5 days before surgery and bridge with therapeutic-dose LMWH, stopping LMWH 24 hours before surgery (D)</p>
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In a patient with APS who has persistent thrombocytopenia despite anticoagulation, which additional therapy might be considered?

<p>Rituximab (A)</p>
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Which statement most accurately reflects the interaction between warfarin and pregnancy in the context of anti-phospholipid syndrome (APS) management?

<p>Warfarin is teratogenic and should be avoided during pregnancy; low-molecular-weight heparin (LMWH) is preferred (C)</p>
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A patient with APS and recurrent arterial thromboses despite therapeutic anticoagulation is found to have persistently elevated levels of anti-beta2-glycoprotein I antibodies. Which of the following additional treatment strategies might be considered to reduce the risk of further thrombotic events?

<p>Adding hydroxychloroquine (A)</p>
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Aside from thrombosis and pregnancy complications, what other clinical manifestation is recognized as a criterion for diagnosing anti-phospholipid syndrome (APS)?

<p>Thrombocytopenia (A)</p>
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A patient with a confirmed diagnosis of anti-phospholipid syndrome (APS) presents with sudden onset of dyspnea, chest pain, and hemoptysis. Which of the following typical sites of thrombosis is most likely?

<p>Pulmonary embolism (D)</p>
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Which of the following conditions is least likely to be included in the differential diagnosis of anti-phospholipid syndrome (APS)?

<p>Hereditary angioedema (D)</p>
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What is the primary reason for using low-molecular-weight heparin (LMWH) instead of warfarin in pregnant women with anti-phospholipid syndrome (APS)?

<p>Warfarin is teratogenic (A)</p>
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A patient with anti-phospholipid syndrome (APS) presents with a sudden onset of multiple thrombotic events affecting the brain, kidneys, and skin, accompanied by a rapid decline in cognitive function. Which of the following complications is most suspected?

<p>Catastrophic Anti-phospholipid Syndrome (CAPS) (B)</p>
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A 40-year-old female with APS presents with new onset of lower extremity edema and proteinuria. A renal biopsy reveals microangiopathic changes. Which of the following manifestations of APS is most likely?

<p>Microangiopathic nephropathy (A)</p>
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Flashcards

Anti-Phospholipid Syndrome (APS)

An autoimmune disorder with recurrent arterial or venous thrombosis and thrombocytopenia due to procoagulatory antibodies.

Primary APS

APS not associated with other autoimmune diseases.

Secondary APS

APS associated with other autoimmune diseases, like SLE.

Typical Thrombosis Sites in APS

Deep vein thrombosis, pulmonary embolism, and cerebrovascular accidents.

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Other Thrombotic Events in APS

Budd Chiari syndrome, cerebral sinus thrombosis, digital/myocardial infarctions.

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Pregnancy Losses in APS

≥3 unexplained miscarriages in first trimester, fetal death after 1st, premature births, eclampsia/preeclampsia.

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Other Manifestations of APS

Thrombocytopenia, mental status changes, livedo reticularis, skin ulcers, microangiopathic nephropathy, cardiac valvular dysfunction.

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Catastrophic APS Thrombosis

Thrombosis in multiple areas (>3 organs in 1 week).

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Catastrophic APS: Microangiopathy

Thrombotic microangiopathy.

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Catastrophic APS: Organ Failure

Multiorgan failure.

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Platelet Levels in APS

Thrombocytopenia.

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aPTT in APS

Prolonged.

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Antiphospholipid Antibodies

Lupus anticoagulant, anticardiolipin antibodies, anti-B2-glycoprotein antibodies.

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Clinical Criteria for APS Diagnosis

Arterial or venous thrombosis (confirmed) and fetal death >10 weeks gestational age.

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Other Clinical Criteria for APS Diagnosis

Recurrent spontaneous abortion before 10 weeks gestational age (≥ 3 times) and premature birth.

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Laboratory Criteria for APS Diagnosis

Lupus anticoagulant (LA), Anticardiolipin antibodies (IgG or IgM), Anti-β2-glycoprotein antibodies (IgG or IgM).

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Differential Diagnosis of APS

SLE, Vasculitis, Hypercoagulable states.

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Treatment for Thrombosis in APS

Lifelong anticoagulation (Warfarin) with target INR 2-3.

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Treatment for Pregnancy with APS

Anticoagulation (SC Heparin) and Antiplatelet (Low dose Aspirin).

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Treatment for Catastrophic APS

Steroids, IV Heparin, IV Ig, Plasma exchange.

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Hydroxychloroquine benefit in APS

Hydroxychloroquine reduces this in APS patients.

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Warfarin and Pregnancy

Important anticoagulant that is teratogenic and cannot be used in pregnancy.

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Study Notes

  • Autoimmune disorder involving recurrent arterial or venous thrombosis and thrombocytopenia due to procoagulatory antibodies

Classification

  • Primary: Idiopathic, not linked to other autoimmune conditions
  • Secondary: Associated with other autoimmune diseases, mainly SLE

Clinical Presentation

  • Often asymptomatic until a thrombotic event or pregnancy loss occurs

Typical Thrombosis Sites

  • Deep vein thrombosis (DVT)
  • Pulmonary embolism
  • Cerebrovascular accidents

Other Thrombotic Events

  • Budd-Chiari syndrome
  • Cerebral sinus thrombosis
  • Digital infarctions
  • Myocardial infarctions

Pregnancy Losses

  • At least 3 unexplained miscarriages during the first trimester
  • Unexplained fetal death after the first trimester
  • Premature births
  • Eclampsia or preeclampsia

Other Manifestations

  • Thrombocytopenia
  • Mental status changes
  • Livedo reticularis
  • Skin ulcers
  • Microangiopathic nephropathy
  • Cardiac valvular dysfunction, commonly mitral regurgitation from Libman-Sacks endocarditis

Complications

  • Catastrophic antiphospholipid syndrome involves
  • Thrombosis in multiple areas, affecting more than 3 organs within a week
  • Thrombotic microangiopathy
  • Multiorgan failure

Diagnosis

  • Routine lab studies show thrombocytopenia, and prolonged aPTT

Antiphospholipid Antibodies

  • Lupus anticoagulant (LA)
  • Anticardiolipin antibodies (IgG and IgM)
  • Anti-β2-glycoprotein antibodies (IgG and IgM)

Diagnostic Criteria

  • One clinical and one laboratory criteria are needed for diagnosis

Clinical Criteria

  • Arterial or venous thrombosis confirmed by imaging or histopathology
  • Fetal death occurring after 10 weeks of gestational age
  • Recurrent spontaneous abortion before 10 weeks gestational age, occurring 3 or more times
  • Premature birth

Laboratory Criteria

  • Must be positive on 2 occasions, 12 weeks apart
  • Lupus anticoagulant (LA)
  • Anticardiolipin antibodies (IgG or IgM)
  • Anti-β2-glycoprotein antibodies (IgG or IgM)

Differential Diagnosis

  • Rule out associated SLE
  • Exclude vasculitis, like Behçet's disease
  • Check for hypercoagulable states, both genetic and acquired

Genetic Hypercoagulable States

  • Antithrombin III deficiency
  • Factor V Leiden mutation
  • Protein C and protein S deficiency

Acquired Hypercoagulable States

  • Drugs like hormonal contraception and steroids
  • Immobility
  • Dehydration
  • Malignancy

Treatment

For Thrombosis

  • Lifelong anticoagulation using Warfarin, targeting an INR of 2-3
  • Hydroxychloroquine helps lower risks of thrombosis and pregnancy loss

For Pregnancy

  • Use anticoagulation with subcutaneous Heparin
  • Low-dose Aspirin as an antiplatelet

For Catastrophic Antiphospholipid Syndrome

  • Steroids
  • IV Heparin
  • IV Immunoglobulin (Ig)
  • Plasma exchange
  • Warfarin contraindicated during pregnancy, teratogenic

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