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Case Report ~ Discussion. Antiphospholipid syndrome  p ulmonary embolism ~ diagnosis and approach. Antiphospholipid Syndrome (APS). APS is characterized by Recurrent venous or arterial thrombosis Recurrent fetal loss Thrombocytopenia

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Case Report ~ Discussion

Antiphospholipid syndrome  pulmonary embolism

~ diagnosis and approach


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Antiphospholipid Syndrome (APS)

APS is characterized by

  • Recurrent venous or arterial thrombosis

  • Recurrent fetal loss

  • Thrombocytopenia

  • Presence of antibodies to phospholipid such as anticardiolipin antibody (aCL) and lupus anticoagulant (LA)


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APS - Epidemiology

  • Prevalence of antiphospholipid antibodies in healthy population is 2% ~ 5%

  • For all the patient with APS –

    female : male = 2 : 1

  • Mean and median ages of patients in most reports is 35 to 45 years old


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APS - Pathophysiology

  • Alteration of endothelial cell function

  • Alteration of the coagulation regulatory system, erythrocyte and platelet

  • A cofactor, beta2 glycoprotein-I ,is required and enhances the binding of aCL to cardiolipid


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APS – Diagnosis 1


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APS – Diagnosis 2


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APS – Clinical Manisfestation


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APS – Thromboembolic Disease

  • Noninflammatory thromboembolic disease

  • All venous and arterial systems have been cited,including large,median and small vessels

  • Most common site and presentation

    v. : lower extremity in the femoral and

    popliteal system

    a. : embolic cerebrovascular accident

    and transient ischemic attack

  • The recurrent rate is high


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APS – pulmonary complication

  • Pulmonary embolism and infarction

  • Pulmonary hypertension

  • Major pulmonary arterial thrombosis

  • Pulmonary microthrombosis

  • Adult respiretory distress syndrome

  • Intraalveolar pulmonary hemorrhage

  • Post partum syndrome


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APS – pulmonary complication ~ Pulmonary embolism and infarction

  • Recurrent deep venous thromboses are the most common vascular occlusive events encountered in patient with antiphosphlipid antibody and these are accompanied by pulmonary embolism and infarction in 1/3 of cases


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Pulmonary Embolism (PE)

  • the third most common cardiovascular emergency after myocardial infarction

  • Mortality rate

    untreated : 30%

    anticoagulant treatment : 10%

  • Nonspecific signs and symptoms ~ cannot be accurately diagnosed on clinical grounds


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PE –Clinical Presentation and Differential Diagnosis

  • Clinical triad : dyspnea , pleuritic chest pain, and hemoptysis

  • Most common symptom : dyspnea

  • Uncommon manifestation include unexplained fever, arterial tachyarhythmias, wheezing


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Diagnosis of PE - Assessment 1

Chest radiography

  • Many patients with PE have a normal chest radiography

  • radiologic abnormalities : nonspecific, cannot distinguished from other pulmonary disorder

    Electrocardiogram

  • Frequently normal or nonspecific

  • Useful in differentiating between PE and myocardial infarction


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Diagnosis of PE – Assessment 2

Blood Gas Estimation

  • A normal arterial PaO2 does not exclude PE (PE patients : 10~15%)

  • A low arterial PaO2 is nonspecific and cannot be used to rule-in PE

  • Danger of hemorrhage following arterial puncture if the patient is treated with heparin or thrombolytic therapy

  • Of limited value in the diagnosis of PE


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Diagnosis of PE ~ Pulmonary angiography 1

  • The standard for diagnosing pulmonary embolism (diagnostic accuracy : 80 ~ 95%)

  • Relative contraindication :

    (1)significant bleeding risk -

    platelet > 75000

    (2)allergy to the contrast medium

    (3)renal insufficiency  adequent

    hydration after angiography


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Diagnosis of PE ~ Pulmonary angiography 2

Side effect

  • Flushing

  • Transient hypotension

  • Catheter – induced ectopic beats


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Diagnosis of PE ~ Pulmonary angiography 3

  • Increased risk of complication

    (1)acute or severe chronic pulmonary

    hypertension

    (2)right heart failure

    (3)resperatory failure

  • Reduced risk of complication : selective arterial injection and limiting amount of contrast medium (low osmolality)


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Diagnosis of PE ~ Pulmonary angiography 4

  • Mortality rate : 0.5 %

  • Mordality required intubation : 0.4%

    required dialysis : 0.3%

  • Limitation : expensive, invasive, has small but significant risks and requires experienced physicians and supporting staff

  • Most commonly ued when ventilation-perfusion scanning is nondiagnostic but clinical suspicion remains high


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Diagnosis of PE ~ Ventilation – perfusion scintigraphy 1

  • Most commonly used non-invasive technique with clinical suspicion

  • Perfusion lung scan : not specific enough for diagnosis of PE

  • Ventilation imaging : differentiate vascular occlusion from disorder of ventilation


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Diagnosis of PE ~ Ventilation – perfusion scintigraphy 2

Segmental defect

  • Occlusion of a branch of a branch of the pulmonary artery

  • Wedge shape and pleural based

  • Conforms to segmental anatomy of the lung

  • Large (>75%), moderate(25~75%), small(<25%)

    Nonsegmental defect


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Diagnosis of PE ~ Ventilation – perfusion scintigraphy 3

V / Q match

  • Both scintigrams are abnormal in the same area, defects of equal size

    V / Q mismatch

  • Abnormal perfusion in the area of normal ventilation or much larger perfusion abnormality than ventilation defect


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Diagnosis of PE ~ Ventilation – perfusion scintigraphy 4

High probability

  • Segmental or lobar perfusion defect with normal ventilation

    Low probability of PE

  • Perfusion defect with matched ventilation abnormality


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Modified PIOPED Criteria

High probability (>80%)

2 large mismatched segmental defects without radiographic abnormality

Any combination of mismatched defects equivalent to the above

(2 moderate = 1 large)

Intermediate probability (20~80%)

Low probability (<20%)

Nonsegmental perfusion defect

Any perfusion defect with a substantially larger radiographic abnormality

Matched ventilation and perfusion defects with normal chest radiograph

Small subsegmental perfusion defects

Normal

( No perfusion defect )

Diagnosis of PE ~ Ventilation – perfusion scintigraphy 5


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Diagnosis of PE ~ Ventilation – perfusion scintigraphy 6

Condition associated with V/Q mismatch

  • Acute or chronic PE

  • Other cause of embolism : drug abuse, iatrogenic

  • Bronchogenic carcinoma

  • Hypoplasia or aplasia of pulmonary artery

  • Vasculitis

  • Post radiation therapy

  • Mediastinal or hilar adenopathy with obstruction of pulmonary artery or veins

  • Swyer – James syndrme


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Diagnosis of PE ~ Ventilation – perfusion scintigraphy 7

Determinining Clinical Likelihood of PE

  • Assessment of risk factor for venous thromboembolism (leg paralysis, bed rest, malignancy, CHF, presence of central venous catheter …)

  • Evaluation of symptoms and signs

  • Interpretation of preliminary investigation (eg. chest radiograph and electrocardiogram)


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Diagnosis of PE ~ Ventilation – perfusion scintigraphy 8


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Diagnosis of PE ~ Ventilation – perfusion scintigraphy 9

  • In PIOPED, ventilation-perfusion scans

    34% were read as low probability

    39% were read as intermediate probability

    additional diagnostic studies must be pursued

  • After pulmonary angiography, PE (+)

    patients with low-probability : 16%

    patients with intermediate-probability : 33%

  • the interobserver disagreement for intermediate- and low-probability ventilation-perfusion scans was 25% and 30%, respectively


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Diagnosis of PE ~ Spiral tomographic scan 1

  • capable of imaging nearly the entire thorax during a single breath-hold intravenous contrast can be timed to arrive pulmonary vasculature

  • Sensitivity : 64 ~ 93 %

    Specificity : 89~100 %

    Especially when PE is involved the main, lobar, or segmental pulmonary arteries


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Diagnosis of PE ~ Spiral tomographic scan 2

Advantage

  • High sensitivity and specificity

  • Visualize the clot

  • Indentify other disease states that can mimic PE (lung tumor, pleyral disease, pericardial disease)  provide alternative diagnosis

  • Cost : 1/6 ~ 1/8 angiography


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Diagnosis of PE ~ Spiral tomographic scan 3

Limitation

  • Inability of spiral scanning to detect PE in subsegmental pulmonary arteries (sensitivity : 29%)


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Diagnosis of PE ~ Spiral tomographic scan 4

Clinical guidelines

  • It should be used as a ”rule-in” modality, rather than a ”rule-out” procedure

  • if an alternative diagnosis is being considered in addition to pulmonary embolism, spiral CT scanning can provide new information that a ventilation-perfusion scan cannot.


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Diagnosis of PE ~ D-dimer assay 1

  • Rapid, noninvasive and inexpensive

  • Commonly found in the circulation when venous thromboembolism is present

  • Also found in other disease state (cancer, CHF, inflammatory condition)


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Diagnosis of PE ~ D-dimer assay 2

  • Two general methods of measuring D-dimers : ELISA method, latex agglutination

  • Elevated D-dimer fragments are too nonspecific for diagnosis of venous thromboembolism by themselves. With negative predictive values close to 100%, certain D-dimer assays have the potential to be the only screening test necessary to” rule out” venous thromboembolism.


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Diagnosis of PE ~ D-dimer assay 3

  • To be used in a diagnostic strategy, the details of the assay should be known : type (latex or ELISA), operating characteristics (sensitivity and negative predictive value), and outcomes of clinical studies supporting the particular assay.

  • Testing for D-dimers should be restricted to patients in whom clinical suspicion of venous thromboembolism is low or moderate.


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Diagnosis of PE ~ MRI 1

  • Helpful for the diagnosis of pelvic and thigh deep venous thrombosis

  • Acute, symptomatic, proximal deep vein thromboses : sensitivity approaching 100%

  • Less sensitive for detecting calf deep venous thrombosis

  • PE : can demonstrate an embolus directly as an intrvascular filling defect

    (sensitivity : < pulmonary angiography)


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Diagnosis of PE ~ MRI 2

Advantage and Limitation


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PE – Diagnostic Approach 1


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PE – Diagnostic Approach 2


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References 1

  • Antiphospholipid-Thrombosis Syndromes / Haemostasis 1999 ; 29:100-110

  • Antiphospholipid Syndrome / The journal of Family Practice, Vol.38, No.6(Jun), 1994

  • Review: Antiphospholipid Antibodies and the Lung / The journal of Rheumatology 1995 ; 22:62-6


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Reference 2

  • The Diagnosis of Pulmonary Embolism / Haemostasis 1995 ; 25:72-87

  • Non-invasive diagnosis of pulmonary aembolism / International Jounal of Cardiology 65(Suppl.1)1998 s83-s86

  • Improving Detection of venous thromboembolism / Postgraduate Medicine vol.108, No.4, September15, 2000


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謝 謝 大 家 !

8501067 韋又菁


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