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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

Case Report ~ Discussion

Antiphospholipid syndrome  pulmonary embolism

~ diagnosis and approach

antiphospholipid syndrome aps
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)
aps epidemiology
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
aps pathophysiology
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
aps thromboembolic disease
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
aps pulmonary complication
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
aps pulmonary complication pulmonary embolism and infarction
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
pulmonary embolism pe
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
pe clinical presentation and differential diagnosis
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
diagnosis of pe assessment 1
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
diagnosis of pe assessment 2
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
diagnosis of pe pulmonary angiography 1
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

diagnosis of pe pulmonary angiography 2
Diagnosis of PE ~ Pulmonary angiography 2

Side effect

  • Flushing
  • Transient hypotension
  • Catheter – induced ectopic beats
diagnosis of pe pulmonary angiography 3
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)
diagnosis of pe pulmonary angiography 4
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
diagnosis of pe ventilation perfusion scintigraphy 1
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
diagnosis of pe ventilation perfusion scintigraphy 2
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

diagnosis of pe ventilation perfusion scintigraphy 3
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
diagnosis of pe ventilation perfusion scintigraphy 4
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
diagnosis of pe ventilation perfusion scintigraphy 5
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
diagnosis of pe ventilation perfusion scintigraphy 6
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
diagnosis of pe ventilation perfusion scintigraphy 7
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)
diagnosis of pe ventilation perfusion scintigraphy 9
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
diagnosis of pe spiral tomographic scan 1
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

diagnosis of pe spiral tomographic scan 2
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
diagnosis of pe spiral tomographic scan 3
Diagnosis of PE ~ Spiral tomographic scan 3

Limitation

  • Inability of spiral scanning to detect PE in subsegmental pulmonary arteries (sensitivity : 29%)
diagnosis of pe spiral tomographic scan 4
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.
diagnosis of pe d dimer assay 1
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)
diagnosis of pe d dimer assay 2
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.
diagnosis of pe d dimer assay 3
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.
diagnosis of pe mri 1
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)

diagnosis of pe mri 2
Diagnosis of PE ~ MRI 2

Advantage and Limitation

references 1
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
reference 2
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
slide41

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