Antiphospholipid syndrome (APS)

1. Antiphospholipid syndrome
(APS)
Anna Rudaja MFIV 3.gr.

2. Characteristic
clinically : recurrent venous
or arterial thrombosis and/or
fetal loss
laboratory : persistently
elevated levels of antibodies
directed against membrane
anionic phospholipids (ie,
anticardiolipin [aCL]
antibody,
antiphosphatidylserine) or
their associated plasma
proteins, predominantly beta-2
glycoprotein I (apolipoprotein
H); or evidence of a circulating
anticoagulant

3. Pathophysiology
1. defect in cellular 1. oxidized beta-2
glycoprotein I is able to
apoptosis bind to and activate
2. membrane dendritic cells in a manner
similar to activation
phospholipids to the triggered by Toll-like
binding of various receptor 4 (TLR-4), which
plasma proteins could amplify the
production of
3. a phospholipid-protein autoantibodies.
complex is formed and 2. Complement activation
a neoepitope is has been increasingly
recognized as a possible
uncovered significant role in the
4. the target of pathogenesis of APS.
autoantibodies

4. Clinically
Clinically, the series of events that leads to hypercoagulability
and recurrent thrombosis can affect virtually any organ system,
including the following:
Peripheral venous system (deep venous thrombosis [DVT])
Central nervous system (cerebrovascular accident [CVA], sinus
thrombosis)
Hematologic (thrombocytopenia, hemolytic anemia)
Obstetric (pregnancy loss, eclampsia)
Pulmonary (pulmonary embolism [PE], pulmonary
hypertension)
Dermatologic (livedo reticularis, purpura, infarcts/ulceration)
Cardiac (Libman-Sacks valvulopathy, MI)
Ocular (amaurosis, retinal thrombosis)
Adrenal (infarction/hemorrhage)
Musculoskeletal (avascular necrosis of bone)

5. Epidemiology
Frequency: unknown. One to 5% of
healthy individuals have aPL antibodies.
No defined racial predominance
A female predominance has been
documented
is more common in young to middle-
aged adults; however, it also manifests in
children and elderly people.

6. Pregnancy morbidity
One or more late-term (>10 weeks' gestation)
spontaneous abortions
One or more premature births of a morphologically
healthy neonate at or before 34 weeks’ gestation
because of severe preeclampsia or eclampsia or
severe placental insufficiency
Three or more unexplained, consecutive, spontaneous
abortions before 10 weeks’ gestation
Th:
preferably low–molecular-weight heparin
long-term anticoagulation is then continued postpartum.
Breastfeeding women may use heparin and warfarin

7. Laboratory criteria:
Patients must have (1) medium to high
levels of immunoglobulin G (IgG) or
immunoglobulin M (IgM) anticardiolipin
(aCL), (2) anti–beta-2 glycoprotein I, or
(3) LA on at least 2 occasions at least 12
weeks apart.

8. Histologic Findings
noninflammatory bland thrombosis
with no signs of perivascular
inflammation or leukocytoclastic
vasculitis

9. Medical Care
Prophylactic therapy: CAPS (generally
nooral contraceptives, very ill ):
smoking, hypertension, or intensive anticoagulation,
hyperlipidemia plasma exchange, and
Low-dose aspirin corticosteroids
(unproven), Clopidogrel Intravenous
statins (hyperlipidemia) immunoglobulin
Thrombosis: Cyclophosphamide (have
chemotherapeutic
full anticoagulation with
activity) (didn’t prove)
i/v or s/b heparin +
warfarin th Rituximab
INR 2.0-3.0 for venous Hydroxychloroquine
thrombosis and 3.0 for (Plaquenil)
arterial thrombosis

10. Consultations, recommendations
Rheumatologist If warfarin therapy is
Hematologist instituted, instruct the
Neurologist, cardiologist, patient to avoid
pulmonologist, excessive consumption
hepatologist, of foods that contain
ophthalmologist vitamin K.
(depending on clinical Limit activity in patients
presentation) with acute DVT.
Obstetrician with Instruct the patient to
experience in high-risk avoid prolonged
pregnancies immobilization.

11. PALDIES PAR UZMANĪBU ;)
http://emedicine.medscape.com/article/