antiphospholipid syndrome

ANTIPHOSPHOLIPID SYNDROME

AIMS

Antiphospholipid syndrome Pathophysiology diagnosis Treatment in pregnancy

APS/APLS/HUGHES SYNDROME

Autoimmune, hypercoagulable state caused by antibodies against cell-membrane phospholipids.

Leads to thrombosis and pregnancy related complications such as miscarriage, stillbirth, preterm delivery and severe preeclampsia

BURDEN OF DISEASE

CMACH:

ANTIPHOSPHOLIPID SYNDROME

Thrombosis (venous, arterial, microcirculation) and/or

Pregnancy morbidity >3 consecutive miscarriage <10 weeks >1 fetal death > 10 weeks >1 preterm birth <34 weeks with normal fetal

morphology due to pre-eclampsia or placental insufficiency

Combined with presence of aPL on 2 occasions, at least 6 weeks apart

DIAGNOSIS

Research criteria for defining the antiphospholipid syndrome. Adapted from Miyakis et al (2006). With permission, John Wiley & Sons,Inc. © 2006 International Society on Thrombosis and Haemostasis.

ANTIPHOSPHOLIPID ANTIBODIES (APL)

Heterogenous antibodies directed against anionic phospholipids/ phospholipid binding proteins (B2gp1, prothrombin, factor V,C,S, annexin-V)

Commonest are anticardiolipin antibody (aCL) and lupus anticoagulant (LA)

TYPES

Primary Alone Genetic marker: HLA-DR7

Secondary Associated with SLE or other autoimmune

disorders Genetic marker: HLA-DR2/3, HLA-B8 Race: Blacks, hispanic, asians, native americans

CLINICAL FEATURES

• Immune thrombocytopenia (ITP), haemolytic anaemia

• Cerebral: epilepsy, infarction, chorea, mirgraine, transverse myelitis

• Heart valve disease• Systemic/pulmonary hypertension• Livedo reticularis• Amorosis fugax• Leg ulcers

PATHOPHYSIOLOGY

DIAGNOSIS

PREGNANCY AND APLS

Risk of thrombosis exacerbated by the hypercoagulable pregnant state

Pre-existing ITP may worsen in pregnancy Increased risk of recc. Miscarriage, vascular

complications Complictions are less in

TREATMENT

RECOMENDATIONS IN PREGNANCY

TO TEST OR NOT TO TEST?

22 year old primip, presents with abdo pain and passing clots PV. Normally fit and well.

27 year old, para 1. 13/40, previous L arm DVT.

31 year old, 4 days postnatal. Previous TIA in postnatal period 1 yr previously.

18 year old with 2 previous miscarriages, presents with femerol DVT.