antiphospholipid syndrome Flashcards
Define antiphospholipid syndrome (APS)
APS is the association of antibodies against own phospholipids (AID) (more than one), with a variety of clinical features-mainly thromboses and pregnancy related morbidity
(Pregnancy morbidity is defined as the loss of 3 or more embryos before the 10th week of gestation)
if no signs, but AB-called icidental antiphospholipid antibodies, not APS
primary APS-with no other AID
Secondary APS-in patient with other APD
Aetiology and risk factors of antiphospholipid syndrome (APS)
Autoimmune disease with very unclear origin
Pathophysiology is also not clear-but AB might activate epithelium and cause hyper prothrombotic states
Secondary APS associated with SLE
Risk factors Age-35 years SLE/RA/other AID Hx of other heam disorder Female 5x men 98% are white
Epidiemology of antiphospholipid syndrome (APS)
Unclear on exact number-and probably higher
1-5% of healthy population have APS, increasing with age
nearly 30% AB presence in SLE
White female most common
Signs and Sx of antiphospholipid syndrome (APS)
its a lot about suspicions on other events-
Vascular Thrombosis/pregnancy morbidity
Hisotry usually described unclear loss of pregnancy, reccurent, before 10 weeks
Apparently spontaneous thrombotic events-stroke, TIA, DVT etc-in young patients
thrombotic MI-nearly only in APS
can see signs of SLE/RA
nephropathy can also be present -oedema and proteinuria (foamy urine)
features of thrombocytopenia
Purple mottling in limbs-livo reticularis
Investigations of antiphospholipid syndrome (APS)
Lupus anticoagulant-
anti-cardiolipin,
anti-beta2-glycoprotein AB
positive 2 times, 12 weeks apart
ANA-can be raised if in SLE
FBC-thrombocytopenia possible
Urea-can be raised if nephropathy
Doppler, USS, CT-can show tons of thrombus
Diagnostic is mainly clinical–
1 sign of vascular thrombosis or pregnancy morbidity
WITH present of Antiphospholipid AB on 2 occasions 12 weeks apart
