CNSLF Path - Laboratory Dx of Connective Tissue Disorders (ii) Antiphospholipid Syndrome

Intro

acquired thrombophilia (prothrombotic/hypercoaguable state)

due to abs against phospholipids

clinical + lab criteria most be fulled to establish dx

may be primary or secondary to another CTD (e.g. SLE)

Clinical criteria

documented venous thrombosis (i.e. not due to long haul flight or anything else)

pul veins

IVC

Hep (budd-chiari syndrome) + portal veins

renal vein

superficial or deep veins of leg

or documented art thrombosis

cerebral arts

retinal arts

brachial arts

coronary arts (MI with no risk factors)

arts to extremities

mesenteric art

or pregnancy associated morbidity

mid to 3rd term loss

recurrent 1st trio less (3 consecutive times, as 20% of 1st tri pregnancies miscarry anyway)

severe pre-eclampsia (PET - pre-eclampsia toxaemia) requiring delivery before 36 wks

severe intrauterine growth retardation

due to clots in placenta - impaired function

Associated features (not diagnostic)

thrombocytopenia

livedo reticularis

mottled rash due to blocked vessels

white patches surrounded by red due to other vessels dilating

can be subtle

transverse myelitis (inflamm of spinal cord)

migraine

Lab criteria

lupus anticoagulant or anti-cardiolipin or anti-beta 2 gp 1

+ve on 2 occasions @ least 12 wks apart

varies with manifestation

aspirin when pregnant

lifelong anticoagulation (warfarin more effective than DOACs)