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Anti-Phospholipid Syndrome (Presentation (signs (purpura, Livedo…
Anti-Phospholipid Syndrome
Presentation
signs
purpura
splinter haemorrhages
Livedo reticularis
common cutaneous finding
valvular heart disease
symptoms
recurrent venous/ arterial thrombi e.g.CVA
Recurrent pulmonary emboli or thrombosis can lead to life-threatening pulmonary hypertension.
Recurrent spontaneous miscarriages
Late spontaneous fetal loss (second or third trimester) is common;
Recurrent early fetal loss (< 10 weeks’ gestation) is also possible.
May have Migraines
Catestrophic APS is a rare, serious, and often fatal manifestation (mortality rate of approximately 50%) characterized by multiorgan infarctions over a period of days to weeks.
Aetiology
risk factors
can be associated with other autoimmune conditions e.g.SLE
incidence- more common in women.
Investigations
Bloods
elevated Activated Partial Thromboplastin Time (APTT)
platlets- Thrombocytopenia
RBCs- Haemolytic anaemia.
antibodies
lupus anticoagulant
Anticardolipin Antibodies
anti-Beta 2 glycoprotein
Managment
Main stay of treatment is anticoagulation
If major thromboembolic event give warfarin
NB: warfarin is teratogenic
Aspirin 75mg daily.
Pts with recurrent pregnancy loss-LMWH during pregnancy
hyper-coaguable state so more prone to clots
