Pregnancy and Rheumatic Diseases

M. Gayed; C. Gordon


Rheumatology. 2007;46(11):1634-1640. 

In This Article

Anti-phospholipid Syndrome

(a) Thrombosis

All women are in a prothrombotic state during pregnancy and for 6 weeks postpartum and so are at increased risk of deep vein thrombosis, pulmonary emboli and stroke, irrespective of the presence or absence of antiphospholipid antibodies.[14,26] APS has been shown to increase the risk of all types of arterial and venous thrombosis in pregnancy, not just deep vein thrombosis and pulmonary emboli. A higher pregnancy success rate has been shown in women with APS and recurrent miscarriages taking low dose aspirin in pregnancy,[27] but this may not be sufficient for women with a history of recurrent miscarriages and with a history of thrombo-embolism, pre-eclampsia or stillbirth.[14,26] These patients are likely to require subcutaneous heparin. The optimal anti-thrombotic dose regime is dependant on the precise history of fetal loss and thrombosis.[1,14,26] It is also important to consider the risk of osteoporosis in women treated with heparin,[28] particularly in women taking steroids as well for SLE.

(b) Pre-eclampsia

Pre-eclampsia is most common in pregnancies where the mother has APS. In patients with APS, it often recurs and may present as very early onset pre-eclampsia (<20 weeks)[29,30]). Pre-eclampsia, is associated with uteroplacental insufficiency, caused by multiple placental thromboses, infarcts and a spinal artery vasculopathy in decidual vessels.[31] Although pre-eclampsia can be treated with bed rest and anti-hypertensives, in resistant cases the fetus should be delivered before progression to the more serious condition of eclampsia and the risk of fetal death. Eclampsia can be defined as a hypertensive crisis, associated with fits and a risk of coma. It is treated supportively to stabilize the epileptic fits and hypertension, but the definitive treatment is delivery of the fetus. Patients with APS also have an increased incidence and severity of the HELLP syndrome than the general population,[32] with or without pre-eclampsia or eclampsia. HELLP is usually treated by supportive care, steroids and delivery, but remission has been observed after plasma exchange in APS.[32] Occasionally, HELLP persists or presents for the first time in the postpartum period.

(c) Thombocytopenia

It should be noted that thrombocytopenia may occur for a variety of reasons in pregnancy. Although APS, lupus or HELLP are the best known associations in patients with rheumatic diseases, it should not be forgotten that approximately 9% of healthy women develop mild thrombocytopenia for non-autoimmune reasons. Isolated anti-platelet antibodies can cause an idiopathic thrombocytopenia. Thrombocytopenia associated with APS may worsen in pregnancy or due to treatment with heparin. However APS is more often the cause than heparin in patients with antiphospholipid antibodies.[14] The risk of heparin-induced thrombocytopenia can be reduced by using low-molecular weight heparin rather than unfractionated heparin. These two drugs are of equal effectiveness at preventing APS related pregnancy loss and thrombosis.[33] Worsening thrombocytopenia has been documented to herald the onset of thrombosis in APS patients, so the recommended management is dependant on the factor Xa levels. If the platelet count falls and the factor Xa level is found to indicate over anticoagulation with heparin, it is best to stop the heparin. If the factor Xa level is found to be in the target region or low, the heparin dose should only be reduced if there are any signs of bleeding or there is other evidence of heparin toxicity.


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