Pregnancy and Rheumatic Diseases

M. Gayed; C. Gordon

Disclosures

Rheumatology. 2007;46(11):1634-1640. 

In This Article

Systemic Sclerosis

Systemic sclerosis does not usually deteriorate during pregnancy, provided that the disease is stable at conception, but it may develop during pregnancy or the postpartum.[11,46] The features of systemic sclerosis in pregnancy are very similar to those described by healthy pregnant women, including gastro-oesophageal reflux disease and shortness of breath on exertion.[46] The shortness of breath on exertion is particularly worse in the third semester as the uterus enlarges, but it is important to rule out pulmonary hypertension during pre-conception counselling, as it is a contraindication to pregnancy due to the associated 50% risk of maternal death in all causes of pulmonary hypertension irrespective of underlying cause. Women with oesophageal disease who vomit during pregnancy have experienced Mallory–Weiss tears. This can be associated with life-threatening bleeding and recurrent vomiting that requires prompt hospital care. The second half of pregnancy is associated with a physiological increase in cardiac output, which usually results in an improvement in the Raynaud's phenomenon. Skin manifestations in systemic sclerosis usually improve during pregnancy, but there is often a deterioration in sclerodermatous skin changes in the post-natal period.[11,46]

Hypertension in systemic sclerosis can lead to a renal crisis. This is the greatest risk to mother and baby, especially as it is difficult to identify and treat, and mimics the presentation of pre-eclampsia and HELLP syndrome. However, a systemic sclerosis renal crisis can be differentiated by the daily increases in creatinine levels and an absence of proteinuria, whereas the HELLP syndrome is usually characterized by elevated liver function tests, proteinuria and oedema.[11,46] Renal crisis has been reported to be commoner in patients who have had systemic sclerosis for less than 5 yrs. ACE inhibitors are a life-saving treatment in hypertensive renal crisis in patients with systemic sclerosis despite their association with congenital malformations and kidney dysfunction in the infant.[46] In contrast to pre-eclampsia, delivery does not affect the hypertension or renal crisis seen in systemic sclerosis. If a woman has experienced a renal crisis during a previous pregnancy, she should avoid pregnancy until disease has been stabilized, which is usually 3--5 yrs from the onset of symptoms. These women are usually treated with nifedipine to maintain blood pressure control, but delivery is usually recommended if appropriate antihypertensives fail. ACE inhibitors may be initiated during pregnancy in severe cases after appropriate counselling about the risk of congenital abnormalities.[46]

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