Connective tissue disease in pregnancy

Published on 09/03/2015 by admin

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Last modified 09/03/2015

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Chapter 49 Connective tissue disease in pregnancy

Systemic lupus erythematosus (SLE)

SLE is a multisystem autoimmune disease, involving direct attack by autoantibodies and deposition of immune complexes. It mainly affects women in the reproductive age group, making it a frequently encountered condition in obstetric practice. SLE has a prevalence of 1 in 5000–10,000 women, with a female to male ratio of 9:1. SLE is thought not to impair fertility. Pregnancy outcome for mother and offspring is best when the disease has been quiescent for at least 6 months prior to conception, and renal function is stable.

Pregnancy probably does not increase the rate of SLE exacerbation compared with the non-pregnant population. Flares occur equally across the trimesters, and are common in the immediate postpartum period. They present most commonly as fever, lymphadenopathy, skin and joint involvement, and renal impairment. Neurological, cardiovascular and respiratory manifestations may also occur. Exacerbation is more common when disease is active at the time of conception. Flare is associated with hypocomplementaemia and an increase in anti-double stranded DNA titres. One-third to one-half of SLE patients have antiphospholipid antibodies (aPL), and one-third have anti-Ro/SSA or anti-La/SSB antibodies.

Maternal effects

• pre-eclampsia: occurs in around 13% of patients with SLE, is much more frequent in women with preexisting renal disease and aPL, and can be difficult to distinguish from lupus nephritis

• lupus nephritis: associated with an increased risk of fetal loss and worsening of renal and extra-renal manifestations

• venous thromboembolism

• postpartum haemorrhage

Fetal effects

• intrauterine growth restriction (IUGR)

• intrauterine fetal death: risk is higher in women with active disease at conception, hypertension, renal disease, aPL, hypocomplementaemia, elevated anti-DNA antibodies and thrombocytopenia

• preterm birth: three times more common than in women without SLE

Neonatal lupus

• results from passive transfer of anti-Ro/SSA or anti-La/SSB antibodies

• causes complete heart block in the fetus/neonate in 2% of cases

• other features include rash, thrombocytopenia, hepatitis and haemolytic anaemia

• no congenital anomalies associated with SLE

Management

Pregnancy in women with SLE should be managed in a high-risk setting by an obstetrician and physician. Ideally, medication should be reviewed and disease management optimised preconceptually.

Initial investigations

• full blood count, renal function tests (including urinalysis, protein/creatinine ratio)

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