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Autoimmune rheumatic disorders and vasculitis in pregnancy 

Autoimmune rheumatic disorders and vasculitis in pregnancy

Chapter:
Autoimmune rheumatic disorders and vasculitis in pregnancy
Author(s):

May Ching Soh

, Kate Bramham

, Sarah Germain

, and Catherine Nelson-Piercy

DOI:
10.1093/med/9780199204854.003.1414_update_002

Update:

Disease-modifying drugs in pregnancy and breastfeeding—updated information.

Antiphospholipid syndrome—discussion of use of low-dose prednisolone to prevent pregnancy losses.

Rheumatoid arthritis—comments on (1) use of rituximab in pregnancy; (2) vaccination after use of anti-TNFα‎ agents in pregnancy.

Scleroderma—expanded notes on effect of scleroderma on pregnancy.

Updated on 28 Nov 2012. The previous version of this content can be found here.
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date: 23 April 2017

Autoimmune diseases affect 5 to 7% of people, are commoner in women of childbearing age, and are frequently encountered in pregnancy. They may remit or improve during pregnancy, but can flare or present in pregnancy with disastrous consequences. The postpartum period is a time of susceptibility to autoimmune disorders, and women who already have an autoimmune disorder may suffer disease exacerbation following pregnancy.

Systemic lupus erythematosus (SLE)

The mother—pregnancy probably exacerbates SLE and increases the likelihood of a flare, which can be difficult to diagnose since many features (e.g. hair loss, oedema, facial erythema, fatigue, musculoskeletal pain) also occur in normal pregnancy. Differentiation of active renal lupus from pre-eclampsia is notoriously difficult, and the two conditions may be superimposed: renal flares are more common if disease is active within 6 months of conception, in particular in women with hypertension, heavy proteinuria, or high baseline serum creatinine. There is an increased risk of maternal thrombosis and premature atherosclerosis.

The fetus—SLE is associated with increased risks of adverse pregnancy outcome including fetal death and IUGR. Most fetal losses occur in association with secondary antiphospholipid syndrome or active disease, particularly renal. For women with SLE in remission and without hypertension, renal involvement, or the antiphospholipid syndrome, the risk of problems in pregnancy is similar to that of the general population.

Management—flares of SLE must be actively managed, pre-pregnancy counselling should be encouraged with treatment depending on both organ involvement and severity. Mild cases can be managed with analgesics alone (paracetamol); rash and arthritis will usually respond to NSAIDs, low dose prednisolone and/or hydroxycholorquine; more severe disease may require introduction of a disease modifying agent e.g. azathioprine or higher steroid dose. Steroids remain first-line treatment for severe lupus flares in pregnancy (and treatment of other autoimmune conditions), with the benefits of rapid disease control outweighing the risks.

The baby—neonatal lupus syndromes are caused by transplacental passage of autoantibodies directed against cytoplasmic ribonucleoproteins Ro and La. Cutaneous neonatal lupus is the most common manifestation (5%) and congenital heart block the most serious (20% mortality).

Antiphospholipid syndrome

Clinical features—antiphospholipid antibodies (aPLs) include anticardiolipin antibodies (IgG and/or IgM), lupus anticoagulant, and anti-β‎2-glycoprotein-I antibody. Antiphospholipid syndrome (APS) is the combination of any of these with one or more of the characteristic clinical features: (1) thrombosis—arterial, venous or capillary; (2) three or more consecutive ≤10-week losses or late pregnancy loss, typically in the second trimester; and (3) adverse pregnancy outcome as a result of placental insufficiency. aPLs are acquired antibodies, are common in the asymptomatic population, and are usually transient. Hence aPLs in the absence of relevant clinical history does not equate to APS and universal screening of all pregnant women is not indicated.

Management—aim is to improve pregnancy outcome and prevent maternal thrombosis. This will require low-dose aspirin from early pregnancy for prevention of pre-eclampsia ± low molecular weight heparin (LMWH). Close fetal and maternal surveillance are required.

Rheumatoid arthritis

Women with rheumatoid arthritis (RA) are less fertile for a variety of reasons. The old adage that RA improves in pregnancy no longer holds true, with less than a quarter of women objectively improving in pregnancy. Improvement is linked to the absence of rheumatoid factor and anti-cyclic citrullinated peptide (anti-CCP). Women with active RA have worse obstetric outcomes. Postpartum flares of RA are common. The main concerns are the disease-modifying antirheumatic drugs (DMARDs) and anti-tumor necrosis factor alpha (anti-TNFα‎) agents and their safety in pregnancy and lactation.

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