What is the recommended management for a patient with Takayasu arteritis during pregnancy?

Management of Takayasu Arteritis During Pregnancy

Pregnancies in women with Takayasu arteritis must be treated as high-risk and require achieving disease quiescence before conception, with close multidisciplinary monitoring throughout pregnancy to prevent life-threatening maternal cardiovascular complications that occur in >5% of cases. 1

Pre-Conception Planning

Disease control before conception is critical to improve both maternal and fetal outcomes. 1

• Ensure disease quiescence for at least 6-12 months before attempting conception, as active disease is independently associated with poor pregnancy outcomes 1
• Optimize blood pressure control before pregnancy, as hypertension and its complications (including preeclampsia) are major risks 1, 2
• Transition to pregnancy-compatible immunosuppressive agents if needed—methotrexate must be discontinued at least 3 months before conception 3
• Perform baseline imaging (MRI or CT angiography) to document vascular status before pregnancy 3

Medical Management During Pregnancy

Immunosuppressive Therapy

• Continue corticosteroids during pregnancy if needed for disease control—prednisone at the lowest effective dose is considered safe 3, 4
• Azathioprine (2 mg/kg/day) is the preferred steroid-sparing agent during pregnancy, as it is compatible with gestation 3, 4
• Avoid methotrexate entirely during pregnancy due to teratogenicity 3
• TNF inhibitors may be continued through early pregnancy if essential for disease control, though data are limited in Takayasu arteritis specifically 3

Hypertension Management

• Aggressive blood pressure control is essential, as hypertension increases complications 13-fold 2
• Use pregnancy-safe antihypertensives (labetalol, nifedipine, methyldopa) as first-line agents 2
• Monitor for refractory hypertension, which may indicate underlying renal artery stenosis requiring further evaluation 2
• Four-extremity blood pressure measurements should be obtained at every prenatal visit to detect asymmetric involvement 3

Antiplatelet Therapy

• Low-dose aspirin (81 mg daily) should be considered to prevent ischemic events and may reduce preeclampsia risk 3

Monitoring Protocol During Pregnancy

Long-term clinical monitoring is strongly recommended even when disease appears quiescent, as vascular changes can occur without clinical symptoms. 3

• Clinical assessment at each prenatal visit should include:

  • Four-extremity blood pressures to detect new stenoses 3
  • Vascular examination for new bruits or pulse deficits 5
  • Assessment for constitutional symptoms suggesting disease flare 3

• Inflammatory markers (ESR/CRP) should be measured regularly, though normal values do not exclude active disease (elevated in only 50% of active cases) 3, 4

• Imaging surveillance should be limited during pregnancy:

  • MRI without gadolinium is preferred if imaging is necessary 3
  • Avoid CT angiography due to radiation exposure 3
  • Reserve imaging for suspected new vascular complications or disease progression 3

Management of Disease Activity During Pregnancy

If new disease activity develops during pregnancy, initiate or increase corticosteroids immediately. 3, 4

• Start prednisone 40-60 mg daily for active inflammation 5, 3, 4
• Add azathioprine if corticosteroids alone are insufficient 3, 4
• Delay any elective revascularization procedures until after delivery and disease quiescence 5, 3
• Emergency revascularization may be necessary for life-threatening ischemia (limb, cardiac, cerebral), but should be performed with high-dose perioperative corticosteroids 3

Obstetric Considerations

Disease activity appears to remain stable or improve during pregnancy in most cases, but complications remain significant. 6

Major Maternal Risks

• Life-threatening cardiovascular complications occur in >5% of pregnancies 1
• Hypertensive disorders including preeclampsia 1, 2, 7
• Congestive heart failure 7
• Aortic complications (rare but catastrophic) 1

Fetal Risks

• Intrauterine growth restriction 6
• Prematurity 6, 7
• Intrauterine fetal death (particularly with uncontrolled disease) 8

Delivery Planning

• Vaginal delivery is preferred unless obstetric indications require cesarean section 1
• Avoid prolonged second stage of labor to minimize cardiovascular stress 1
• Consider assisted second stage (vacuum/forceps) to shorten pushing 1
• Epidural anesthesia is generally safe and may help control blood pressure during labor 1
• Have cardiovascular surgery backup available for high-risk patients 1

Multidisciplinary Team Approach

Management requires close collaboration between rheumatology, maternal-fetal medicine, cardiology, and vascular surgery. 8, 6

• Rheumatology: Disease activity monitoring and immunosuppression management 8
• Maternal-fetal medicine: High-risk obstetric care and fetal surveillance 8
• Cardiology: Cardiovascular risk assessment and hypertension management 8
• Vascular surgery: Consultation for potential vascular complications 3

Common Pitfalls to Avoid

• Do not rely solely on inflammatory markers to assess disease activity—they are normal in 50% of active cases 3
• Do not perform elective vascular procedures during pregnancy unless life- or organ-threatening ischemia is present 5, 3
• Do not assume disease quiescence based on clinical stability alone—subclinical vascular progression can occur 3
• Do not delay treatment of active disease during pregnancy—untreated inflammation poses greater risk than corticosteroid therapy 1

Postpartum Management

• Continue disease monitoring closely in the postpartum period 6
• Resume or optimize immunosuppressive therapy as needed 6
• Consider breastfeeding compatibility when selecting medications (prednisone and azathioprine are generally compatible; methotrexate is contraindicated) 3
• Plan long-term contraception and future pregnancy timing based on disease control 6