A 2‑month pregnant patient with Takayasu arteritis on azathioprine, low‑dose aspirin (acetylsalicylic acid) and prednisone—what is the recommended therapy during pregnancy?

Management of Takayasu Arteritis During Pregnancy

Continue azathioprine, prednisone (wysolone), and low-dose aspirin (ecosprin) throughout pregnancy without dose reduction, as this regimen is compatible with pregnancy and minimizes the risk of disease flare that could threaten both maternal and fetal outcomes. 1

Medication Management Strategy

Azathioprine (Continue)

• Azathioprine should be continued throughout pregnancy as it is considered compatible with pregnancy by the American College of Rheumatology 1
• The 2020 ACR guidelines strongly recommend continuing azathioprine during pregnancy for rheumatic diseases 1
• While azathioprine is FDA category D and can cause fetal harm in animal studies, extensive human data from inflammatory bowel disease, lupus nephritis, and autoimmune hepatitis patients demonstrate relative safety 1
• Azathioprine has been associated with low birth weight and preterm birth but not with increased congenital malformations in large cohort studies 1
• Do not taper or discontinue azathioprine during pregnancy, as this increases the risk of disease flare which poses greater risk to mother and fetus than continued therapy 1

Prednisone/Wysolone (Continue)

• Continue prednisone at the current maintenance dose throughout pregnancy 1
• Prednisone is considered low-risk during pregnancy and crosses the placenta minimally due to metabolism by placental 11β-hydroxysteroid dehydrogenase 1
• The ACR conditionally recommends continuing regular low-dose prednisone during pregnancy 1
• Do not taper corticosteroids during pregnancy or for at least 3 months postpartum to prevent disease flare 1
• Monitor for gestational diabetes and hypertension, which are increased with corticosteroid use 1

Low-Dose Aspirin/Ecosprin (Continue and Optimize)

• Continue low-dose aspirin (75-162 mg daily) starting in the first trimester 1
• Low-dose aspirin reduces the risk of fetal loss, preeclampsia, and intrauterine growth restriction in patients with inflammatory diseases 1
• The KDIGO guidelines suggest administration of low-dose aspirin during pregnancy to decrease the risk of fetal loss (Grade 2C) 1
• Aspirin should be continued throughout pregnancy and can be safely used during breastfeeding 1

Disease Activity Monitoring

Surveillance Schedule

• Monitor disease activity and inflammatory markers (ESR, CRP) at least once per trimester 1
• Assess blood pressure at each visit, as hypertension is common in Takayasu arteritis and can worsen during pregnancy 2, 3
• Perform complete blood counts monthly to monitor for azathioprine-related cytopenias 4
• Monitor for signs of disease flare including new vascular symptoms, constitutional symptoms, or rising inflammatory markers 2

Management of Disease Flare

• If disease flares during pregnancy, increase prednisone dose rather than adding new immunosuppressants 1
• Azathioprine dose can be optimized (up to 2 mg/kg/day) if needed for disease control 1
• Avoid adding methotrexate, mycophenolate, leflunomide, or cyclophosphamide, which are teratogenic 1

Critical Pitfalls to Avoid

Medication Discontinuation

• Do not discontinue azathioprine or prednisone due to pregnancy, as uncontrolled Takayasu arteritis poses greater risk than these medications 1, 2
• Stopping immunosuppression increases risk of disease flare, which can lead to vascular complications, hypertensive crisis, and adverse pregnancy outcomes 1, 2

Postpartum Management

• Anticipate increased risk of disease flare in the early postpartum period (first 3-6 months) 1
• Do not taper immunosuppression for at least 3 months postpartum 1
• Consider preemptively maintaining or slightly increasing immunosuppression immediately postpartum 1

Obstetric Considerations

• Ensure high-risk obstetric care with maternal-fetal medicine consultation 3
• Plan delivery at a tertiary center with capability to manage hypertensive emergencies and vascular complications 3
• Cesarean section may be preferred if there is significant aortic or iliac involvement affecting pelvic blood flow 3

Breastfeeding Compatibility

• All three medications (azathioprine, prednisone, low-dose aspirin) are compatible with breastfeeding 1
• Small amounts of azathioprine metabolites appear in breast milk but have not been associated with adverse infant outcomes 1
• Prednisone is considered compatible with breastfeeding 1
• Low-dose aspirin is safe during breastfeeding 1

Contraindicated Medications in Takayasu Arteritis Pregnancy

• Never use methotrexate, mycophenolate mofetil, leflunomide, or cyclophosphamide during pregnancy (all are teratogenic) 1, 5
• Avoid ACE inhibitors and ARBs if hypertension develops, as they cause fetal renal dysplasia 1, 6
• Use methyldopa, nifedipine, or labetalol for hypertension management instead 6