Pregnancy in Women Diagnosed with Antineutrophil Cytoplasmic Antibody-associated Vasculitis: Outcome for the Mother and the Child

Objective: Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is infrequently seen in women of childbearing age. Only a limited number of pregnancies in women with AAV have been reported, and often they were associated with complications.

Methods: This was a single-center retrospective observational study. All pregnancies in women with granulomatosis with polyangiitis (Wegener's) (n = 13) and microscopic polyangiitis (n = 1) were included. Women of childbearing age were counseled to abstain from pregnancy during or shortly after disease activity or <1 year after cyclophosphamide treatment.

Results: We described 22 pregnancies in 14 women with AAV (median age at diagnosis was 25 years [range 19-36 years]) diagnosed between 1982-2008. The ear, nose, and throat region (71%) and kidneys (50%) were predominantly involved. All women were in remission at conception and cyclophosphamide had been administered to 9 women (15 pregnancies). The median gestational age was 39+4 weeks, including 2 preterm deliveries. The median birth weight was 3,400 gm (1,860-3,890 gm). Hypothyroidism occurred in 1 newborn and a cleft palate in 1 newborn of a twin pregnancy. Otherwise, the fetal outcome was excellent. Preeclampsia was diagnosed in 2 pregnancies. A caesarean section was performed in 2 patients. The median followup after the last conception was 98 months (range 11-307 months). Eight women experienced a relapse 21 months (range 7-62 months) after conception, 1 during pregnancy, and 7 after delivery.

Conclusion: In this study, the pregnancy outcome in patients with AAV in remission was excellent. Pregnancy in women with AAV in remission does not seem to be associated with increased risk of relapse. Counseling, careful management, and close followup are essential in pregnant women with AAV.