Pregnant Women with ANCA-associated Vasculitis More Prone to Complications, Review Shows

Women who develop ANCA-associated vasculitis while pregnant are more likely to suffer maternal and fetal complications, according to a systematic literature review. To ensure the best possible outcome, investigators recommend these patients be closely monitored by a multidisciplinary team of healthcare providers.

The systematic review, “De novo antineutrophil cytoplasmic antibody-associated vasculitis in pregnancy: a systematic review on maternal, pregnancy and fetal outcomes,” was published in the Clinical Kidney Journal.

Anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis is an autoimmune disease caused by the production of autoantibodies (antibodies that target and attack healthy cells in the body by mistake), leading to blood vessel inflammation and swelling in several tissues and organs.

Although most patients start to experience their first symptoms only after the age of 40, ANCA-associated vasculitis (AAV) also can occur during pregnancy.

Unfortunately, conventional immunosuppressive therapies for AAV are potentially dangerous to the fetus, and the safety and effectiveness of new treatments, including intravenous immunoglobulin (IVIG) and Rituxan (rituximab), are still lacking validation.

Moreover, definite data on pregnancy outcomes of women affected by AAV is scarce, which translates into poor guidance from clinicians regarding the optimal safe treatment for these patients.

In this systematic literature review, researchers gathered data from studies performed on women who developed AAV while pregnant, and evaluated clinical presentation, disease management strategies, and maternal and fetal outcomes.

The review included 27 cases of AAV pregnant women — between five and 39 weeks of gestation — with the majority being diagnosed in the second trimester of pregnancy (median of 20 weeks).

Most women were prescribed steroids (89%), followed by cyclophosphamide (37%), and other immunosuppressive agents, such as azathioprine, IVIG and plasma exchange (PLEX), as a treatment for AAV; 11% received no therapy.

Pooled data revealed a relatively high incidence of serious complications, including preeclampsia (high blood pressure) during pregnancy (29%) and maternal death (7%). Although most pregnancies led to a live birth (73%), most births occurred prematurely, before 37 weeks of gestation (73%).

Abortion rates also were relatively high (23%) and only one spontaneous death in the womb occurred shortly after therapy initiation (4%). Conversely, fetal congenital abnormalities were rare (6%), with only one infant suffering from solitary kidney — a medical condition in which a person has only one functional kidney — after the mother had been treated with steroids, cyclophosphamide and PLEX.

These findings indicate that gestation in women affected by AAV is linked to a higher incidence of pregnancy complications. To minimize this issue and improve pregnancy outcomes, investigators recommend the adoption of a multidisciplinary approach by clinicians and patients alike.

“This review highlights the need for practitioners to continue reporting cases of de novo AAV in pregnancy, especially those treated with rituximab. In addition, publishing cases where serious maternal complications or death occur is important in order to identify women with high-risk features wherein termination should be strongly recommended. When managing these patients, a multidisciplinary team is required to counsel patients and to optimize outcomes given the complexity of medical issues in this population,” the researchers concluded.