By Carol A Langford, MD, MHS
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A 34-year-old female you have followed for granulomatosis with polyangiitis (GPA) comes to your clinic to discuss whether she is able to pursue a pregnancy. She was diagnosed with GPA at age 30 and presented with features that included sinus disease, pulmonary nodules, arthralgias, glomerulonephritis with a peak creatinine of 3.0 mg/dL, and a positive PR3-cANCA. For this she was treated with prednisone and rituximab on which her creatinine improved to a baseline of 1.7 mg/dL. She has remained in remission since that time on her current medications of azathioprine 150 mg daily, lisinopril 20 mg daily, and an oral contraceptive. What are the key discussion points you need to have with your patient?
Vasculitis and Pregnancy
With the evolution of management options, pregnancy has become a feasible and realistic goal for many women with vasculitis. Most forms of vasculitis can impact women capable of having a child but prominently include Takayasu arteritis and ANCA-associated vasculitis.1-3 The published literature on pregnancies in vasculitis remains small and often encompasses all of these diseases, although there are some unique differences.3
For young women with vasculitis, it is important to begin the discussion about the potential for pregnancy early in their disease course. This is not only related to the teratogenicity of some medications but also because careful planning and counseling prior to pursuing conception provides a woman and her child with the greatest potential for a safe outcome. When a woman begins to plan for a future pregnancy there are several key issues that should be discussed:
- Pregnancy Timing. The recency of active vasculitis and the overall disease course should be examined in evaluating the timing and advisability of pregnancy. As some series have found improved outcomes with disease control prior to pregnancy, achieving a stable remission before pregnancy is strongly recommended.
- Organ Damage. Underlying permanent organ or blood vessel damage is one of the most important factors to be weighed in the safety of pregnancy for a woman with vasculitis. Renal insufficiency can occur as a consequence of many forms of vasculitis. During pregnancy, there is the potential for worsening renal function, pre-eclampsia or eclampsia. Women with renal disease should be seen by a nephrologist for pre-conception counseling as well as for monitoring during the pregnancy. Decreased diaphragmatic excursion during pregnancy can impact those with severe lung disease. For women with Takayasu arteritis the location of large vessel involvement and how this would be impacted by a pregnancy must be examined. The abdominal aorta and its branches play a very significant role during pregnancy, both in terms of providing placental circulation to the fetus and the potential for further reduction in blood flow to the mother through these vessels from the gravid uterus. As pregnancy can impact blood pressure and blood flow, it must be carefully evaluated how this would impact women with aortic aneurysms or stenotic vessels providing brain perfusion. New or worsening hypertension is a significant concern during pregnancy. It is believed to impact outcome in pregnant women with Takayasu arteritis but can factor into the pregnancy course of all forms of vasculitis.
- Medications. As most forms of vasculitis carry the potential for relapse, whether to continue remission maintenance medication during pregnancy must be considered. Factoring into this is that a number of medications that are used in vasculitis cannot be taken during pregnancy. The American College of Rheumatology recently published guidelines for the management of reproductive health, which includes valuable information applicable to medication decision-making for pregnant women with vasculitis.4
- Potential for Relapse. It is unclear how or if a pregnancy impacts the risk for a vasculitis disease relapse. The potential that a relapse could occur during pregnancy should be openly discussed prior to conception. Individual factors that should be examined include if the patient has had prior relapses, how these have manifest, and what therapies they have needed to control these. Should a relapse occur during a pregnancy, treatment considerations would depend on multiple factors including the type of vasculitis, severity of the relapse, and pregnancy trimester in which the relapse was occurring.
- Pregnancy Management. It has been my recommendation that all women with vasculitis who are considering a pregnancy receive preconception counselling from a high-risk obstetrician and have such a specialist involved during their pregnancy. While it is the hope that high-risk skills will not always be needed, patients with vasculitis can present with complex and unpredictable medical issues. In addition to their vasculitis care provider, the need for other specialists to play an active role during pregnancy will be based on patterns of organ or vessel involvement. The method of delivery would be individually determined. It is not mandatory for all women with vasculitis to undergo a cesarean section, although this may provide advantages for women where a controlled delivery optimizing management of blood pressure is desirable.
Return to the Case Patient
Each of these issues was carefully discussed with the patient. As she was in a stable remission, it was an appropriate time for her to consider a pregnancy. The main feature of concern was her renal insufficiency for which she was seen prior to conception by nephrology. In addition to discussion about the potential impact of pregnancy on her kidney function, the lisinopril was stopped — as this cannot be taken during pregnancy — and replaced by labetalol with home blood pressure monitoring. She was also seen by high-risk obstetrics. After counseling, the patient chose to pursue conception and remain on azathioprine during her pregnancy.
As more women with vasculitis are choosing to pursue pregnancies, it is critical for us to gain a greater understanding of their experiences. The Vasculitis Pregnancy Registry (V-PREG) that is being conducted through the Vasculitis Patient-Powered Research Network is an important initiative that will provide valuable prospective information on how vasculitis impacts reproductive health and pregnancy outcomes. Further information on the registry and how your patients can participate can be found at (https://www.vpprn.org/vpreg).
- Machen L, Clowse ME. Vasculitis and pregnancy. Rheum Dis Clin North Am. 2017;43(2):239–247.
- Fredi M, Lazzaroni MG, Tani C, et al. Systemic Vasculitis and pregnancy: A multicenter study on maternal and neonatal outcomes of 65 prospectively followed pregnancies. Autoimmun Rev. 2015;14(8):686–691.
- Comarmond C, Mirault T, Biard L, et al. Takayasu arteritis and pregnancy. Arthritis Rheumatol. 2015;67(12):3262–3269.
- Sammaritano LR, Bermis BL, Chakravarty EE, et al. 2020 American College of Rheumatology guideline for the management of reproductive health in rheumatic and musculoskeletal diseases. Arthritis and Rheumatol. 2020; 72(4):529–556.