Management of Rheumatologic Diseases in Pregnancy

Oier Ateka-Barrutia; Catherine Nelson-Piercy


Int J Clin Rheumatol. 2012;7(5):541-558. 

In This Article


Systemic vasculitides are rare and therefore data on pregnancy outcome are limited to a few case series and case reports. Overall, it seems that if women embark on pregnancy with vasculitis in remission their risk of complications is low, and therefore, pregnancy should ideally be planned after prolonged quiescent disease. Disease flares can occur at any stage of pregnancy and postpartum.

Erythrocyte sedimentation rate is not a reliable marker for disease activity monitoring during pregnancy, but C-reactive protein levels remain reliable.[68] Clinically, infections and vasculitis can mimic each other, and excluding infection is important for correct management, especially in patients on immunosuppressive drugs.[69] Vasculitis flare may also be difficult to distinguish from preeclampsia. An active urinary sediment and other systemic clinical manifestations usually point towards the former, whereas isolated proteinuria and the presence of characteristic clinical manifestations of preeclampsia (hypertension, headache, epigastric pain, edema and small baby) may indicate the latter.

Low-dose steroids (prednisone <7.5–10 mg) and azathioprine are the cornerstones of maintenance treatment during pregnancy. However, in severe flares or life-threatening situations, CYC should be considered as the drug of choice. As the latter is contraindicated during the first and early second trimesters, intravenous immunoglobulins (IVIG) may be a useful option to control the disease until CYC can be used.[69]

Among all forms of primary systemic vasculitis, Takayasu's arteritis deserves special attention as it is a disease characteristically diagnosed in women of child-bearing age. Eight case series published in the literature identified more than 145 women, with Japanese and Indian predominance, showing variable maternal and fetal outcomes.[70–77] Overall, Takayasu's arteritis does not seem to worsen during pregnancy, and the most important factor for poor outcomes seems to be the development of hypertension and related disorders, including preeclampsia. Peripheral BP monitoring may be challenging in these women, and BP checking in different extremities or invasive monitoring may be necessary. Patients with stenosis of the aorta and/or its principal branches should receive comprehensive cardiovascular assessment together with antenatal review by an obstetric anesthetist in order to plan the best mode of delivery and anesthesia.[69]

The impact of antineutrophil cytoplasmic antibody (ANCA)-related vasculitis on pregnancy outcomes will depend on the activity and long-term organ damage, with preeclampsia and prematurity being the most common complications.[69]

Behçet's syndrome can present with a broad range of clinical features, varying from mild mucocutaneous lesions to severe CNS involvement or thrombosis in both venous and arterial systems. This heterogeneity impacts on outcome data in and out of pregnancy.[78] At present, it is still unclear whether pregnancy influences the activity of Behçet's, although it seems that remissions are achieved in a large proportion, regardless of HLA-B51 serology profile.[79] However, flare rates between 25 and 65% were described in two case series, where the main manifestations were arthritis and mucocutaneous ulcerations.[80,81] With regard to pregnancy outcomes, the rate of miscarriage seems to be higher in these women, whereas the risk for other obstetric complications seems not to be increased.[79] The etiology and pathogenesis of thromboembolic events in patients with Behçet's is unclear. Pregnant women are theoretically at higher risk of these thromboembolic events. Close medical and obstetric monitoring are hence recommended throughout pregnancy and postpartum.