Background/Purpose: Autoimmune disease in pregnancy creates the potential for increased complications and poor pregnancy outcomes. There is a paucity of outcomes data in less-common autoimmune diseases.  We present the rare rheumatologic pregnancies managed over a decade from a university rheumatology pregnancy clinic.   

Methods: Data were prospectively collected over a 10-year period in a pregnancy registry for women with autoimmune disease at an academic medical center. Disease activity during pregnancy was measured by physician global assessment (PGA) score and classified as low, medium, or high. Pregnancy outcomes in each group were analyzed with simple statistics.  

Results: Data on 37 pregnancies in 28 patients with rare autoimmune diseases were analyzed, with an overall mean age of 30.2 years. Patient diagnoses included myositis (n=11), morphea (n=4), scleroderma (n=7) vasculitis (n=6), and sarcoidosis (n=2). There were 7 patients designated as “other autoimmune disease,” consisting of autoimmune hepatitis (n=2 in the same patient), gout, celiac disease, idiopathic optic neuritis (n=2) and orbital pseudotumor. The myositis group included 9 patients with dermatomyositis and 2 patients with polymositis. The vasculitis group consisted of Bechet’s (n=1), Buerger’s disease (n=1), eGPA (n=1), cutaneous PAN (2), and thrombotic vasculopathy (n=1). 

Disease activity during pregnancy overall was low, with only 1 patient with dermatomyositis and 3 patients in the other autoimmune disease category (n=2 autoimmune hepatitis and n=1 Celiac) having high disease activity. The mean gestational age across all groups was 37.4 weeks, ranging from 26 to 40 weeks, with only 6 total preterm births. There were 3 miscarriages (myositis, scleroderma, autoimmune hepatitis), 1 stillbirth (myositis) and 1 medically-indicated termination (myositis with severe lung disease).  

Among the 11 myositis pregnancies, 3 resulted in pregnancy losses (miscarriage, stillbirth, the above termination). The one patient with active disease resulted in a live, term birth. Eight patients (72.7%) in the myositis group took prednisone, and 5 (45.5%) azathioprine.  

Among the 6 vasculitis pregnancies, 4/6 (66.7%) patients took corticosteroids during pregnancy. Despite 2 patients having moderate disease activity, there was a 100% live birth rate and only 1 preterm delivery.  

Conclusion: Despite having autoimmune diseases with a number of potential complications, patients overall had low disease activity and good pregnancy outcomes. This data suggests that patients with rare autoimmune diseases who are carefully managed by a rheumatologist during pregnancy can have a successful pregnancy. 

Table 1. Pregnancy outcomes among women with rare autoimmune diseases (n=37).