LONG-TERM POSTPARTUM FOLLOW-UP OF UNDIFFERENTIATED CONNECTIVE TISSUE DISEASES NEWLY DIAGNOSED DURING PREGNANCY

Background: Undifferentiated connective tissue disease (UCTD) is a group of systemic autoimmune conditions not fulfilling the classification criteria for a definite connective tissue disease (CTD). While an average of 20% of UCTD patients develop a defined CTD during follow-up, the remaining patients maintain an undefined disease. Pregnancy is considered to be an important risk factor that may alter the course of autoimmune diseases. Objective: The objective was to assess flare or differentiation into well-defined CTD of newly diagnosed UCTD and to compare these findings with the epidemiological data regarding the population of non-pregnant women with UCTD. Patients and methods: We examined 51 patients with UCTD newly diagnosed during pregnancy. Patients were followed during pregnancy and after delivery at our rheumatologic unit. Diagnosis of UCTD in these women was made at the time of pregnancy. Women in their first trimester (T0) were screened using a two-step approach using a self-administered 10-item questionnaire, a subsequent testing for rheumatic autoantibodies (ANA, anti-dsDNA, ENA, anticardiolipin antibodies, anti-β2-glycoprotein I antibodies and lupus anticoagulant) and evaluation by a rheumatologist, who made the final diagnosis. The same questionnaire was re-administered at least one year after delivery (T1) and the same rheumatic autoantibodies were tested at the same time. Results: We examined 51 women with newly diagnosed UCTD during pregnancy. Among these patients 13 (25,49%) had a second pregnancy during the time between T0 and T1. Three were twin pregnancies. We observed some obstetrical complications: 3 miscarriages, 3 fetal loss, 3 hypertensive disorders, 4 intrauterine growth restriction, 9 preterm deliveries and 8 small for gestational age babies. The mean number of positive items of the questionnaire at the screening was 3, while at T1 it was 4. All the patients showed at least one positivity for autoantibodies at screening, while 12 of them had negative results at T1. Almost 65%(33/51) of patients showed worsening of symptoms and diagnosis of UCTD was confirmed in 26/51 patients (50%). Thirty-two% (16/51) of women received a final diagnosis of CTD: 12 patients with Systemic Lupus Erithematosus (23,5%), 2 (4%) Antiphospholipid syndrome, 1(2%) spondyloarthritis and 1(2%) Behçet disease. Amelioration of symptoms with a resulting unconfirmed diagnosis was seen among 9/51 (18%) of them. Among women with confirmed diagnosis, treatment was started in 31 patients: hydroxychloroquine in 18 cases, corticosteroids in 13 cases, 2 methotrexate, 1 adalimumab and 1 cardioaspirin. Conclusion: Pregnancy appears to be a risk factor for flare up or evolution into well-defined CTD, mostly in UCTD newly diagnosed during pregnancy.