Antisynthetase syndrome in pregnancy: a case report and review of the literature

Abstract

Aim: Antisynthetase syndrome (anti-SS) is a rare systemic autoimmune disease characterised by autoantibodies against aminoacyl-tRNA synthetases manifesting as one or more components of the classic triad: interstitial lung disease (ILD), arthritis and myositis. Whilst it is well-recognised that autoimmune rheumatological disorders can contribute to multiple pregnancy complications, little is known about how anti-SS itself affects pregnancy outcomes. This report aims to identify pregnancy complications associated with anti-SS and their targeted management.

Method: Described is the case of a 26-year-old pregnant woman with anti-SS who had a complicated pregnancy on a background of multiple prior miscarriages. Her pregnancy was complicated by a disease flare at 23 weeks' gestation, placental dysfunction identified on obstetric ultrasound at 24+4 weeks' gestation, and subsequent extremely premature delivery at 24+6 weeks' gestation. Her placenta demonstrated features consistent with maternal vascular malperfusion (MVM) and borderline massive perivillous fibrin deposition (MPVFD) with a normal karyotype. A review of all published cases of anti-SS syndrome in pregnancy was performed. Their outcomes were compared and factors contributing to pregnancy complications, as well as potential management strategies, were identified.

Result: Disease activity and the presence of ILD, anti-Ro/SSA and anti-La/SSB antibodies and antiphospholipid syndrome were identified as key factors leading to poor pregnancy outcomes. Additionally, it was hypothesised that autoimmunity and inflammation in anti-SS may contribute to placental dysfunction in the form of MVM and MPVFD which can lead to miscarriage, stillbirth, intrauterine growth restriction and prematurity.

Conclusion: Overall, this case highlights the complexity of managing anti-SS in pregnancy, and explores potential pregnancy complications associated with anti-SS and their management. These include pre-pregnancy counselling and optimisation of disease control prior to conception, regular monitoring for both maternal and fetal complications, specific medications such as low-dose aspirin and low-molecular-weight heparin to reduce the risk of placental dysfunction, and a multidisciplinary team approach.