Systemic sclerosis in pregnancy

Abstract

Systemic sclerosis is a rare multisystem connective tissue disease. It predominantly affects women and poses a significant risk to mother and baby during pregnancy if not managed appropriately. The commonest manifestations are skin fibrosis and Raynaud's phenomenon. Subgroups of women have an increased risk of organ involvement, especially interstitial lung disease, pulmonary arterial hypertension and renal crises. Pregnancy increases the risk to the mother, especially those with established organ involvement, but also the development of new organ dysfunction; and risks to the fetus. Optimising these women prior to conception, along with careful management and surveillance during pregnancy, is vital for optimising pregnancy outcome. Women with scleroderma need to be managed in a specialised centre with coordinated care from the multi-disciplinary teams including physicians, obstetricians, anaesthetists, neonatologists and midwives. This review aims to describe the risks associated with systemic sclerosis and pregnancy, with management advice for physicians looking after pregnant women with this chronic condition.

Keywords: High-risk pregnancy; perinatal medicine; rheumatology; systemic sclerosis.

Figure 1.

Figure depicting screening required and considerations for managing pregnant women with SSc during different stages of pregnancy. Cr: creatinine; CK: creatinine kinase; Hb: haemoglobin; OGD: oesophago-gastroduodenoscopy; BP: blood pressure; GI: gastrointestinal; LDH: lactate dehydrogenase; MAHA: microangiopathic haemolytic anaemia; SRC: scleroderma renal crisis; ACE: angiotensin converting enzyme; SSc: systemic sclerosis; ECG: electrocardiogram; PAH: pulmonary arterial hypertension; ITU: intensive therapy unit.