Systemic sclerosis

Abstract

Systemic sclerosis (SSc) is characterized by a non-inflammatory vasculopathy as well as fibrosis of the skin and vital organs. It presents in two distinct subtypes depending on the extent of cutaneous involvement, with each subtype (diffuse vs limited SSc) having different relative prevalence of extra-cutaneous manifestations. Experience describing pregnancy outcomes in SSc is limited because of disease onset mainly in the 4th and 5th decade. Common symptoms, including cutaneous fibrosis and Raynaud’s phenomenon, are not worsened, and may even improve during pregnancy. Severe organ manifestations of SSc, including pulmonary fibrosis, scleroderma renal crisis, and pulmonary arterial hypertension, are associated with increased risks of pregnancy complications and can be more difficult to treat during pregnancy. Therapies for SSc are mostly directed at managing symptoms with vasodilators, angiotensin-renin antagonists, proton pump inhibitors, and immunosuppressives in the case of pulmonary fibrosis.