Systemic sclerosis (SSc) is an acquired, sporadic, autoimmune, connective tissue disease with two subsets: limited cutaneous scleroderma (lcSSc) and diffuse cutaneous scleroderma (dcSSc). In the U.S., the annual incidence is about 20 cases per 1 million adults, with a prevalence of about 240 cases per 1 million adults.1 As with other connective tissue disorders, SSc…
A case report
Greater knowledge of how vasculitis, fibrosis, and autoimmunity interact in these destructive diseases will result in better clinical management
Understanding the role of autoantibodies in systemic sclerosis (SSc) may help predict the course of the disease and guide clinical trials
This disease presents a range of hard-to-solve clinical difficulties.
Advances in genetics and potential therapies shed new light on the disease
The U.S. Food & Drug Administration (FDA) approved nintedanib for systemic sclerosis associated interstitial lung disease (SSc-ILD) on Sept. 6 after a randomized, controlled trial (SENSCIS) demonstrated significant benefit against placebo.1 At a cost of $96,000 per year, treatment reduced the adjusted annual rate of change in forced vital capacity (FVC) from –93.3 mL in…
Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis. SRC occurs in 2–15% of patients with diffuse sclerosis and usually within the first five years from the time of diagnosis. Risk factors for SRC include, but are not limited to, early diagnosis, corticosteroid or cyclosporine use, and the presence of anti-RNA polymerase III…
CHICAGO—About 30 years ago, pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) began to outpace renal crisis as the main causes of death in scleroderma (SSc). But treating these lung complications has proved vexing for clinicians. There is no easy way to predict who will develop PAH. There is no telltale antibody and no…
A yearlong pilot study that evaluated the safety and efficacy of belimumab in a small group of patients with early diffuse systemic sclerosis found no significant difference in the number of adverse events between those treated with the drug and those who received a placebo. Currently, no drugs are approved specifically for the treatment of…