We decided to terminate recruit earlier because patient recruiting was not active as expected at the beginning of the study.

This study evaluates the efficacy and safety of routine medical treatments of digital ulcers in patients with systemic sclerosis in a prospective cohort study.

Systemic sclerosis (SSc) is a chronic autoimmune disease of unknown etiology with high morbidity and mortality. SSc manifests by fibrosis of skin and internal organs. Although the underlying mechanisms are still subject to investigation, endothelial dysfunction and abnormal immune response are thought to contribute to vascular dysfunction and fibrosis in SSc.

A hallmark feature of SSc is the presence of Raynaud phenomenon (RP) that is caused by reversible vasoconstriction of digital arteries triggered by exposure to cold or emotional stress. In up to 33-43% of SSc patients, the ischemia can progress to digital ulcers (DUs), which are often associated not only with pain but also with severe limitation in the daily activity, anxiety and depression among others. In severe cases, RP can be complicated by superinfection or gangrene, requiring surgical amputation.

The mainstay treatment of RP is restoration of the decreased blood flow using calcium channel blocker, protstanoids, phosphodiesterse V inhibitor, and endothelin receptor blocker. However, the efficacy and safety of those medications in RP-associated ulcers have not been definitive, especially in Korean patients with SSc. In addition, it remains unknown whether Korean SSc patients respond better to a treatment over others.

Study aims include

1. To establish a new prospective cohort of SSc patients with DU in Korea..
2. To investigate the current situation of SSc patients in Korea.
3. To investigate and compare the efficacy and safety of current medical treatments of DU in SSc patients in Korea

Inclusion Criteria:

• Age of 18 years or older
• SSc diagnosis according to 1980 or 2013 ACR classification criteria
• 1 or more active DU
• Patients who start or change medical treatment for DU
• Patients who are willing to participate

Exclusion Criteria:

• Pregnancy or active breast feeding
• Patients with life expectancy < 6 months

Patients with systemic sclerosis