Katja Adriany

To study vasculopathy in systemic sclerosis

Systemic sclerosis is a rare connective tissue disease characterized by the triad of inflammation, vasculopathy and fibrosis. These pathophysiologic processes result in impaired quality of life and high mortality compared with other rheumatic diseases. Vasculopathic disease manifestations occur early in disease development and include Raynaud's phenomenon, acral ulcers, necrosis, and pulmonary arterial hypertension. Currently, acral vasculopathic features are assessed by capillaroscopy in clinical practice. However, capillaroscopy is limited to the nail fold and cannot assess the deep vascular plexus, changes in dynamic perfusion, or inflammatory and fibrotic disease manifestations. These limitations do not exist with the optical technique of dynamic optical coherence tomography (D-OCT). Therefore, in this project, we are characterizing vasculopathy in the skin of patients with systemic sclerosis using D-OCT. D-OCT is an imaging technique based on an infrared laser. D-OCT provides a simultaneous three-dimensional, high-resolution view of fibrotic and inflammatory changes as well as vasculopathy in SSc. In addition, D-OCT could be a useful tool to assess the progression of early systemic sclerosis. Currently, there are few therapeutic options to treat acral vasculopathy, which are also often associated with side effects. In this study, we investigate the changes in dynamic perfusion under three vasoactive therapy.