Scleroderma is a rare and often fatal autoimmune disease affecting about 300,000 Americans. Scleroderma causes inflammation, hardening, and thickening of connective tissues within the body. There are two major types of disease: localized, which only affects the skin, and systemic, which affects other organs as well as the skin, causing more severe complications. There are many ways this disease can manifest, including loss of mobility, gastrointestinal tract and blood vessel dysfunction, kidney damage, and impaired lung capacity. There is no cure for the disease, and current treatments only help manage symptoms. Additionally, the disease affects each patient differently, which requires the need for individualized treatment plans.
The Garlick Lab collaborates with Celdara and the Whitfield lab to create patient specific skin tissues from scleroderma cells. To create these skin models, fibroblast and keratinocyte cells are isolated from patient biopsies, along with peripheral monocytes from blood. These three cell types are seeded into our in vitro skin tissue model, self-assembled skin equivalent (SaSE). We have previously shown that this model is able to replicate the scleroderma phenotype in thickness, inflammation, and hardness. This model allows us to better understand the disease and conduct various drug testing in vitro without endangering patients. In collaboration with the Whitfield lab we are conducting genetic analyses to determine a marker for scleroderma common among the various disease phenotypes. This marker can be used to facilitate future patient diagnosis and drug discovery.
Human Scleroderma Skin