When you cut yourself and start to heal, your body forms a matrix of tissue largely composed of collagen. It’s the basis of the scar tissue that forms where the cut was. For people with scleroderma, a rare auto-immune disease, their tissues continue developing even without an injury, resulting in the overproduction of collagen and other fibrous tissues, called fibroblasts, in the skin and internal organs.
People with scleroderma often experience pain and inflammation. As is the case with many autoimmune diseases, there aren’t any therapies that have been specifically developed to treat this condition. The scarcity of treatment options makes scleroderma an “orphan disease” – a special classification that the Food and Drug Administration (FDA) uses to incentivize drug-makers to develop new and innovative “orphan drugs” for the treatment of rare diseases.
Degenerative autoimmune diseases are often poorly understood, and such is the case with scleroderma. Medical scientists don’t really know what causes it, or how to treat it.
Two compounds that target the endocannabinoid system are currently in development to treat scleroderma – and they’re already in clinical trials. Scleroderma patients are hoping that these efforts will prove to be as successful as Epidiolex, the cannabis-derived CBD pharmaceutical approved by the FDA in 2018 as an orphan drug for two forms of severe pediatric epilepsy (Lennox-Gastaut Syndrome and Dravet Syndrome).
WHAT IS SCLERODERMA?
Degenerative autoimmune diseases are often poorly understood, and such is the case with scleroderma. Medical scientists don’t really know what causes it, or how to treat it. It’s theorized that both genetics and environmental factors can play a role in its development.
The earliest symptoms of scleroderma usually involve a thickening of the skin and a condition known as Reynaud’s phenomenon, which constricts the arteries and turns the fingers blue when they’re exposed to cold.
There are two primary types of scleroderma – localized scleroderma (morphea), which affects specific areas of the skin; and systemic scleroderma, a potentially fatal condition characterized by the thickening of tissues around organs. Systemic scleroderma can lead to excess collagen and scar tissue in the lungs (interstitial lung disease), resulting in the constriction and blockage of pulmonary arteries (pulmonary artery hypertension).