progressive systemic scleroderma

Progressive systemic scleroderma, also known as systemic sclerosis, is a chronic autoimmune disease affecting the skin, blood vessels, and internal organs. It is characterized by the excessive deposition of collagen, a protein found in connective tissues, leading to hardening and thickening of affected areas.1

There are localized and systemic forms. Systemic scleroderma can be life-threatening and involves organs such as the lungs, heart, kidneys, and digestive tract. It can progress gradually or rapidly, with diffuse forms causing more severe organ complications. A common variant is limited cutaneous systemic sclerosis (lcSSc), or CREST syndrome, which includes calcinosis cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia.1

The exact cause is unknown, but it's believed to stem from a malfunctioning immune system attacking the body's own tissues, possibly triggered by infections or environmental factors in susceptible individuals. While there is no cure, treatments focus on managing symptoms.1