This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Limited systemic sclerosis (also known as sine scleroderma) is a type of systemic scleroderma that is characterized by Raynaud’s phenomenon and the buildup of scar tissue (fibrosis) on one or more internal organs but not the skin. While the exact cause of limited systemic sclerosis is unknown, it is believed to originate from an autoimmune reaction which leads to the overproduction of collagen (a tough protein which normally strengthens and supports connective tissues throughout the body).
When fibrosis affects internal organs, it can lead to impairment or failure of the affected organs. The most commonly affected organs are the esophagus, heart, lungs, and kidneys. Internal organ involvement may be signaled by heartburn, difficulty swallowing (dysphagia), high blood pressure (hypertension), kidney problems, shortness of breath, diarrhea, or impairment of the muscle contractions that move food through the digestive tract (intestinal pseudo-obstruction). While there is no treatment that controls or stops the underlying problem (the overproduction of collagen), many of the symptoms of limited systemic sclerosis can be managed. Many people require a team of specialists to address their symptoms.
For more information, visit GARD.