Systemic sclerosis is a connective tissue disorder characterized by overactive fibroblasts, cells that produce fibrous materials like collagen in the body. People with systemic sclerosis develop deposits of collagen in their connective tissues that lead to a variety of medical problems. This condition involves both the skin and internal organs and it cannot be cured, although the symptoms can be managed with medications and other treatments.
This condition is also known as systemic scleroderma. In contrast with localized sclerosis, which only involves the skin, this is a multi-system connective tissue disorder. It is more common in women than men and can appear in people of any racial background. The causes are not fully understood but are believed to be linked to changes to the immune system. Systemic sclerosis is chronic and it is very rare.
Skin changes are often the first sign of disease. Patients can develop discolored, shiny, rough, patchy skin. Over time, the skin tightens and people may develop contractures and limited range of movement because of the skin tightening. Fragile nails and skin ulcerations are also common. Raynaud's phenomenon, where the blood vessels clamp down in response to cold and limit circulation in the hands, is another symptom of systemic sclerosis. These changes often lead patients to a dermatologist, who can examine the patient and provide a referral to other specialists for a diagnosis.
Internally, systemic scleroderma can cause damage to the gastrointestinal tract, liver, kidneys, and other internal organs. Gastroesophageal reflux is a common symptom and patients can also experience impaired liver and kidney function. Medical imaging studies can be used to identify abnormalities in the organs and blood tests can be used to check for autoantibodies, which are antibodies developed by the immune system that target the body itself. Because other conditions can be linked with skin hardening and other symptoms associated with systemic sclerosis, it is important to undergo testing to confirm the diagnosis.
Treatments include skin care to soften the skin and ease inflammation, along with changes to exercise and dietary patterns designed to limit organ damage and keep patients more comfortable. Some patients benefit from physical therapy and other types of available treatments. Because this condition manifests very differently in different patients, treatment plans usually need to be tailored to the individual in order to address specific sets of symptoms. Patients will need treatment for life, including monitoring for signs of complications because the disease can be progressive in nature.