Systemic scleroderma is an autoimmune disease that affects the skin and internal organs. It is characterized by scar tissue (fibrosis) on the skin and other organs. Fibrosis is caused by the production of too much collagen in the body. Collagen normally strengthens and supports connective tissues. The signs and symptoms of systemic scleroderma usually begin with episodes of Raynaud’s phenomenon, which may occur weeks or years before fibrosis. The hands can swell and then the skin thickens and hardens. Fibrosis can also affect internal organs and can cause deterioration or failure of affected organs. The most commonly affected organs are the esophagus, the heart, the lungs, and the kidneys.
What Is The Life Expectancy For A Person With Scleroderma?
The life expectancy for a person with scleroderma depends on the involvement of major organs, especially lung and heart, as well as the existence of severe digital vascular lesions. If there is interstitial lung disease or pulmonary arterial hypertension, life expectancy is reduced. If there is no involvement of these organs or digital vasculitis, and the appropriate medical controls are followed, a good life expectancy is expected.
Survival is determined by the subtype of the disease and the internal manifestations of the organ. The annual proactive and routine examination allows for early intervention with disease-modifying medications. These have led to an improvement in the forecast and long-term results in recent years.
The majority of deaths among patients with systemic sclerosis are related to pulmonary fibrosis, pulmonary arterial hypertension, or heart disease. Other important causes of death include kidney disease, cancer, gastrointestinal and infectious.
The risk factors are extensive involvement of the skin, the involvement of the heart, and/or lung, kidney disease and the presence of antibodies antitiropesomerasa I and/or anti-Th. When the disease begins at a younger age, the prognosis is worse. People with the diffuse cutaneous systemic scleroderma type have a worse life expectancy than the limited cutaneous type.
There are three types of systemic scleroderma, defined by the affected tissues in the disorder.
-Systemic Diffuse Cutaneous Sclerosis affecting the hands, forearms, upper arms, thighs or trunk. It can affect the face.
-Limited Systemic Cutaneous Sclerosis where thickening of the skin occurs only on the hands and forearms, feet and lower legs. It can affect the face.
-Limited Systemic Sclerosis is a rare modality (approximately 5% of all cases) and is characterized by affecting the internal organs without causing a thickening of the skin.
Treatment depends on the symptoms that are present and the organs that are affected by the disease and may include immunosuppressive therapy.
What Is The Cause Of Scleroderma?
The exact cause of systemic sclerosis is unknown. It is thought to be caused by an injury to the cells lining the blood vessels that result in excessive activation of skin connective tissue cells called fibroblasts. Fibroblasts usually produce collagen and other proteins. The accumulation of collagen in the skin and other organs causes the signs and symptoms of the condition.
It is suspected that the following factors may be important for the development of scleroderma.
-Abnormal immune or inflammatory activity
-Genetic Susceptibility: Although specific genes are not believed to cause scleroderma, certain genes (or combinations of genes) may increase a person’s risk of being affected. These genes include several genes of the human leukocyte antigen (HLA) complex, and also genes not related to HLA, such as the genes TNFAIP3, CD247, IRF5, STAT4, and PTPN22. However, while the risk of being affected by the disease increases when there is an affected relative, the condition is not transmitted directly from parents to children.
-Environmental Triggers: Certain infections, injuries, medications (eg, vitamin K, cocaine, penicillamine, appetite suppressants and some chemotherapeutic agents) and chemicals eg, silica, organic solvents, pesticides, aliphatic hydrocarbons and epoxy resin can trigger the disease.
-Hormones: Because women develop scleroderma more often than men, researchers suspect that hormones may play a role. However, the role of female hormones has not been proven.
Generalized scleroderma can also occur in association with other autoimmune diseases, such as systemic lupus erythematosus and polymyositis.