What is Systemic Scleroderma?

C. K. Lanz

Systemic scleroderma is an autoimmune disorder characterized by widespread thickening, hardening, and tightening of the body’s connective tissues, skin, blood vessels, and internal organs. The heart, kidneys, and lungs can all be affected by systemic scleroderma in addition to the skin. This disease is progressive and rare, and over time the loss of flexibility can limit a patient’s ability to perform daily tasks. Self-esteem can also be negatively affected. This disorder can be inherited but is not believed to be contagious.

Sexual dysfunction in both men and women can result from systemic scleroderma.
Sexual dysfunction in both men and women can result from systemic scleroderma.

A patient with systemic scleroderma can present a varying set of symptoms depending on the organs affected. Some symptoms aren’t immediately associated with this disorder, making it hard to diagnose before eliminating other possibilities. Common symptoms include Raynaud’s phenomenon, gastroesophageal reflux disease, and obvious skin changes.

Skin changes are visible symptoms that can be caused by systemic scleroderma. Fingers and hands can swell, and the skin in these areas can become thick, tight, and red. The skin’s tightness can limit movement and create a shiny appearance. Scar tissue will also form on the affected internal organs.

Scarring of internal organs like the lungs, kidneys, and heart can cause myriad problems for patients with systemic scleroderma. Lung function can be reduced, and pulmonary hypertension can result. If the kidneys are affected, high blood pressure and kidney failure are possible complications. Arrhythmias, congestive heart failure, and pericarditis can all result if heart tissue is scarred.

Additional complications such as dental and sexual problems are also possible. Sexual dysfunction in men and women can result from systemic scleroderma. If facial skin becomes tight enough to restrict jaw and mouth movement, patients can find it difficult to eat or brush their teeth.

A doctor who suspects systemic scleroderma will often check a patient’s skin for thick patches and note any changes in joints and tendons or the presence of Raynaud’s. Blood tests to check for elevated levels of specific antibodies may also be ordered. A tissue sample taken from an affected area of skin can also help diagnose this disorder.

There is no cure for systemic scleroderma, but treatment options are available. A patient may begin a regimen of drugs that dilate blood vessels or suppress the immune system. Physical therapy can improve mobility and flexibility. In severe cases, a lung transplant or amputation of fingers affected by Raynaud’s may be necessary.

Many doctors believe that systemic scleroderma is the result of the body’s overproduction and storage of collagen. Collagen is a protein used to build connective tissues and skin. It is likely that the immune system is involved in triggering this overproduction of collagen, though there may be other factors such as race, gender, and environment.

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