Systemic Sclerosis: What Is It?
Systemic sclerosis, shortened to SS, is an autoimmune condition. The disorder makes the body attack itself and healthy tissue is destroyed by the immune system, which believes it is a virus or a foreign substance. There are different types of autoimmune systems and each will have difference affects on the body.
SS causes the appearance and texture of the skin to change because the collagen production increases. The collagen is a connective tissue component. The disorder will affect other areas as well as the skin, including the muscles, the blood vessels and some organs (kidney, lungs, digestive system and heart). It is possible for SS features to appear within other disorders that affect the immune system, which is when they are called mixed connective disorders.
Individuals between 30 and 50 are more likely to suffer from this disease but any age can be affected. Men are four times less likely to suffer from it than women. Depending on the organs and systems involved, the severity and symptoms differ considerably between patients.
Other names for SS include progressive systemic sclerosis, CREST or scleroderma. When the name CREST is given, it is a limited form.
Systemic Sclerosis Symptoms
In the early stages, the skin is usually affected by thickening. This is often around the nose, mouth, fingers and the bony parts of the body and makes the skin look shiny and tight.
When it starts to progress, other symptoms are noticed, including:
- Loss of hair
- Joint pain
- Calcium deposits (white lumps) underneath the skin – usually in CREST syndrome
- Blood vessels dilate under the surface of the skin
- Short breaths
- Dry coughing
- Trouble swallowing
- Bloating in the abdomen after eating
- Esophageal reflux
The most characteristic feature of SS in the skin is within the blood vessels. There will be spasms of them within the toes and fingers. The tips can turn white or blue while cold or during a time of emotional stress. This is known as Raynaud’s phenomenon.
Systemic Sclerosis Risk Factors
The cause is currently unknown but there are some risk factors for SS and certain people more likely to develop it. These include:
- Being female (four times more than men)
- Being African American or Native American
- Being exposed to organic solvents and silica dust
- Using certain chemotherapy treatments, including Bleomycin
No known methods have been developed to prevent it except to reduce the risk factors where possible.
Diagnosing Systemic Sclerosis
Skin changes will show up during a physical exam. To see that there are kidney changes, the blood pressure will be taken. There will also be blood tests, including the antibody testing and the doctor will check the sedimentation rate and the rheumatoid factor. There are other tests used for diagnosis SS, including:
- Blood tests
- X-ray of the chest
- CT scan of the lungs
- Biopsies of the skin
Depending on the symptoms, there may be other tests involved.
Systemic Sclerosis Treatment
There are different treatments depending on the symptoms and possible complications. Some of the general treatments include:
- Nonsteroidal anti-inflammatory medications
- Immunosuppressants, such as cytoxan or methotrexate
Treatment can also be for the individual symptoms,such as:
- Medications to improve breathing
- Medication for the blood pressure
- Light therapy (phototherapy UV1)
- Physical therapy
- Nitroglycerin ointment for the skin tightening
Your doctor may also recommend the following changes to your lifestyle:
- Doing plenty of exercise
- Quit/avoid smoking
- Avoiding foods that are linked to heartburn
Systemic Sclerosis Complications
For some, the symptoms of SS can progress in later life. The possible complications include kidney failure, heart failure, high blood pressure and cancer. It can cause death and the National Institutes of Health states that this is usually due to scarring of the lungs or pulmonary fibrosis.