Scleroderma
Systemic sclerosis (SS) or Scleroderma is an autoimmune disorder. This means it is a condition in which the immune system attacks the body. Healthy tissue is destroyed because the immune system mistakenly thinks it is a foreign substance or infection. There are many kinds of autoimmune disorders that can affect different body systems.
SS is characterized by changes in the texture and appearance of the skin. This is due to increased collagen production. Collagen is a component of connective tissue.
But the disorder is not confined to skin changes. It can affect your:
- blood vessels
- muscles
- heart
- digestive system
- lungs
- kidneys
Features of systemic sclerosis can appear in other autoimmune disorders. When this occurs, it is called a mixed connective disorder.
The disease is typically seen in people 30 to 50 years old, but it can be diagnosed at any age. Women are more likely than men to be diagnosed with this condition. The symptoms and severity of the condition vary from one person to another based on the systems and organs involved.
Systemic sclerosis or scleroderma, is also called progressive systemic sclerosis, or CREST syndrome. “CREST” stands for:
- calcinosis
- Raynaud’s phenomenon
- esophageal dysmotility
- sclerodactyly
- telangiectasia
CREST syndrome is a limited form of the disorder.
Key Facts
- CREST syndrome is a limited form of scleroderma.
- Patients with scleroderma can have specific antibodies (ANA, anticentromere, or anti-topoisomerase) in their blood that suggest autoimmunity.
- Treatment of scleroderma is primarily directed toward the particular individual’s symptoms.
Symptoms
SS may only affect the skin in the early stages of the disease. You may notice your skin thickening and shiny areas developing around your mouth, nose, fingers, and other bony areas.
As the condition progresses, you may begin start to have limited movement of the affected areas. Other symptoms include:
- hair loss
- calcium deposits, or white lumps under the skin
- small, dilated blood vessels under the skin’s surface
- joint pain
- shortness of breath
- a dry cough
- diarrhea
- constipation
- difficulty swallowing
- esophageal reflux
- abdominal bloating after meals
You may begin to experience spasms of the blood vessels in your fingers and toes. Then, your extremities may turn white and blue when you’re in the cold or feeling extreme emotional stress. This is called Raynaud’s phenomenon.
Causes
The cause of scleroderma is not known. Researchers have found some evidence that certain genes are important hereditary factors, but the environment seems to also play a role. The result is activation of the immune system in a susceptible individual, causing damage to the inner lining of tiny blood vessels and injury to tissues that result in scar tissue formation and the accumulation of excess collagen.
The fact that genes seem to cause a predisposition to developing scleroderma means that inheritance at least plays a partial role. It is not unusual to find other autoimmune diseases in families of scleroderma patients.
Treatment cannot cure the condition, but it can help reduce symptoms and slow disease progression. Treatment is typically based on a person’s symptoms and the need to prevent complications.
Treatment for generalized symptoms may involve:
- corticosteroids
- immunosuppressants, such as methotrexate or Cytoxan
- nonsteroidal anti-inflammatory drugs
Depending on your symptoms, treatment can also include:
- blood pressure medication
- medication to aid breathing
- physical therapy
- light therapy, such as ultraviolet A1 phototherapy
- nitroglycerin ointment to treat localized areas of tightening of the skin
You can make lifestyle changes to stay healthy with scleroderma, such as avoiding smoking cigarettes, remaining physically active, and avoiding foods that trigger heartburn.
Treatments for scleroderma have drastically improved in the past 30 years. Although there is still no cure for scleroderma, there are many different treatments that can help you manage your symptoms. Talk to your doctor if any of your symptoms is getting in the way of your daily life. They can work with you to adjust your treatment plan.
You should also ask your doctor to help you find local support groups for SS. Talking to other people who have similar experiences as you can make it easier to cope with a chronic condition.
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Scleroderma
Systemic sclerosis (SS) or Scleroderma is an autoimmune disorder. This means it is a condition in which the immune system attacks the body. Healthy tissue is destroyed because the immune system mistakenly thinks it is a foreign substance or infection. There are many kinds of autoimmune disorders that can affect different body systems.
SS is characterized by changes in the texture and appearance of the skin. This is due to increased collagen production. Collagen is a component of connective tissue.
But the disorder is not confined to skin changes. It can affect your:
- blood vessels
- muscles
- heart
- digestive system
- lungs
- kidneys
Features of systemic sclerosis can appear in other autoimmune disorders. When this occurs, it is called a mixed connective disorder.
The disease is typically seen in people 30 to 50 years old, but it can be diagnosed at any age. Women are more likely than men to be diagnosed with this condition. The symptoms and severity of the condition vary from one person to another based on the systems and organs involved.
Systemic sclerosis or scleroderma, is also called progressive systemic sclerosis, or CREST syndrome. “CREST” stands for:
- calcinosis
- Raynaud’s phenomenon
- esophageal dysmotility
- sclerodactyly
- telangiectasia
CREST syndrome is a limited form of the disorder.
Key Facts
- CREST syndrome is a limited form of scleroderma.
- Patients with scleroderma can have specific antibodies (ANA, anticentromere, or anti-topoisomerase) in their blood that suggest autoimmunity.
- Treatment of scleroderma is primarily directed toward the particular individual’s symptoms.
Symptoms
SS may only affect the skin in the early stages of the disease. You may notice your skin thickening and shiny areas developing around your mouth, nose, fingers, and other bony areas.
As the condition progresses, you may begin start to have limited movement of the affected areas. Other symptoms include:
- hair loss
- calcium deposits, or white lumps under the skin
- small, dilated blood vessels under the skin’s surface
- joint pain
- shortness of breath
- a dry cough
- diarrhea
- constipation
- difficulty swallowing
- esophageal reflux
- abdominal bloating after meals
You may begin to experience spasms of the blood vessels in your fingers and toes. Then, your extremities may turn white and blue when you’re in the cold or feeling extreme emotional stress. This is called Raynaud’s phenomenon.
Causes
The cause of scleroderma is not known. Researchers have found some evidence that certain genes are important hereditary factors, but the environment seems to also play a role. The result is activation of the immune system in a susceptible individual, causing damage to the inner lining of tiny blood vessels and injury to tissues that result in scar tissue formation and the accumulation of excess collagen.
The fact that genes seem to cause a predisposition to developing scleroderma means that inheritance at least plays a partial role. It is not unusual to find other autoimmune diseases in families of scleroderma patients.
Treatment cannot cure the condition, but it can help reduce symptoms and slow disease progression. Treatment is typically based on a person’s symptoms and the need to prevent complications.
Treatment for generalized symptoms may involve:
- corticosteroids
- immunosuppressants, such as methotrexate or Cytoxan
- nonsteroidal anti-inflammatory drugs
Depending on your symptoms, treatment can also include:
- blood pressure medication
- medication to aid breathing
- physical therapy
- light therapy, such as ultraviolet A1 phototherapy
- nitroglycerin ointment to treat localized areas of tightening of the skin
You can make lifestyle changes to stay healthy with scleroderma, such as avoiding smoking cigarettes, remaining physically active, and avoiding foods that trigger heartburn.
Treatments for scleroderma have drastically improved in the past 30 years. Although there is still no cure for scleroderma, there are many different treatments that can help you manage your symptoms. Talk to your doctor if any of your symptoms is getting in the way of your daily life. They can work with you to adjust your treatment plan.
You should also ask your doctor to help you find local support groups for SS. Talking to other people who have similar experiences as you can make it easier to cope with a chronic condition.
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