What are the other Names for this Condition? (Also known as/Synonyms)

• CREST Syndrome
• lcSSc (Limited Cutaneous Form of Systemic Sclerosis)
• Systemic Scleroderma, Limited Type

What is Limited Systemic Scleroderma? (Definition/Background Information)

• Limited Systemic Scleroderma is a subtype of Scleroderma that affects a variety of organs, such as the gastrointestinal tract, heart, muscles, and joints, potentially causing severe damage
• Scleroderma is a chronic connective tissue disorder of unknown cause. Connective tissues give support to the organs and skin. Individuals with Scleroderma have thick or hard connective tissue

There is currently a debate about how Scleroderma should be classified. A majority believe that it should be classified as an autoimmune rheumatologic disorder. Due to this, many researchers believe that the damage is caused by immune cells mistakenly attacking one’s own healthy tissue.

Scleroderma can be classified into 2 broad categories, which include Localized Scleroderma and Systemic Scleroderma:

• Localized Scleroderma:
  • It often affects the skin tissues and does not cause harm to the other major organs
  • There are two types of Localized Scleroderma, namely Morphea and Localized Linear Scleroderma
• Systemic Scleroderma:
  • It affects the skin, tissue beneath the skin, major organs, and blood vessels
  • Systemic Scleroderma also has 2 different types, namely Limited Systemic Scleroderma and Diffuse Systemic Scleroderma

Limited Systemic Scleroderma is a type of Systemic Scleroderma of unknown cause.

• Commonly, the organs involved include the esophagus (food pipe), stomach, small intestines, large intestines, heart, muscles, joints, and kidneys. However, it may take years for hardening of the tissue to occur, which in turn, can cause a much slower progression of the signs and symptoms
• Typically, Diffuse Systemic Scleroderma progresses rapidly, resulting in severe signs and symptoms and even life-threatening complications. With Limited Systemic Scleroderma, there is no rapid progression of the condition
• Limited Systemic Scleroderma is also called as CREST Syndrome. The term CREST stands for:
  • Calcinosis: Calcium deposits on the skin
  • Raynaud’s phenomenon: Blood vessel spasms in response to cold temperatures or stress
  • Esophageal dysfunction: Acid reflux and less motility in the esophagus
  • Sclerodactyly: Thickening and tightening of the skin of the fingers and toes
  • Telangiectasias: Red capillary dilation on the skin of the face and chest
• There is currently no cure for Limited Systemic Scleroderma, but medications, therapy, and surgery may be used to control symptoms and prevent complications. The treatment measures are dependent upon the tissues or organs affected
• The way Limited Systemic Scleroderma changes and progresses over time vary among individuals. The rapidity of progression of signs and symptoms and the organs involved affects the prognosis. Typically, individuals who respond to treatment have a better prognosis, than individuals who do not respond to treatment

Who gets Limited Systemic Scleroderma? (Age and Sex Distribution)

• Individuals of any age can be affected by Scleroderma; however, this connective tissue disorder usually occurs from ages 25 to 55 years
• A majority of Systemic Scleroderma cases often occur in individuals between the age of 30 and 50 years, although even children may be affected
• Like Scleroderma, it is thought that Limited Systemic Scleroderma occurs more often in females
• Scleroderma is known to occur worldwide, in all races and ethnic groups. Systemic Scleroderma is more common in Native Americans and African Americans than individuals of European decent

What are the Risk Factors for Limited Systemic Scleroderma? (Predisposing Factors)

The risk factors for Limited Systemic Scleroderma may include:

• Gender: Females are more likely than males to have Limited Systemic Scleroderma
• Native Americans and African Americans are more susceptible to Systemic Scleroderma than individuals of European decent
• Some studies have shown that individuals who have worked with or been exposed to silica dust and polyvinyl chloride (PVC) may have an increased incidence of the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Limited Systemic Scleroderma? (Etiology)

The exact cause of Limited Systemic Scleroderma is presently unknown. Research is being currently performed, to identify the relevant causal factors.

• Many researchers believe that Scleroderma is an autoimmune disorder, where the body’s immune system mistakenly attacks healthy tissues resulting in damage of the tissues and organs
• Some studies have revealed that there may be a higher risk for the condition, due to occupational exposure to chemicals such as silica dust and PVC

However, research has established the following on Limited Systemic Scleroderma:

• It is not an infectious or contagious condition, which means that it cannot be passed on from one individual to another through contact. It is also not a malignant condition
• It is not a genetic disorder, which means it cannot be transmitted to the children of an individual diagnosed with Scleroderma

What are the Signs and Symptoms of Limited Systemic Scleroderma?

The signs and symptoms of Limited Systemic Scleroderma can vary greatly from individual to individual. This might create confusion in many, since one may not have all of the signs and symptoms described for the condition. In some cases, Limited Systemic Scleroderma can be severe, causing significant signs, symptoms, and complications.

The signs and symptoms of Limited Systemic Scleroderma can include:

• Hardening and tightening of the skin in a patch formation, which can be ovals or straight lines. This can cause restricted movement of the affected area of the skin
• Raynaud’s phenomenon: In Raynaud’s phenomenon, the blood vessels of the hands and feet constrict either due to cold weather or when the individual is anxious. This constriction of blood vessels causes severe pain in the hands and feet. It also results in skin color changes in the fingers and toes
• Acid reflux
• Hair loss
• Irregular pigmentation of skin
• Joint pain and painful joint movement, pain in the feet
• Ulcer formation on the fingertips or toes
• Difficulty breathing and swallowing, dry cough
• Constipation and diarrhea
• Difficulty absorbing nutrients in the stomach, as the intestinal muscles might not move food properly
• Disrupted heart, lung, or liver functioning, which can be life-threatening

In Diffuse Systemic Scleroderma, the progression of the signs and symptoms is usually fast, causing severe and sometimes life-threatening complications. However, in some individuals, the signs and symptoms do not progress in a rapid manor, even though they are diagnosed with Systemic Scleroderma, hence the name Limited Systemic Scleroderma.

How is Limited Systemic Scleroderma Diagnosed?

Scleroderma can be difficult to diagnose, as other conditions may have similar signs and symptoms. The presence of common symptoms or skin that thickens quickly makes it easier to diagnose Scleroderma. The following tests and procedures may be used to diagnose Limited Systemic Scleroderma:

• A thorough physical examination along with a complete medical and family history
• Blood tests, such as anti-nuclear antibody (ANA) panel, erythrocyte sedimentation rate (ESR), complete blood count (CBC), rheumatoid factor (RF) levels, antibody testing for other autoimmune disorders, and other tests
• Kidney function tests including creatine level, BUN level, glomerular filtration rate (GFR), and urine analysis
• Chest X-ray and CT scan of the lungs to determine the extent of lung scarring (pulmonary fibrosis)
• Pulmonary function test (PFT)
• Electrocardiogram and echocardiogram to determine the extent of heart involvement, caused by heart scarring (cardiac fibrosis)
• Skin biopsy: Cells and tissues are examined by a pathologist under a microscope. Pathological examination is generally considered to be a ‘gold standard’ for a definitive diagnosis. The pathologist arrives at a diagnosis after analyzing pathology findings along with clinical information of the patient. A pathologist may perform special studies on tissue samples to aid in the final diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Limited Systemic Scleroderma?

The hardening of organs due to abnormal fibrosis of the tissue can severely affect the organ function. Complications due to Limited Systemic Scleroderma can range from mild to severe and could include:

• Scarring of lung tissue can occur with reduced lung function, lessened ability to breathe, and decreased tolerance for physical activity. This is known as pulmonary fibrosis. One may also develop hypertension (high blood pressure) in the arteries leading to the lungs, known as pulmonary hypertension
• If Limited Systemic Scleroderma affects the kidneys, there may be elevated blood pressure and protein in the urine. More severe kidney complications could include renal crisis, leading to rapid kidney failure
• If Raynaud’s phenomenon that develops from Limited Systemic Scleroderma is severe enough, then restricted blood flow at the fingertips can cause damage to the tissue, leading to the formation of skin ulcers. In severe cases, amputation may be necessary to avoid gangrene formation
• Increased blood pressure can also raise the pressure on the heart’s right side and cause wear and tear, leading to scarring of heart tissue. This condition increases the risk for cardiac arrhythmias and congestive heart failures
• Tightening of the facial skin to a great degree can cause the mouth to become narrower and smaller, making it difficult to clean the teeth. It is common for individuals with Scleroderma to produce inadequate amounts of saliva, which also increases the risk for dental decay
• Complications with the digestive system can occur, leading to acid reflux and swallowing difficulties. Alternating episodes of constipation and diarrhea are often seen
• Men may experience erectile dysfunction, whereas women experience decreased lubrication and constriction of the vaginal opening

How is Limited Systemic Scleroderma Treated?

Currently, Scleroderma is a condition that has no cure, but is managed through medications, lifestyle modifications, therapy, and surgery. These measures can help control symptoms and prevent complications. The skin-related signs and symptoms may fade on their own within 3-5 years. Systemic Scleroderma usually worsens over time, as it affects the internal organs.

The treatment options for Limited Systemic Scleroderma include:

• Occupational or physical therapists can help individuals with Limited Systemic Scleroderma manage pain, increase their strength, and uphold their independence in their everyday lives
• Surgery is considered to be a last resort. Amputation may be necessary in patients, whose finger ulcers have developed gangrene. Those with high blood pressure in the lung arteries due to Limited Systemic Scleroderma may need lung transplants

The following are the treatment measures for Raynaud’s phenomenon:

• Cessation of smoking: Smoking causes constriction of blood vessels, which causes an increase in symptoms due to Raynaud’s phenomenon
• Keeping hands and feet warm (particularly in colder temperatures), taking medications for anxiety, taking medications that open up the small blood vessels, all are factors that cause a better blood flow to the hands and feet (vasodilators)
• Regular exercise can also help with blood flow to the hands and feet
• Nitroglycerin gel can dilate local finger vessels to aid in healing of ulcers

Treatment measures for painful joints with or without restricted movements include:

• Regular exercise and stretching that helps loosen up the joints
• Pain medication to reduce joint pain

Treatment measures for skin signs and symptoms include:

• Regular use of sunscreen, avoiding hot showers, and regular exercising
• For individuals living in a dry climate with low humidity, the use of a humidifier can help with the signs and symptoms
• Antibiotic ointment for fingertip ulcers
• Plastic surgery may be an option for cosmetic issues; a plastic surgeon can advise on the available treatment options
• It is always important to follow the physician’s advice and take the prescribed medication regularly
• A therapist or certain support groups may be consulted on the variety of social issues that can be caused by Scleroderma, which include self-esteem issues, depression, anxiety, and relationships with friends, family, and partners

Treatment measures for dry mouth and dental issues include:

• Individuals with Scleroderma could experience tightening of the facial skin that may prevent adequate oral care; this can result in the development of tooth decay
• The following measures can help decrease the signs and symptoms:
  • Regular brushing and flossing of teeth
  • Regular dental check-ups
  • Keeping mouth wet by constantly sipping water
  • Avoid mouthwash that contains alcohol, since alcohol in mouthwashes can cause dryness of the mouth

Individuals with Limited Systemic Scleroderma can have a variety of gastrointestinal symptoms such as diarrhea, constipation, excess gas formation, difficulty in swallowing, and heartburn. The following treatment measures can help in such cases:

• Medication for heart burn, diarrhea, and constipation
• Eating small and frequent meals, which helps with movement of the food and digestion
• Avoidance of spicy and fatty foods, alcohol, and caffeinated drinks
• Avoiding food items that are hard and difficult to swallow

Individuals with Limited Systemic Scleroderma can have a variety of signs and symptoms related to the lungs, which include difficulty in breathing due to excessive scarring and high blood pressure in the lungs (pulmonary hypertension). Lung infection, in an individual with poor lung function, may result in severe complications such as severe pneumonia.

The following treatment options may be beneficial:

• It is important to see a pulmonologist on a regular basis to manage one’s signs and symptoms
• Getting regular flu shots and pneumonia shots will help prevent unnecessary infection

Individuals with heart and kidney symptoms should get regular health check-ups from a cardiologist and nephrologist respectively.

How can Limited Systemic Scleroderma be Prevented?

Currently, there are no specific methods or guidelines to prevent Limited Systemic Scleroderma. However, through proper dietary and lifestyle changes, some of the signs and symptoms may be controlled.

What is the Prognosis of Limited Systemic Scleroderma? (Outcomes/Resolutions)

• The prognosis for individuals with Systemic Scleroderma (both Limited and Diffuse types) is worse than with Localized Scleroderma (both Morphea and Linear types)
• The manner in which Limited Systemic Scleroderma changes and progresses over time varies among individuals. The rapidity of progression of signs and symptoms and the types of organs involved affects the prognosis
• Some individuals respond well to treatment and generally have a better prognosis than those who do not respond well to treatment

Additional and Relevant Useful Information for Limited Systemic Scleroderma:

• Research is currently underway to identify and understand more about Scleroderma. Studies are being completed to understand the genes that may be involved with the development of the condition, medicines to prevent the skin from thickening, and to treat the kidney/lung conditions, as well as medicines to completely treat Limited Systemic Scleroderma
• Scleroderma used to be called progressive systemic sclerosis, but this term is no longer used since Scleroderma does not always progress by nature. This means that the signs and symptoms do not progress with equal severity in all individuals