Brief overview
- What is scleroderma?: disease of connective tissue, two forms: circumscritic and systemic scleroderma
- Symptoms: thickening of the skin, Raynaud’s syndrome, mask face, joint and muscle pain
- Course and prognosis: Depends on which organs are affected
- Treatment: Not curable, depends on which organ is affected
- Causes and risk factors: Autoimmune disease of unknown cause, genetic predisposition.
- Prevention: No preventive measures known
What is scleroderma?
Scleroderma refers to a group of diseases in which the skin and connective tissue thicken and harden. If the symptoms are limited to the skin, it is called circumscritic scleroderma. If internal organs such as the lungs, intestines, heart or kidneys are also affected, it is called systemic scleroderma (also: progressive systemic sclerosis).
Forms of scleroderma
The trigger for scleroderma is a disease of the connective tissue. Since this tissue is found almost everywhere in the body, scleroderma usually affects not only the skin, but spreads throughout the entire body. Depending on which organs are affected, there are two forms of scleroderma.
Systemic scleroderma
In systemic scleroderma (also known as progressive systemic sclerosis), the disease is not limited to the skin, but also affects other organs.
Limited form: The skin lesions are found only from the fingers to the elbow or from the toes to the knee. Other parts of the body such as the chest, abdomen and back remain free, and the head is rarely affected. In some circumstances, systemic scleroderma extends to the internal organs.
Systemic sclerosis sine scleroderma: It is a special form of sclerosis because the changes are found on the organs but not on the skin.
Circumscribed Scleroderma
Circumscribed scleroderma is also called morphea. Characteristic of this form is that the changes affect only the skin. Internal organs are not involved.
Depending on the spread and depth of the skin changes (plaques), circumstrictive scleroderma is divided into four forms:
- Limited form
- Generalized form
- Linear form
- Deep circumscritic scleroderma (Deep morphea)
Frequency of scleroderma
About 1,500 people are diagnosed with systemic sclerosis every year, and an estimated total of 25,000 people with the disease live in Germany. Most often, the first symptoms appear between the ages of 50 and 60, in some cases even after the age of 65. Women are four times more likely to develop scleroderma than men.
How do I recognize scleroderma?
Symptoms of systemic scleroderma
With systemic scleroderma, symptoms are possible throughout the body. Typically, the following symptoms occur:
- Raynaud’s syndrome:
Raynaud’s syndrome is often the first symptom of systemic scleroderma. See a doctor as soon as possible if you experience these symptoms!
- Changes in the skin:
Hardening and scarring are found in almost all patients with systemic scleroderma, and in principle at every skin site.
- Involvement of the joints:
Sometimes painful calcifications (calcinoses), palpable as hard nodules, are found near small joints. This is caused by accumulations of calcium salts under the skin.
- Involvement of the musculature:
If the musculature is also affected by scleroderma, pain typically occurs during movement. Affected individuals report that their muscles tire quickly and that they feel powerless.
- Damage to internal organs:
Heart: In 15 percent of all cases, scleroderma damages the heart. Most frequently, inflammation of the heart muscle or pericardium occurs. If left untreated, this can develop into life-threatening heart failure or cardiac arrhythmias.
Typical signs that the heart is also affected are chest pain, severe palpitations, fainting spells or swollen legs.
Digestive tract: Symptoms that occur in the digestive tract in scleroderma include flatulence or constipation. Dry mouth and heartburn are other possible complaints.
- Other symptoms
Systemic scleroderma can affect the entire body. The symptoms are correspondingly varied and non-specific: they range from fatigue to sleep problems to hoarseness.
Symptoms of Circumscribed Scleroderma
- Limited form:
The skin lesions are larger than two centimeters and are located on one to two areas of the body, usually on the trunk (chest, abdomen, back).
- Generalized form:
The skin lesions appear in at least three locations, often on the trunk and thigh, and are often symmetrical.
- Linear form:
- Deep circumscritic scleroderma (Deep morphea):
In this very rare form, the indurations are found in the fatty tissue and muscles. It occurs symmetrically on arms and legs and often begins in childhood. Typical symptom is muscle pain.
How long can you live with scleroderma? Is scleroderma fatal?
Circumscribed scleroderma
Scleroderma is not curable, but symptoms can be treated well. In circumstrict scleroderma, the indurations remain confined to the skin. Affected individuals therefore have the same life expectancy as non-affected individuals. In some cases, the disease heals on its own.
Systemic scleroderma
According to statistics, the so-called 10-year survival rate for systemic scleroderma is currently 70 to 80 percent. This means that 70 to 80 percent of patients are still alive ten years after diagnosis.
If scleroderma affects the lungs, the prognosis is usually worse. The most common causes of death in scleroderma are pulmonary hypertension and pulmonary fibrosis.
What can be done about scleroderma?
According to current knowledge, scleroderma is not curable. Depending on which organ is affected, the doctor uses different treatments. In this way, he slows down the progression of the disease and alleviates the symptoms.
Treatment of systemic scleroderma
Therapy is primarily based on which organs are affected by scleroderma and which symptoms are to be alleviated.
If the lungs are affected in scleroderma, the cytostatic drug cyclophosphamide is often used. If the kidneys are involved, ACE inhibitors are used.
Light therapy (PUVA) as well as lymphatic drainage, physical therapy and physiotherapy help to counteract stiffening of the fingers in scleroderma.
What can you do yourself?
- Take care of your skin regularly to prevent scarring. Ask your doctor which skin care products are suitable.
- Get enough exercise. Regular exercise keeps you fit and contributes to your well-being.
- Eat a healthy diet: Diet also helps to alleviate the symptoms of scleroderma. Eat little red meat, but plenty of fruits and vegetables and unsaturated omega-3 fatty acids (such as in fish). This will help your body minimize inflammation.
Circumscribed scleroderma treatment
Light treatment (phototherapy) with UVA light is the most effective treatment for circumskripten scleroderma. It is supposed to help against the inflammation, hardening and thickening of the skin. Together with an active ingredient from the psoralens group, which makes the skin more sensitive to light, this treatment is called PUVA. PUVA can be applied as a cream (cream PUVA), bath (bath PUVA) or tablet (systemic PUVA). The hardened skin areas usually become much softer.
Causes and risk factors
Causes
Why the immune system does not work properly is unknown. Medical experts assume that several factors play a role.
Possible triggers for the autoimmune disease are:
- Genetic predisposition
- Hormones (women fall ill more often than men)
- Environmental factors such as infections with viruses and bacteria (Borrelia) or smoking
- Medications such as bleomycin, pentazocine
- Chemicals such as organic solvents, gasoline, formaldehyde
Risk factors
Examinations and diagnosis
The first thing that stands out are the changes in the skin, often associated with Raynaud’s syndrome in systemic scleroderma. The first contact person when scleroderma is suspected is the internist or dermatologist. He or she will first inquire about the symptoms, followed by a thorough physical examination.
Examination of the skin
The physician looks for typical skin changes that are indicative of scleroderma. Depending on where they occur, he or she can further narrow down the diagnosis. Raynaud’s syndrome, for example, does not occur in circumscritic scleroderma. So, if it is present, it is more indicative of systemic scleroderma.
Examination of the small nail fold vessels
Blood test
If systemic sclerosis is suspected, the doctor examines the blood. In almost all scleroderma patients, certain antibodies, so-called antinuclear antibodies (ANA), are found in the blood. The blood test also gives the first indication of whether the organs are affected.
X-ray
Computer tomography (CT)
If the doctor suspects that internal organs such as the lungs, kidneys or heart are affected, he or she orders a computed tomography scan.
Magnetic resonance imaging (MRI)
Some changes can be better detected by magnetic resonance imaging (MRI). For example, if the doctor detects an “en coup de sabre,” he uses MRI of the head to examine whether the brain is also affected by scleroderma.
Further examinations
Prevention
Since the exact trigger for scleroderma is not known, there are no concrete measures to prevent the disease. At the first signs pointing to scleroderma, it is all the more important to consult a doctor at an early stage. In this way, the course of the autoimmune disease can be favorably influenced.
