Scleroderma | Specialists and information

Scleroderma is an autoimmune-mediated chronic disease of the connective tissue in the skin and organs. The proliferation and hardening of the elastic connective tissue causes the skin to sclerotize, i.e. the skin hardens and becomes less elastic. Below you will find further information and specialists for the treatment of scleroderma.

Scleroderma - autoimmune thickening of the skin

Scleroderma, also known as systemic sclerosis, is an autoimmune disease that belongs to the group of connective tissue diseases (collagenoses). Scleroderma is chronic and progressive and leads to an increase and hardening of the connective tissue in the skin and in a number of organs, such as the lungs. The disease is also a major psychological burden for patients. Those affected should consult a doctor at an early stage in order to be able to influence the course of the disease favorably with the help of therapy.

What forms of scleroderma are there?

As a rule, women are affected more frequently than men. Nevertheless, with around 50 cases per 100,000 inhabitants, scleroderma is a rare disease. A distinction is also made between two forms of scleroderma, each of which represents a separate clinical picture:

1. Circumscribed (narrowly circumscribed) scleroderma, which occurs strictly localized in the skin.
2. Progressive, systemic scleroderma, which affects other organs as well as the skin.

Causes of the disease

The exact causes or possible triggers of scleroderma are not known. It is very likely that errors in the body's immune system are involved. In addition, those affected have malformations in the collagenous connective tissue, which leads to an overproduction of collagen. This oversupply of collagen fibres causes the skin to harden; doctors call this process sclerotization.

What are the symptoms of scleroderma?

An early symptom typical of both forms of scleroderma is the so-called "Raynaud's syndrome". When exposed to cold or stress, the fingers suddenly turn pale, sometimes painfully, followed by reactive hyperemia, i.e. increased blood flow to the fingers. This is caused by circulatory disorders resulting from the sclerotization of the skin and blood vessels.

In Raynaud's syndrome, individual fingers become pale, numb or painful @ Petra Richli /AdobeStock

As the condition progresses, inflammation and swelling occur on the skin, lower legs, toes and fingers. The skin becomes thicker and less well supplied with blood, giving it a waxy, pale appearance. Due to the increasing thickening of the skin, joints also become immobile. In addition, in the late stages of scleroderma, characteristic changes can occur in the fingers and face, for example, the mouth can only be opened with difficulty.

In systemic scleroderma, the internal organs can also be affected, often primarily the lungs. Pulmonary fibrosis, shortness of breath and coughing are the result. The heart and kidneys are also often affected, and in rare cases this can even lead to kidney failure.

What diagnostic options do doctors have?

Scleroderma is diagnosed primarily on the basis of the symptoms. In addition to the classic appearance of the skin, tissue samples are taken and examined. In addition, the erythrocyte sedimentation rate and possible autoantibodies can also be determined in the laboratory. In most cases, for example, nuclear antibodies (ANA) are found in the blood of those affected. Anti-Scl-70 (anti-topoisomerase I) or anti-centromere antibodies are described particularly frequently.

Laboratory values can also be used to detect organ involvement in scleroderma. For example, altered kidney values and muscle enzymes are regularly conspicuous. X-rays reveal bony and hardened structures in the skin and organs. ECG, ultrasound examinations or computer tomography (CT) also reveal lung and/or heart involvement.

When should you consult a doctor?

In principle, patients with scleroderma should always have their disease treated by a doctor. As the disease progresses, severe restrictions in quality of life can be expected without treatment.

In particular, if circulatory disorders occur or prolonged pallor of the skin and pain indicate scleroderma, those affected should have their symptoms checked out by a doctor.

Which specialists treat scleroderma?

Scleroderma patients will find that general practitioners ("family doctors") and orthopaedic specialists are the first port of call for treatment. Whether other specialist groups need to be consulted depends on the extent and severity of the scleroderma.

Treatment options for scleroderma

The treatment of scleroderma depends on the respective symptoms. There is no causal, i.e. cause-related, treatment. However, treated scleroderma or systemic sclerosis does not limit life expectancy.

An improvement in quality of life is usually achieved through appropriate physiotherapy or occupational therapy. Additional relief is provided by

• Heat treatments,
• acupuncture and
• massages.

Acupuncture can promote blood circulation and relieve chronic pain in scleroderma @ Andrey Popov /AdobeStock

Possible complications of scleroderma include pain, restricted movement and severe inflammation. Anti-inflammatory medication is usually used here, such as acetylsalicylic acid, immunosuppressants, but also ACE inhibitors or glucocorticoids.

Prognosis

Scleroderma cannot be cured by current means. Self-healing is also not to be expected. The treatment of systemic sclerosis is therefore primarily based on the respective symptoms and essentially aims to improve the quality of life of those affected. The course of the disease cannot be predicted.

In general, it is necessary for those affected to take various medications regularly and in appropriate doses in order to slow down the progression of the disease and maintain their quality of life. Check-ups with the doctor also ensure that changes to the organs are detected as early as possible.