Systemic Sclerosis or Scleroderma

"It is a chronic disease but in most cases the patients can live a normal life".

DR. ENRIQUE ORNILLA LARAUNDOGOITIA
SPECIALIST. RHEUMATOLOGY SERVICE

Systemic sclerosis, also known as scleroderma, is a chronic autoimmune disease that primarily affects the skin.

Systemic sclerosis or scleroderma, in addition to affecting the skin, can affect internal organs (lungs, intestines, kidney or heart). The skin changes are caused by the increase and accumulation of collagen fibers and other proteins. The skin becomes sclerotic (hard and rigid).

At first, the lesions consist of an inflammation, which later turns into a hardening.

Systemic sclerosis also hinders the blood supply by occluding the small arteries and capillaries that carry blood to the tissues.

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What are the symptoms of systemic sclerosis?

The most visible effects are skin lesions or morphia. It usually starts with a slight swelling of the skin of the hands and feet, sometimes also of the face, which spreads. Later, the skin becomes rigid and hard, difficult to pinch and sometimes limits the movements of the joints. 

However, in those people with involvement of the entire body, the scars and thickening extends to the underlying structures (fat, muscle and rarely, bone) . There are usually changes in the color of the hands that, when exposed to the cold, become excessively pale and then violet, sometimes accompanied by pain or tingling. These symptoms are called Raynaud's phenomenon. Poor circulation can lead to serious damage, such as ulcers on the fingers or gangrene.
 
There can be dilated blood vessels in the face, hands, nail folds and other places. Some patients develop calcium deposits (calcinosis) in the skin and other organs such as the lungs, muscles and kidneys.

Systemic sclerosis can also cause joint pain, fatigue, digestive problems such as difficulty swallowing, heartburn, constipation or diarrhea, and cardiorespiratory problems such as shortness of breath, high blood pressure or chest pain.

The most common symptoms are:

  • Morphia.
  • Stiff and hard skin.
  • Pain in the joints.
  • Raynaud's phenomenon.

Do you have any of these symptoms?

You may have systemic sclerosis or scleroderma

How is scleroderma diagnosed?

<p>Laboratorio de extracciones</p>

The diagnosis is made through a specialized medical examination by the rheumatologist, which will include the account of the symptoms, a skin test and a general examination.

Complementary examinations such as tests and a chest X-ray are always performed, and only in some cases other digestive or cardiorespiratory tests.

The purpose of these tests is to better classify the type of disease and to assess its severity and extent.

How is systemic sclerosis treated?

No drug has been shown to be effective in 100% of patients with systemic sclerosis, although there are partial responses to immunosuppressants in cases of systemic affectation.

Treatment of the inflammatory stage of the skin lesion usually requires the administration of steroids. 

Interstitial lung involvement may be improved, in initial stages, with oral cyclophosphamide/mycophenolate mofetil and steroids.

In the case of pulmonary hypertension, calcium blockers, prostacyclin or analogues may be used.

Gastrointestinal involvement is improved with anti-reflux postural measures, H2-blockers, prokinetics and erythromycin. Broad-spectrum antibiotics can be used for malabsorption.

Calcium blockers, together with antiaggregants, are effective in Raynaud's phenomenon.

Periodic blood pressure control is essential in patients with diffuse sclerosis. Treatment should be initiated when blood pressure is higher than 140/90 mmHg. Cases of marked calcinosis may require microsurgery.

For treatment of muscle involvement, the use of corticosteroids in vigorous guidelines is recommended.

The treatment of arthralgias, arthritis or tenosynovitis, requires the use of non-steroidal anti-inflammatory drugs (NSAIDs) and rest. The use of local infiltrations can be very useful.

Where do we treat it?

IN NAVARRE AND MADRID

The Rheumatology Service
of the Clínica Universidad de Navarra

The Rheumatology Service has a multidisciplinary team highly specialized in the diagnosis and treatment of rheumatological diseases, from osteoarthritis, arthritis or osteoporosis to autoimmune or inflammatory diseases.

In addition, we have doctors specialized in assisting pregnant women with autoimmune diseases, in order to guarantee the maximum safety of the fetus.

Organized in specialized units

  • Inflammatory arthropathies.
  • Degenerative arthropathies.
  • Microcrystalline arthropathies.
  • Bone pathology.
  • Systemic autoimmune diseases.
  • Autoinflammatory diseases.
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  • Valoración integral del paciente.
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  • Tecnología de vanguardia.

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