Scleroderma: what is it and how to treat a chronic autoimmune disease that makes the skin hard and stiff

The systemic sclerosis or “scleroderma” is a chronic autoimmune disease which, although it mainly affects the skin (hence its name: scleroderma means “hard skin”), It can also affect internal organs such as the lungs, kidneys, intestine, esophagus, joints and heart.

Consequence of the disease, whose World Day is commemorated every June 29, the skin becomes harder and stiffer, but at first it can manifest itself as swelling of the skin of the hands and feet, sometimes of the face. You can also have more dilated blood vessels in the face, hands and folds. One of the clinical features of a large portion of patients with this disease is the presence of what is called “Raynaud’s phenomenon”. This is the visualization of color changes in the fingers or toes which, when exposed to cold, become excessively pale, then purple, in some cases accompanied by pain or tingling. In the digestive system, a person with systemic sclerosis may have symptoms of gastroesophageal reflux disease, heartburn, and difficulty swallowing., among others.

It should be noted that respiratory system this is one of the most frequently affected by systemic sclerosis. According to different studies, over 80% of patients with systemic sclerosis have lung involvement. Additionally, the leading cause of death in people diagnosed with systemic sclerosis is interstitial lung disease.

Is considered an orphan or rare disease because its prevalence is 9 people per million inhabitants and an incidence of 5 to 12 cases per million people per year. It is 3 to 4 times more common in women and the maximum incidence is between 30 and 50 years.

the lung damage it is very common and he usually presents with interstitial lung disease and pulmonary hypertension. Normally the patient begins to develop it before showing any symptoms, so it is very important to rule out the affectation early by further studies.

In order to identify interstitial lung disease questioning and physical examination are essential, In addition, a chest CT scan (which should be high resolution) and lung function tests are ordered. the When the patient has symptoms, they usually present shortness of breath and cough when you exercise. For the study of pulmonary hypertension additional studies are also requested, being important cardiac ultrasound.

While the diagnostic of systemic sclerosis is based on the clinical presentation of the patient, there are additional studies that are very useful such as the presence of certain autoantibodies. And because of the potential organ compromise that systemic scleroderma presents, evaluation within interdisciplinary teams is essential.

As part of pharmacological treatment, three major groups of drugs to treat three of the most important changes in the disease: vasodilators, immunosuppressants and anti-fibroproliferatives.

In addition to this, there are a number of general recommended measures:

– Take care of the skin, moisturize it daily and treat any injuries and protect it from the sun

– Avoid the cold and use gloves

– Avoid stress

– No smoking

– To be strict with the oral hygiene and uses special dry mouth toothpaste

– Carry out muscle stretch Yes physiotherapy exercises Yes rehabilitation

– Take anti-reflux measures, in case the patient suffers from it: avoid large meals, chew food well, andavoid lying down for up to 2-3 hours after eating and raising the head of the bed

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