Scleroderma, the physical and emotional impact

Scleroderma is a rare autoimmune disease, characterised by fibrosis of the skin and of the internal organs and by vascular problems. It should be referred to by the more appropriate name “Systemic Sclerosis” due to its probability of involving different organs in addition to the skin. It is more frequent in women and can develop in any age group, although it is more common in patients who are between 20 and 50 years old. It is estimated that there are 3000 cases in Portugal.

The symptoms vary for each person as well as the seriousness of the disease. The most common clinical manifestations are: Raynaud (reduced blood flow in the fingers), acid reflux, difficulties to swallow, thickening of the skin, joint pain, shortness of breath. Any part of the skin can be affected, although it is more usual in the hands and face. The lungs, the heart, the digestive tract and the kidneys are the most critical internal organs for this disease.

According to the different skin involvement, there are three types of systemic sclerosis:

• Limited cutaneous scleroderma (affects the face, hands and forearms)
• Diffuse cutaneous scleroderma (affects the skin in a more comprehensive way, in addition to the limited cutaneous scleroderma areas)
• Systemic sclerosis without scleroderma (does not involve the skin but affects the internal organs)

Any of these can affect the internal organs.

The severity of the disease varies for each patient but it is generally more serious for diffuse cutaneous scleroderma patients whose internal organs are also affected. Despite the progressive evolution, it is more frequent for these patients to experience a quick development of their disease between the first 3 to 5 years, after which it stabilizes.

The diagnosis is based in the clinical manifestations in addition to laboratorial changes (presence of specific autoantibodies). Additional medical examination should be made in order to evaluate the involvement of the internal organs, mainly the lungs and the heart, which, at a primary stage, can show no symptoms to the patient.

Even though there is no cure for this disease, there are currently some immunosuppressive medications that seem to alter and minimize the evolution of the disease. In addition, there are symptomatic treatments which focus on the symptoms of each patient.

The disease can have a big impact in each patient, whether physical or psychological. In terms of physical effects, blood vessel (Raynaud and skin ulcers), joint and gastrointestinal tract problems are the ones that seem to matter the most in the everyday life of patients. The loss of quality of life can obviously lead to psychological problems too. Furthermore, the skin involvement can cause a big change to the patients’ appearance and consequently lead to a negative image of themselves.

In the current pandemic situation, it is highly important to emphasize a few points. Some systemic sclerosis patients are being medicated with immunosuppressants but this does not seem to increase the risk of being infected by the coronavirus or its severity. However, if systemic sclerosis patients who suffer from interstitial lung disease get infected, they can develop a more serious illness with shortness of breath, so they must follow thoroughly the recommendations such as social distancing, wearing a face mask and seeking medical assessment in case the respiratory symptomatology returns.

Author:

Susana Oliveira
Graduated Hospital Assistant,
Systemic Sclerosis Consulting Coordinator
Professor Doutor Fernando Fonseca Hospital