Scleroderma (Systemic Sclerosis)
Scleroderma is an autoimmune disease that affects the skin and other organs. The underlying cause is not fully understood, and treatment varies depending on the affected area.
The lack of effective treatment and the ability to affect organs such as the kidneys, heart, and lungs make early diagnosis important.
What Is Scleroderma?
Scleroderma is an autoimmune disease characterized by hardening and thickening of the skin, and involvement of internal organs. It is also known as systemic sclerosis, and its Greek meaning is “hard skin.” This disease, which causes hardening of the skin, can cause damage to different internal organs such as the lungs, heart, blood vessels, stomach, esophagus, and kidneys.
The different regions affected by the disease cause it to progress in different ways. Therefore, in addition to mild skin involvement, there are also cases of scleroderma that are extremely serious and have a life-threatening risk factor. Disease symptoms also vary from person to person.
What Causes Scleroderma?
Scleroderma is a disease whose cause is not fully understood, and it is thought that many factors, including genetic, environmental, and structural factors, play a role in the development of the disease.
Under normal circumstances, the immune system’s job is to protect the human body from harmful external factors. In scleroderma, the immune system mistakenly attacks the body’s own tissues and organs.
As a result, damage occurs in many tissues and organs in the body. The underlying problem of the disease lies in this problem that arises for any reason in the body’s defense mechanism. The damage to the immune system increases the number of fibroblast cells in the body. Following this, an increase in collagen is observed in the connective tissue and thickening is seen in the tissues.
Although it is thought that this is the main problem underlying the disease, the following conditions are also accepted as important risk factors:
GENETICS
People with a family history of scleroderma are at a much higher risk of developing the disease. Therefore, genetic factors are an important risk factor. The disease can be observed due to chromosome breaks or changes in the person’s genetic code. In addition, diseases such as SLM, Hashimoto’s thyroiditis, and rheumatoid arthritis, which are transmitted through the genetic code, can also cause scleroderma.
AGE
The risk of disease is very high in individuals between the ages of 30 and 50. In addition, this disease is seen 6 times more often in women of childbearing age than in men.
ENVIRONMENTAL FACTORS
Individuals exposed to toxic substances such as benzene, glue, and polyvinyl chloride are at a higher risk of developing scleroderma than other people. These substances can trigger the development of the disease.
RACE
Scleroderma is seen more often in black people than in white people. Therefore, it is accepted that race factor also has an effect on the development of the disease.
In addition, scleroderma can be triggered at the end of a number of viral infections and exposure to various viruses.
What Are The Symptoms Of Scleroderma?
Scleroderma can affect different parts of the body, and the symptoms vary depending on the affected area.
The most common affected areas are the blood vessels, skin, musculoskeletal system, lungs, kidneys, and heart. Raynaud phenomenon is also a distinguishing symptom of scleroderma. The most common symptoms are listed as follows:
- Raynaud’s phenomenon is defined as discolouration of the extremities of the body, such as the fingers, especially due to cold and stress. In this condition, the blood flow in the fingertips is temporarily interrupted and white, purple, red colour changes occur. This phenomenon is observed in 90% of scleroderma patients. The discolouration of the fingers and toes may be accompanied by swelling, pain, numbness, skin ulcers and, in advanced cases, gangrene.
Another symptom is hardening and thickening of the skin. Facial appearance changes such as shiny and tense skin, line formation and shrinkage in the corners of the mouth, loss of teeth, and tapering of the nose may be observed. - Calcium deposition under the skin may begin.
- High blood pressure is observed in cases of kidney involvement.
- Shortness of breath and joint pain may occur.
- Other symptoms include bloating and constipation after eating, reflux in the stomach and oesophagus, burning and difficulty in swallowing.
Types Of Scleroderma
On its basis, scleroderma is divided into two types: localised and systemic. Both types are divided into groups within themselves. In localised scleroderma, there is no involvement of internal organs and the typical features are that the skin, subcutaneous tissues and muscle tissues are affected.
Dermatological problems are usually seen in this group, which does not carry life risk factors. In localised linear scleroderma that starts in childhood, developmental disorders of the arms and legs may occur.
Systemic scleroderma is a group in which internal organ involvement is observed in more serious conditions. It is divided into many subgroups according to the site of involvement, mode of onset and clinical differences. This group is more life-threatening than the other groups because it involves the internal organs.
How Is Scleroderma Diagnosed?
In the diagnosis of scleroderma, the history of the disease and findings such as Raynoud’s Phenomenon are distinctive. In addition, other symptoms such as joint symptoms, cough and shortness of breath are evaluated together.
Definitive diagnosis can be made with blood tests and biopsies. When deemed necessary by the physician, advanced imaging techniques such as lung and oesophageal X-rays, lung tomography, endoscopic examinations and echocardiography may be used.
What Are Scleroderma Treatment Methods?
The course of scleroderma varies greatly from person to person. In some cases, the disease progresses slowly and insidiously, making it difficult to diagnose. In other cases, the disease can progress rapidly.
In general, the earlier scleroderma is diagnosed, the better the prognosis. This is especially true for organ involvement, such as lung disease. Early diagnosis and treatment can help to slow the progression of the disease and improve the quality of life.
- Skin changes caused by localised scleroderma can be treated with cortisone ointments and moisturisers. If a larger area is affected, different cosmetic treatments or laser treatment can be applied.
- If Raynaud’s phenomenon is observed; methods to accelerate blood circulation should be used. Calcium channel blockers and aspirin can be used for this. Drugs that reduce the clotting properties of the blood and open the blood vessels can also be used to protect the stomach from the acid effect. In these patients, protecting the extremities of the body from cold reduces the frequency of symptoms.
- In case of reflux, a special diet programme and regular medication should be followed. Especially limiting the consumption of chocolate, coffee and fatty foods that trigger reflux helps the treatment.
- In case of kidney stiffness; treatment can be provided with blood pressure medication. Drugs used in the early period are effective in preventing kidney damage and help to significantly reduce life risks.
- In muscle and joint stiffness; medications can be used to relieve pain in the area. In these cases, appropriate medication should be administered to prevent symptoms of weakness and fatigue, especially in the morning. In addition, physiotherapy sessions are also effective in preventing pain by increasing joint flexibility.
- Lung stiffness may develop in different ways. In these cases, which cause symptoms such as shortness of breath, pulmonary hypertension and heart failure, drugs are used to relax the pulmonary vessels. In addition, oral and inhaled medications prevent excessive growth in the lungs. Oxygen support can be provided to the patient if necessary, and lung transplantation can be considered in advanced cases.
- Digestive system disorders cause various symptoms such as indigestion, fatigue, heartburn, diarrhoea and constipation. Treatment should alleviate these symptoms and the mental problems caused by them. For this, the use of antibiotics may be recommended as well as drugs that relieve the digestive system.
- Oral and dental problems can occur in various ways such as weakening of the gums, tooth loss, narrowing of the mouth width and decreased salivation. In all of these cases, special brushes and oral care products should be used. Physician-recommended cleaning methods and treatments that prevent dry mouth help to reduce caries formation. Additional treatment methods such as chewing sugar-free gum, gargling and drinking plenty of water can also be applied.
