Treatment of Systemic Scleroderma

Systemic scleroderma (SSc) belongs to rare autoimmune diseases that can affect not only the skin, but also blood vessels and internal organs. The course of the disease differs in each person, therefore a comprehensive examination is important for timely establishment of the diagnosis and choice of treatment.

Modern methods of therapy do not eliminate the disease completely, but allow its activity to be controlled, progression to be slowed, and the risk of complications to be reduced. The earlier treatment is started, the better the prognosis.

At the medical center «Oxford Medical», patients can undergo comprehensive diagnostics, receive a consultation with a rheumatologist and an individual treatment plan taking into account the form of the disease, its activity, and the general state of health. You can make an appointment for a consultation through the form on the website or by the contact center phone number.

Systemic scleroderma (or systemic sclerosis) — is a chronic autoimmune disease of connective tissue, which is characterized by gradual thickening of the skin and damage to blood vessels. Unlike localized forms of scleroderma, in the systemic form the pathological process can spread to internal organs, in particular the lungs, heart, kidneys, and digestive tract.

During the development of the disease, the immune system mistakenly attacks the body’s own tissues, which leads to excessive formation of collagen. Because of this, tissues gradually lose elasticity, and the function of affected organs may be impaired.

Doctors of «Oxford Medical» recommend not postponing a consultation with a rheumatologist when unclear skin changes or other symptoms appear that persist for a long time. Early establishment of the diagnosis and timely start of treatment help slow the progression of the disease.

Scleroderma combines a group of diseases that differ in the prevalence of the pathological process and course.

The main forms include:

• Localized (focal) scleroderma. The disease manifests as thickening of separate areas of the skin and usually does not spread to internal organs.

• Systemic scleroderma. The disease can affect not only the skin, but also blood vessels and internal organs. Depending on the prevalence of skin thickening, limited and diffuse forms are distinguished. The diffuse form is more often characterized by faster progression and a higher risk of damage to internal organs.

Determining the form of scleroderma is important for the prognosis of the disease, the choice of treatment tactics, and further observation of the patient.

The mechanism of development of SSc is still not fully clarified. According to National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), the disease is considered multifactorial: its development is associated with a combination of hereditary predisposition and disturbances of the immune response.

Today, specialists believe that the development of the disease may be promoted by:

• genetic predisposition to autoimmune disorders;

• changes in the functioning of the immune system, because of which it begins to attack its own tissues;

• previous infections, which in individual cases may become a trigger factor;

• the influence of certain environmental factors in people with hereditary predisposition;

• female sex, since the disease is diagnosed significantly more often in women.

In some patients, systemic scleroderma develops independently, while in others it may be combined with other autoimmune diseases, in particular with systemic lupus erythematosus or rheumatoid arthritis. At the same time, the exact mechanism that triggers the development of the disease in each specific case remains unknown.

If the disease affects internal organs, additional symptoms may appear. In particular, when the lungs are affected, shortness of breath sometimes occurs, and when the function of the kidneys or cardiovascular system is impaired, swelling of the lower extremities may appear. Swallowing disorders, heartburn, or other manifestations from the digestive system are also possible.

Specialists of «Oxford Medical» recommend consulting a rheumatologist if skin changes are combined with Raynaud's phenomenon, joint pain, or other symptoms that do not pass for a long time. Timely diagnosis helps start treatment before the development of irreversible changes.

Diagnosis of SSc begins with a consultation with a rheumatologist. The doctor assesses complaints, examines the skin and joints, clarifies when the symptoms appeared, and also determines whether there are signs of damage to internal organs.

To confirm the diagnosis and assess the prevalence of the disease, the following may be prescribed:

• general and biochemical blood tests;

• immunological studies, in particular determination of specific autoantibodies;

• capillaroscopy of the nail bed — a method of examining small blood vessels near the nails, which helps detect changes characteristic of systemic scleroderma;

• computed tomography of the chest organs — if there is suspicion of lung involvement;

• spirometry and other studies of lung function — when damage to the respiratory system is suspected;

• ultrasound examination of the heart (echocardiography) — in the presence of symptoms that may indicate damage to the heart;

• kidney ultrasound and other studies of the urinary system — if the doctor suspects impaired kidney function.

If necessary, the rheumatologist may recommend consultations with a cardiologist, pulmonologist, nephrologist, gastroenterologist, or other specialists. The scope of the examination is determined individually depending on the clinical picture and possible organ damage.

Doctors of «Oxford Medical» recommend undergoing a comprehensive examination even with minor manifestations of the disease. This helps not only to confirm the diagnosis, but also to timely detect damage to internal organs, on which further treatment tactics largely depend.

According to the recommendations of European Alliance of Associations for Rheumatology (EULAR), treatment of systemic scleroderma is selected individually, taking into account the form of the disease, its activity, and the degree of damage to internal organs. The main goal of therapy is to control autoimmune inflammation, slow the progression of the disease, reduce the severity of symptoms, and prevent the development of complications.

Treatment may include:

• immunosuppressive preparations — to reduce excessive activity of the immune system and control autoimmune inflammation;

• preparations for the treatment of vascular disorders — to improve blood circulation and reduce manifestations of Raynaud's phenomenon;

• preparations for the treatment of damage to internal organs — in the presence of changes from the lungs, heart, kidneys, digestive system, or other organs;

• symptomatic preparations — to reduce pain, heartburn, digestive disorders, and other manifestations of the disease.

Since systemic scleroderma is a chronic disease, treatment is usually long-term. In the process of therapy, the doctor may change the treatment regimen depending on the activity of the disease, the effectiveness of the prescribed preparations, and the general condition of the patient.

Doctors of «Oxford Medical» recommend not changing the prescribed treatment independently and regularly undergoing follow-up examinations. This makes it possible to assess the effectiveness of therapy, correct it in a timely manner, and reduce the risk of disease progression.

Without timely treatment, systemic scleroderma can lead to the progression of damage not only to the skin, but also to internal organs.

Possible complications:

• interstitial lung disease;

• pulmonary arterial hypertension;

• heart failure and heart rhythm disorders;

• scleroderma renal crisis;

• joint contractures;

• esophageal motility disorders.

Timely treatment and regular monitoring of the course of the disease help reduce the risk of developing complications.

Since the causes of the development of systemic scleroderma have not been definitively clarified, methods that would allow its occurrence to be completely prevented do not exist. At the same time, timely treatment and regular monitoring of the course of the disease help slow its progression and reduce the risk of damage to internal organs.

To maintain a stable course of the disease, it is recommended to:

• regularly undergo follow-up examinations with a doctor;

• take medications according to prescriptions;

• avoid hypothermia, since it can provoke vascular spasms and intensify manifestations of Raynaud's phenomenon;

• give up smoking, which negatively affects the condition of blood vessels;

• maintain physical activity.

Doctors of «Oxford Medical» recommend paying close attention to any changes in well-being and not postponing planned visits to a rheumatologist. Regular monitoring of the course of the disease allows treatment to be corrected in a timely manner, the function of internal organs to be maintained, and quality of life to be preserved.

The cost of treatment of systemic scleroderma depends on the clinical form of the disease, the degree of damage to internal organs, the necessary scope of examinations, and the prescribed therapy. For each patient, the treatment plan is prepared individually.

You can preliminarily familiarize yourself with the prices for doctors' consultations here. During the appointment, the specialist will determine the necessary scope of diagnostic procedures, and then prepare a treatment plan. Also, if necessary, they will orient you regarding the cost of medical services. To make an appointment for a consultation with a rheumatologist, contact the contact center of «Oxford Medical» or use the online appointment form on the website.

The information is for informational purposes and does not replace a doctor's consultation.

Sources:

• National Institute of Arthritis and Musculoskeletal and Skin Diseases
• European Alliance of Associations for Rheumatology
• US National Library of Medicine