Scleroderma is a disease whose causes have not been fully explained. They take into account, for example, the genetic, immunological, vascular background and the harmfulness of the external environment. In scleroderma, the production of collagen by fibroblasts is increased. Hardened swelling occurs initially, followed by induration and disappearance.

Systemic sclerosis(Latinscleroderma ) is a rare, chronic, incurable autoimmune disease involving connective tissue. Because connective tissue is present throughout the body, people with systemic sclerosis can have different combinations of symptoms, which is one reason their experiences of the disease can vary.

The disease can scar the skin and major organs such as the heart, lungs, digestive system and kidneys, and can lead to life-threatening complications. unlike the systemic type, it applies only to the skin and deeper tissues.

Systemic sclerosis - causes

The causes of systemic scleroderma are not yet fully understood. Genetic and environmental factors, such as exposure to silicon, vinyl chloride, or trichlorethylene, are likely to play a role. A factor that may also influence the pathogenesis of scleroderma is the injection of silicone during breast plastic surgery.

The prevalence of the disease in women can be explained by the theory of microchimerism. It is the survival of the fetal cells in the mother's body, which usually remain inactive. It is suspected that the activation of these cells by various factors may result in symptoms of the disease.

Systemic scleroderma - symptoms

Scleroderma causes numerous changes in the body, so symptoms can be divided according to the area they concern.

Symptoms of scleroderma visible on the skin:

  • Raynaud's syndrome, i.e. paroxysmal paling and later blue hands and swelling
  • atrophic changes in red lips and nose
  • hardening of the fingers, phalanges may thin out and necrosis
  • changes in the type of sclerotic edema or signs may occur in the upper and lower limbs, face, neck and trunk
  • in advanced lesions, contracture and immobilization of the hands may occur

Symptoms of scleroderma can also appear as changes in the skeletal system:

  • joint pain and inflammation of the tendon bags
  • narrowing of articular capsules
  • disappearance of nail tubercles
  • osteoporosis

Changes in the muscular system give symptoms in the form of muscle pain caused by overgrowth and inflammation of the intermuscular tissue.

Changes in microcirculation detected during capillaroscopic examinations result in:

  • atrophy of capillaries
  • Vascular loops in Raynaud's syndrome dilated in the middle part

Organ changes give symptoms in the form of:

  • difficulty swallowing food due to widening and atony of the esophagus
  • hypertension due to changes in the fine vessels of the kidney
  • shortness of breath and cough due to pulmonary fibrosis
  • arrhythmias
  • conduction disturbances
  • pulmonary hypertension
  • pericarditis

Systemic sclerosis - diagnosis

Changes occurring in the course of scleroderma are usually so characteristic that they leave no doubt as to its diagnosis. Laboratory tests useful in making a diagnosis are:

  • testing for the level of antibodies against ACA centromeres
  • testing the level of antibodies against the nuclear antigen Topoisomerase I Scl-70

It happens that additional tests are also ordered, such as capillaroscopy, histological or electromyographic examination of the muscle tissue, as well as spirometry, ECG and echocardiography.

Systemic sclerosis - treatment

Due to the previously unknown pathogenesis of scleroderma, there is no effective causal treatment yet. The therapy is directed against the processes of fibrosis as well as vascular and inflammatory changes and is aimed at relieving symptoms and preventing complications. The drugs used are mainly:

  • immunosuppressants, e.g. cyclophosphamide, methotrexate, cyclosporin A and in severe cases corticosteroids and captopril
  • drugs that act on the fibrotic process, such as penicillamine and gamma-interferon
  • vascular drugs: nifedipine, prostacyclin, low molecular weight dextran, vitamin E

Particular attention should be paid to changes in the lungs, which occur in many patients with systemic sclerosis, but are not always noticeable.

Interstitial lung disease is the leading cause of mortality in patients with systemic sclerosis, accounting for nearly 35% of deaths associated with the disease, with one in four developingnotable lung disease within three years of diagnosis.

It is important for patients and their loved ones to be aware that even small changes may suggest lung disease, because the sooner such symptoms are reported, the sooner the patient can be diagnosed and receive appropriate support. Symptoms of pulmonary fibrosis include:

  • breathless
  • short of breath when exercising, going uphill or down stairs
  • as well as a dry, irritating cough that does not go away

Category:

Dermatology