CLNICAL TRIALS WARSZAW

Systemic Sclerosis (Scleroderma)

A chronic, rare autoimmune disease that can lead to life-threatening complications. Systemic sclerosis is also called scleroderma (Greek for “hard skin”). It is a connective tissue disease characterized by progressive fibrosis of the skin and internal organs, eventually damaging them and preventing proper function. It occurs relatively rarely (max. 3 cases per 100,000 people), more often in women between 30 and 50.

The disease has several forms:

  1. Limited Systemic Sclerosis: Skin changes occur slowly, starting on the feet and hands and never crossing the elbows or knees.
  2. Diffuse Systemic Sclerosis: Skin changes involve the hands, arms, thighs, and trunk. The disease develops quickly and usually has a sudden onset, with skin, vascular, and internal organ symptoms appearing simultaneously.

Early Symptoms

Early symptoms are observed in the skin, but over time the process involves all vital organs.

  • Raynaud’s Phenomenon: Sudden attacks where fingers turn pale, then blue, accompanied by strong pain, triggered by cold or stress.
  • Induration: The skin loses elasticity, becoming thick and hard; discoloration may occur.
  • Atrophy: Damage to blood vessels leads to hard-to-heal scars, ulcers, and the wasting of nails and skin.
  • Contractures: Thickening of the skin causes limited joint mobility.
  • Facial Changes: Loss of facial expression (“mask-like” face), inability to fully close eyelids, and difficulty opening the mouth (“bird-like” mouth).

Treatment of Systemic Sclerosis

Any internal organ ailment following skin changes is an indication for a specialist consultation. The goal of a rheumatological consultation is to determine if the skin changes are characteristic of scleroderma, refer the patient for capillaroscopy and blood tests, and rule out other underlying causes (e.g., cancer).

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Systemic Sclerosis (Scleroderma)

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