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Scleroderma (Systemic Sclerosis)

Autoimmune disease causing fibrosis and vascular damage of skin and internal organs.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Scleroderma (Systemic Sclerosis)?

Scleroderma (systemic sclerosis) is a chronic autoimmune disease in which excessive collagen deposition, microvascular damage and immune dysfunction affect the skin and internal organs. It is divided into limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subtypes; sine scleroderma — without clinical skin involvement but with internal-organ disease — is also recognised.

In limited disease, skin thickening stays distal to the elbows and knees; CREST syndrome (calcinosis, Raynaud, oesophageal dysmotility, sclerodactyly, telangiectasia) is typical. In diffuse disease, thickening extends to the trunk and serious complications such as lung fibrosis, scleroderma renal crisis and cardiac involvement are more common.

Anti-centromere antibodies are associated with the limited form; anti-Scl-70 (topoisomerase I) and anti-RNA polymerase III with the diffuse form. Treatment is individualised by subtype and organ involvement; no therapy yet halts the disease entirely.

Symptoms

Raynaud phenomenon (colour changes in the fingers)
Skin thickening in the fingers and hands
Telangiectasias and digital ulcers
Dysphagia and reflux
Shortness of breath and dry cough
Joint pain and contractures
Loss of facial expression and limited mouth opening
Weight loss and fatigue

Risk Factors

Female sex (4-5 times more common)
Age 30-50
Family history of scleroderma or autoimmune disease
Exposure to silica dust or organic solvents
Exposure to certain chemotherapy agents
Vinyl chloride exposure
Genetic factors such as HLA-DRB1
Epstein-Barr virus infection

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • New-onset Raynaud phenomenon
  • Skin thickening and swelling of the fingers
  • Dysphagia and resistant reflux
  • Unexplained shortness of breath
  • New hypertension and impaired renal function
  • Non-healing ulcers on the fingertips

Treatment Methods

01
Calcium-channel blockers and prostacyclin analogues for Raynaud
02
Bosentan and sildenafil for digital ulcers
03
Mycophenolate, cyclophosphamide or nintedanib for lung fibrosis
04
Early ACE inhibitor therapy for scleroderma renal crisis
05
Endothelin-receptor antagonists for pulmonary hypertension
06
High-dose PPIs for reflux
07
Physiotherapy and prevention of joint contractures
08
Autologous stem-cell transplant in selected cases

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Dahiliye (İç Hastalıkları) Department

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You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.