DISEASE SCANNER

Global Incurable Diseases Tracker

Back to Globe
Autoimmune Disease

Systemic Sclerosis (Scleroderma)

HIGH SEVERITY

A chronic autoimmune disease causing fibrosis of skin and internal organs (diffuse cutaneous) or primarily skin (limited cutaneous/CREST). Characterized by vasculopathy and immune activation. Limited form associated with anti-centromere antibodies, diffuse with anti-Scl-70.

Global Affected

300.0K

Countries

15

Symptoms

Skin thickening and tightening
Raynaud's phenomenon
Esophageal dysmotility/GERD
Interstitial lung disease
Pulmonary hypertension
Renal crisis (scleroderma renal crisis)
Telangiectasias

Treatment Options

Corticosteroids
Immunosuppressants
Biologic agents
DMARDs
Plasmapheresis
IVIG
Physical therapy
Symptom management

Risk Factors

1Female gender
2Family history
3Genetic predisposition
4Environmental triggers
5Infections
6Hormonal factors
7Smoking

Diagnostic Methods

  • 1Autoantibody testing
  • 2Inflammatory markers
  • 3Imaging
  • 4Biopsy
  • 5Clinical criteria
  • 6Exclusion of other causes

Prognosis

Variable; many autoimmune diseases are chronic with relapsing-remitting courses. Early treatment and modern biologics have significantly improved outcomes.

Prevention

  • No specific prevention
  • Smoking cessation
  • Avoidance of triggers
  • Early diagnosis and treatment
  • Regular monitoring

Research Status

Nintedanib and tocilizumab for ILD. Tocilizumab and mycophenolate effective. Autologous stem cell transplant for severe diffuse disease. ACE inhibitors for scleroderma renal crisis.

Affected Countries

AustraliaBrazilCanadaChinaGermanySpainFranceUnited KingdomCroatiaIndiaItalyJapanLVThailandUnited States

Sources

  • https://www.ncbi.nlm.nih.gov/books/NBK519046
  • https://www.ncbi.nlm.nih.gov/books
  • https://www.mayoclinic.org/diseases-conditions
  • https://rarediseases.org/rare-diseases

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.