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Scleroderma Lung Disease

  • Brent W. Kinder
Chapter
Part of the Respiratory Medicine book series (RM)

Abstract

The hallmarks of systemic sclerosis (scleroderma) are autoimmunity and inflammation, widespread vasculopathy affecting many vascular beds, and progressive interstitial and perivascular fibrosis. The most commonly used classification system divides the disorders based on the extent of skin involvement into limited or diffuse. The diffuse form of the disease is regularly accompanied by internal organ involvement including the lungs, with prevalence estimates of up to 80%. Diffuse interstitial lung disease is the most widespread pulmonary manifestation followed by pulmonary hypertension. These two manifestations can occur in isolation or together. In general the degree of pulmonary involvement by scleroderma is not correlated with the extent of extra-pulmonary involvement, and early pulmonary involvement is often asymptomatic. The prognostic significance of lung involvement in scleroderma is illustrated by the fact that it is now the leading cause of death in this patient population. Treatment is typically directed at suppression of the immune system and efficacy has been demonstrated in scleroderma-associated interstitial lung disease.

Keywords

scleroderma systemic sclerosis interstitial lung disease epidemiology pulmonary autoimmunity 

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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Brent W. Kinder
    • 1
  1. 1.Department of Internal MedicineUniversity of Cincinnati School of MedicineCincinnatiUSA

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