Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive sclerosis, affecting the skin and the internal organs, vasculopathy, and immune dysregulation. Interstitial pneumonias are a group of diffuse parenchymal lung diseases (DPLDs), or also called interstitial lung diseases (ILDs). ILD is a leading complication of SSc and is a leading cause of mortality in patients with SSc. The pathogenesis of lung injury involves three major processes - persistent endothelial cell damage, immune system activation, and fibroblast activation leading to the accumulation of extracellular matrix and tissue damage. A comprehensive approach is required to properly diagnose and to select appropriate therapy.
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