title: [Etiopathogenesis. New concepts].
abstract: Systemic sclerosis is a complex, progressive autoimmune disease. The origin is, so far, unknown and it is characterized by immunological and endothelial damage followed by fibrosis. Interaction between the host genetic backgrounds with environmental factors is thought to turn out an abnormal immune response characterized by clonal expansion of Th2 repertoire, upregulation of pro-fibrotic cytokines and dysregulated B cells. Specific autoantibodies profiles are associated with clinical subtypes of the diseases. Endothelial activation is an early feature with damage of the vasocontrictive and vasodilation response. Finally, persistent tissue ischemia and abnormal immune response produce myofibroblast proliferation andoverproduction of extracellular matrix proteins and fibrosis.
authors.affiliation: Subdirección de Investigación Clínica. Instituto Nacional de Enfermedades Respiratorias. Tlalpan. México DF. México.
authors.forename: Carmen
authors.initials: C
authors.lastname: Navarro
authors.forename: Martha L
authors.initials: ML
authors.lastname: Bustos