The immunopathology of progressive systemic sclerosis (PSS)

Role of Mast Cells in Progressive Renal Diseases

“…84 The availability of mast cell-deficient and mast cell-"knock-in" (bone marrow reconstituted) mice confirm a role for mast cells in some forms of tissue fibrosis in vivo. The models include homocysteineinduced cardiac remodeling 85 and pancreatic fibrosis, 86 where the unexpected outcomes suggest mast cells protect against the development of fibrosis.…”

Endothelial cell apoptosis is a primary pathogenetic event underlying skin lesions in avian and human scleroderma.

“…During disease progression, fibrotic destruction of internal organs such as lung, heart, kidney, and the gastrointestinal tract takes place (1)(2)(3). Fibrotic stages of SSc with the full-blown clinical symptoms often fail to exhibit prominent perivascular skin infiltration, which can always be found in the early inflammatory stage of both SSc and localized scleroderma (loSc) (4).…”

“…Systemic sclerosis (SSc) 1 is a multistage autoimmune disorder characterized by three morphological hallmarks in early skin lesions: structural and functional vascular and microvascular abnormalities, perivascular and tissue infiltration of mononuclear inflammatory cells, and increased collagenous and noncollagenous extracellular matrix molecules. During disease progression, fibrotic destruction of internal organs such as lung, heart, kidney, and the gastrointestinal tract takes place (1)(2)(3).…”

The Use of Streptolysin O for the Treatment of Scars, Adhesions and Fibrosis: Initial Investigations Using Murine Models of Scleroderma

“…Scleroderma often progresses as an autoimmune disease in which the body's immune system attacks its own connective tissues (Haynes and Gershwin, 1982;Krieg and Meurer, 1988;LeRoy et al, 1988;Mayes, 1997;Sapadin et al, 2001;Haustein, 2002;Trojanowska, 2002). There are two types of scleroderma: systemic 68 S. W. Mamber et al scleroderma, which affects the internal organs, and localized scleroderma, which affects the local area of the skin.…”

“…There are two types of scleroderma: systemic 68 S. W. Mamber et al scleroderma, which affects the internal organs, and localized scleroderma, which affects the local area of the skin. The disease is characterized by excessive collagen deposition in the affected skin, as well as in various internal organs (lungs, heart, kidneys, esophagus, myocardium, and gastrointestinal tract), and vascular injury (Haynes and Gershwin, 1982;Krieg and Meurer, 1988;LeRoy et al, 1988;Tuffanelli, 1998;Hawk and English, 2001;Sapadin et al, 2001;Haustein, 2002;Mori et al, 2002;Sehgal et al, 2002). The causes of scleroderma are unknown (Artlett et al, 1999;Johnson et al, 2002;Sapadin and Fleischmajer, 2002).…”