Abstract
We describe the detection and the growth of fibroblasts with human smooth muscle cell differentiation features from bronchoalveolar lavage (BAL) fluid of 53% of patients with scleroderma, but not from healthy controls. The binding of alpha-actin, vimentin and desmin antibodies by scleroderma lung fibroblasts exceeded that of normal adult lung fibroblasts, indicating that scleroderma lung fibroblasts express some markers of human smooth muscle cell differentiation (myofibroblasts), which may account for differences in biological behavior. A mesenchymal cell phenotype was documented by mRNA analysis, showing high expression of collagen type I and fibronectin in these cells. Fibronectin is also released in significantly higher amounts by scleroderma alveolar macrophages than by macrophages from healthy donors.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, Non-P.H.S.
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Actins / immunology
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Adult
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Antibodies / analysis
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Antibodies / metabolism
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Blotting, Northern
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Bronchoalveolar Lavage Fluid / pathology*
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Cell Differentiation
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Cell Division
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Cells, Cultured
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Collagen / genetics
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Collagen / metabolism
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Cytoskeletal Proteins / immunology
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Desmin / immunology
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Female
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Fibroblasts / metabolism
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Fibroblasts / pathology*
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Fibronectins / genetics
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Fibronectins / metabolism
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Humans
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Male
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Middle Aged
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Muscle, Smooth / chemistry
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Phenotype
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Prospective Studies
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RNA, Messenger / analysis
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RNA, Messenger / genetics
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Scleroderma, Systemic / epidemiology
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Scleroderma, Systemic / pathology*
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Vimentin / immunology
Substances
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Actins
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Antibodies
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Cytoskeletal Proteins
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Desmin
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Fibronectins
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RNA, Messenger
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Vimentin
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Collagen