Juvenile systemic sclerosis.
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Biomedical subjects
Publications and source records attributed to E Martinez-Cordero.
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T cells from 18 untreated SLE patients produced significantly more B cell growth factor (BCGF) than did those from normal subjects. Those from SLE patients with active disease produced significantly more than did those from patients with inactive disease. The response to BCGF of SAC-stimulated B lymphocytes from SLE patients was higher than that of B lymphocytes from normal individuals. Similarly preactivated B cells from five of seven SLE patients also proliferated upon the addition of interleukin 1 (IL-1) whereas those of normal subjects did not. Simultaneous addition of IL-1 and BCGF had a synergistic proliferative effect on B cells from two of seven SLE patients but not on any of the controls. Interleukin 2 (IL-2) had no proliferative effect in either SLE or normal B cells. Supernatant fractions from T cells of seven of 10 patients with active SLE and three of 10 with inactive SLE induced more IgG production by CESS cells than did those of normal subjects indicating a higher production of B cell differentiation factor by SLE T cells than by those of controls. Our findings may explain the reported preactivation and predifferentiation of peripheral blood B cells from SLE patients and give insight into the mechanisms leading to the production of autoantibodies in this disease.
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Interleukin-1 (IL-1) is a monocyte derived factor that participates in immune regulation and in the regulation of fibroblast proliferation and collagen deposition. It therefore, seems particularly pertinent to study in scleroderma, a disorder of immune regulation where increased collagen deposition is a hallmark. The production of IL-1 by lipopolysaccharide stimulated monocytes from 18 untreated scleroderma patients was akin to that of their normal matched controls. However, the unstimulated monocytes from six of the 18 scleroderma patients released IL-1 activity spontaneously into their supernatants. All six patients with spontaneous IL-1 release had less than 5 years disease duration. The response to IL-1 by T lymphocytes from patients with scleroderma was low as compared to those from controls. The presence of spontaneous IL-1 production with decreased response to IL-1 in scleroderma may indicate an in vivo pre-activation of monocytes to produce this factor that might have a bearing in the pathogenesis of collagen deposition in scleroderma.
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