calcimycin and Scleroderma--Systemic

The aim of this study was to evaluate the function of basophil granulocytes in 37 patients with progressive systemic sclerosis (PSS). The in vitro proteinase release test using Chromozyme TH as a chromogenic substrate was performed to measure the basophil cell releasability. Anti-IgE, calcium-ionophore (A 23187) and N-formyl-methyonil-leucyl-phenylalanine were used as activatory agents. Our results showed that the reactivity pattern to those agents did not differ in PSS compared to controls. Patients whose basophil cells release proteinases to all of the 3 activators tended to have more severe organ symptoms compared to the rest of the patients, while teleangiectasia was significantly less frequently (p < 0.02) found in cases with proteinase release. These findings suggest that there may be a certain relationship between basophil function and severity of the PSS.

Topics: Adult; Aged; Basophils; Calcimycin; Endopeptidases; Female; Humans; Immunoglobulin E; Male; Middle Aged; N-Formylmethionine Leucyl-Phenylalanine; Scleroderma, Systemic

We evaluated basophil releasability in 16 female patients with scleroderma (systemic sclerosis) and in 16 normal age- and sex-matched donors. Basophils from patients with scleroderma released significantly more histamine "spontaneously" than did those from normal donors (12.9 +/- 2.1% versus 4.5 +/- 0.7%; P less than 0.0005). Basophil reactivity (maximal percentage histamine release) to anti-IgE was higher in patients with scleroderma than in controls (57.0 +/- 7.5% versus 35.4 +/- 7.8%; P less than 0.05). Basophil sensitivity (the concentration of anti-IgE that causes 40% of maximal percentage histamine release) to anti-IgE in scleroderma patients was similar to that found in controls (4.6 +/- 2.8 x 10(-2) micrograms/ml versus 2.3 +/- 1.0 x 10(-1) micrograms/ml; P not significant). Scleroderma patients also showed enhanced releasability compared with that of the controls when challenged in vitro with interleukin-3 (8.3 +/- 1.7% versus 3.2 +/- 0.6%; P less than 0.01). Releasability induced by the formyl-containing tripeptide, f-met peptide, was significantly higher in the scleroderma patients than in the controls at the 2 lower concentrations used. No differences in basophil reactivity and sensitivity to f-met peptide and calcium ionophore A23187 were found between patients and normal donors. These results show that spontaneous basophil releasability and releasability in response to IgE cross-linking and activation of interleukin-3 receptors are increased in patients with scleroderma.

Topics: Adult; Aged; Basophils; Calcimycin; Dose-Response Relationship, Drug; Female; Histamine Release; Humans; Immunoglobulin E; Interleukin-3; Middle Aged; N-Formylmethionine; Recombinant Proteins; Scleroderma, Systemic

Peripheral blood lymphocytes from patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), Reiter's disease, osteoarthritis, and from healthy volunteers were investigated for interferon-gamma (IFN-gamma) production after mitogen activation. Phytohaemagglutinin stimulation revealed an impaired IFN-gamma production in RA, SLE, and PSS but normal levels in Reiter's disease and osteoarthritis. In RA this deficiency was also seen after pokeweed mitogen, OKT3, and concanavalin A activation. No major differences were found in interleukin 2 (IL-2) production and cell proliferation. The IL-2 receptor expression was reduced on stimulated RA lymphocytes. The deficient IFN-gamma production was compensated in RA by co-stimulation of PHA or OKT3 with phorbol myristic acetate (PMA). In addition, the combination of the calcium ionophore A 23187 and PMA induced a strong IFN-gamma secretion in all patient groups and in the controls.

Topics: Adolescent; Adult; Aged; Arthritis, Reactive; Arthritis, Rheumatoid; Autoimmune Diseases; Calcimycin; Drug Synergism; Humans; Interferon-gamma; Lectins; Lupus Erythematosus, Systemic; Lymphocyte Activation; Middle Aged; Osteoarthritis; Receptors, Immunologic; Receptors, Interleukin-2; Scleroderma, Systemic; Tetradecanoylphorbol Acetate