Analysis of leukemia inhibitory factor, type 1 and type 2 cytokine production in patients with eosinophilic fasciitis

Objective. Eosinophilic fasciitis (EF) is a scleroderma-like disease of unk nown etiology characterized by skin induration, elevated immune globulins, and peripheral eosinophilia. The hallmarks of the chronic cutaneous involve ment in this syndrome are inflammation and fibrosis of the fascia. To deter mine how the inflammatory process in EF may be regulated, we investigated t he spontaneous and mitogen induced [lipopolysaccharide (LPS), phytohemagglu tinin (PHA) or both LPS+PHA] syntheses of interleukins (IL)-2, 5 and 10, in terferon-gamma (IFN-gamma), and leukemia inhibitory factor (LIF) cytokines by peripheral blood mononuclear cells (PBMC) from 4 patients with active EF and compared them to those of 10 healthy individuals. Methods. We used a short term whole blood assay and culture supernatants we re collected after 24 h to measure the IL-2 and IFN-gamma contents and afte r 48 h to evaluate IL-5, IL-10, and LIF Supernatant cytokine concentrations were determined by ELISA. Results. All 3 patients had similar patterns of cytokine secretion. Cytokin e production did not differ between patients and controls under basal condi tions or when LPS was added to the cultures. In contrast, under PHA or LPSPHA stimulation, significantly higher amounts of all 5 cytokines were detec ted in samples from patients compared to those from controls. Conclusion. Overall, our data suggest that EF is characterized by an increa sed capacity of PBMC to produce IL-5 and IL-10, possibly leading to eosinop hilia and immune globulin overexpression. In this context, the simultaneous elevations of type 1 cytokines (IL-2 and IFN-gamma) and LIF production by the same cells may be an attempt by the immune system to limit the exacerba tion of a type 2 dominant response.