1999
DOI: 10.2169/internalmedicine.38.612
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Abstract: We report a 15-year-old Japanese girl with severe systemic Weber-Christian disease (WCD) who presented with acute onset of high fever associated with tender subcutaneous nodules. Laboratory tests showed an elevated serum concentration of lactate dehydrogenase (LDH), leukopenia, and coagulation abnormalities. The anti-nuclear and anti-DNAantibodies were negative, and the serum pancreatic enzymesand alpha 1-antitrypsin levels were normal. Pulse steroid therapy was not effective, and eventually cerebellar hemorrh… Show more

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Cited by 37 publications
(43 citation statements)
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“…Usuki et al (8) has successfully treated WCDby CyA and additional reports followed since then. In this aspect, Iwasaki et al (9) reported the successful treatment of WCD with CyAwith normalization of increased serum interferon y (IFN-y) and soluble IL-2 receptor suggesting that T cell-mediated immuneresponse is involved in the pathogenesis of WCD as shownin this issue.…”
mentioning
confidence: 96%
“…Usuki et al (8) has successfully treated WCDby CyA and additional reports followed since then. In this aspect, Iwasaki et al (9) reported the successful treatment of WCD with CyAwith normalization of increased serum interferon y (IFN-y) and soluble IL-2 receptor suggesting that T cell-mediated immuneresponse is involved in the pathogenesis of WCD as shownin this issue.…”
mentioning
confidence: 96%
“…The mechanism of action of cyclosporine in SPTL is thought to be down regulation of cytokines as serum levels of interferon gamma and soluble interleukin-2 receptor were elevated during the active disease but normalized after cyclosporine [43]. Several reports suggest that SPTCL patients without HPS may benefit from cyclosporine and steroids [2,44].…”
Section: Discussionmentioning
confidence: 99%
“…Iwasaki et al 13) reported a patient with WCD showing increased serum levels of soluble IL 2 receptor, IFN g, IL 6, IL 4 and IL 10, who was successfully treated with cyclosporine A. Hojo et al 14) also reported a patient with WCD associated with myelodysplastic syndrome, in whom the levels of soluble IL 2 receptor, IFN g, IL 1b, IL 6 and tumor necrosis factor a were elevated during the active state and then returned to normal after prednisolone therapy. Pongratz et al 12) reported a patient with WCD successfully treated with cyclosporine A. T cell immune response may be involved in the pathogenesis of WCD.…”
Section: Discussionmentioning
confidence: 99%