Cyclophosphamide therapy in Sweet's syndrome complicating refractory Crohn's disease — Efficacy and mechanism of action

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Background: Sweet's syndrome is a rare extraintestinal manifestation of Crohn's disease that is usually treated by corticosteroids. Cyclophosphamide therapy has been shown to be effective in steroid-refractory Crohn's disease with extraintestinal manifestations. The mechanism of action remains obscure. Here, we report about a case of steroid-refractory Sweet's syndrome accompanying Crohn's colitis treated by cyclophosphamide.

Methods: At baseline and two weeks after initiating cyclophosphamide pulse therapy, clinical symptoms were evaluated and apoptosis in mononuclear cells of the colon mucosa was quantified via immunofluorescence TUNEL-labeling. Ongoing clinical follow-up lasts for more than three years.

Results: Cyclophosphamide pulse therapy resulted in complete resolution of luminal activity and extraintestinal manifestations. TUNEL-marked CD4+, CD8+ and CD68+ cells in intestinal biopsies showed a 338% increase as compared to baseline.

Conclusions: Cyclophosphamide therapy was highly effective in steroid-refractory Crohn's colitis accompanied by Sweet's syndrome for induction of remission. Furthermore, apoptosis of mononuclear cells in the colon mucosa, including CD68+ macrophages as well as CD4+ and CD8+ cells, appears to be a component of the anti-inflammatory effect of cyclophosphamide in Crohn's disease.

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