title: Cyclophosphamide therapy in Sweet's syndrome complicating refractory Crohn's disease − Efficacy and mechanism of action.
abstract: 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.
authors: Department of Internal Medicine I, University Hospital Schleswig-Holstein, Campus Lübeck, Germany.
authors: Christian
authors: C
authors: Meinhardt
authors: Jürgen
authors: J
authors: Büning
authors: Klaus
authors: K
authors: Fellermann
authors: Hendrik
authors: H
authors: Lehnert
authors: Klaus J
authors: KJ
authors: Schmidt
mesh_terms: D017209:Apoptosis / Q000187:drug effects; D003424:Crohn Disease / Q000150:complications* / Q000188:drug therapy*; D003520:Cyclophosphamide / Q000494:pharmacology / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000494:pharmacology / Q000627:therapeutic use*; D007413:Intestinal Mucosa / Q000187:drug effects; D008297:Male; D008875:Middle Aged; D016463:Sweet Syndrome / Q000150:complications*; D013601:T-Lymphocytes / Q000187:drug effects
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide