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: Christian Meinhardt, Jürgen Büning, Klaus Fellermann, Hendrik Lehnert, Klaus J Schmidt
• Journal: Journal of Crohn's & colitis (J Crohns Colitis) Vol. 5 Issue 6 Pg. 633-7 (Dec 2011) ISSN: 1876-4479 [Electronic] England
• PMID: 22115387 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2011 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved.

Chemical References

• Immunosuppressive Agents
• Cyclophosphamide

Topics

• Apoptosis (drug effects)
• Crohn Disease (complications, drug therapy)
• Cyclophosphamide (pharmacology, therapeutic use)
• Humans
• Immunosuppressive Agents (pharmacology, therapeutic use)
• Intestinal Mucosa (drug effects)
• Male
• Middle Aged
• Sweet Syndrome (complications)
• T-Lymphocytes (drug effects)