Cyclic Neutropenia in Crohn's Ileocolitis: Efficacy of Granulocyte Colony-Stimulating Factor

Fata, F. Ramón de; Myers, Pamela; Addeo, J; Grinberg, Mikhail; Nawabi, Ismat U.; Cappell, Mitchell S.

doi:10.1097/00004836-199706000-00015

Cited by 33 publications

(36 citation statements)

References 17 publications

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“…Genetic syndromes like chronic granulomatous disease [19], glycogen storage disease [20, 21, 22], leukocyte adhesion deficiency [41]and cyclic neutropenias [42, 43]are characterized by deficiencies in quantitative or qualitative neutrophil functions and may present with CD-like intestinal lesions. In chronic granulomatous disease and glycogen storage disease Ib, rhG-CSF treatment promoted healing of the intestinal inflammation [15, 16, 17, 18].…”

Section: Discussionmentioning

confidence: 99%

An Open-Label Pilot Study of Granulocyte Colony-Stimulating Factor for the Treatment of Severe Endoscopic Postoperative Recurrence in Crohn’s Disease

Dejaco¹,

Lichtenberger²,

Miehsler³

et al. 2003

Digestion

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Background/Aim: Recombinant human granulocyte colony-stimulating factor (rhG-CSF) promoted healing of Crohn’s disease (CD)-like intestinal lesions in chronic granulomatous disease and glycogen storage disease Ib, both characterized by defective neutrophil functions. We performed a prospective, open-label pilot study with rhG-CSF for the treatment of CD. Patients andMethods: Five patients with clinically inactive CD, but with severe endoscopic ileitis within 1 year after intestinal resection and ileocolonic anastomosis, received 300 µg of rhG-CSF (Filgrastim; Neupogen^®) subcutaneously, three times weekly for a total of 12 weeks. Safety was evaluated by assessment of clinical and laboratory data and disease activity. The primary parameter of efficacy was complete mucosal healing, as defined by the Rutgeerts score. Anti-inflammatory mediators were repeatedly measured during treatment. Results: All patients completed the protocol in clinical remission. In 1 subject transient headache resolved after halving the rhG-CSF dosage. Complete mucosal healing was observed in 2 patients: in 1 patient after 12 weeks of therapy and in 1 patient 9 months after treatment cessation. In a single patient, closure of an anovaginal and of a perianal fistula was noted. Neutrophil counts and interleukin-1 receptor antagonist and soluble tumor necrosis factor receptor p55 and p75 levels were found to be increased during drug administration. Conclusion: rhG-CSF seems to be safe, well tolerated, and might provide efficacy in CD.

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Section: Discussionmentioning

confidence: 99%

An Open-Label Pilot Study of Granulocyte Colony-Stimulating Factor for the Treatment of Severe Endoscopic Postoperative Recurrence in Crohn’s Disease

Dejaco¹,

Lichtenberger²,

Miehsler³

et al. 2003

Digestion

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“…34,35 Bowel inflammation has been documented in both the ileocecal region and the colon, with the formation of skip lesions, and in association with noncaseating granulomata. Clinical responses in these patients have been documented following administration of 5-aminosalicylates or G-CSF.…”

Section: Cyclic Neutropeniamentioning

confidence: 99%

Phagocyte dysfunction and inflammatory bowel disease

Rahman

Marks

Hayee

et al. 2008

Inflammatory Bowel Diseases

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Inflammatory bowel diseases are common chronic inflammatory disorders. The majority are idiopathic and can be broadly divided into Crohn's disease and ulcerative colitis. Their cause is unknown, but most hypotheses focus on a primary role for T-cell dysfunction. Conversely, there is a collection of congenital disorders of phagocyte function that result not only in immunodeficiency but also in noninfectious inflammatory bowel disease. In all cases, the latter is strikingly reminiscent of the clinical and pathological features of Crohn's disease. This coincides with recent work demonstrating that despite previous emphasis on adaptive immune dysfunction, patients with Crohn's disease actually possess an unusually weak acute innate inflammatory response. This review consolidates the literature on inflammatory bowel disease in congenital immunodeficiencies and considers the role of phagocyte dysfunction in Crohn's disease. Concepts about pathogenesis and treatment that can be carried across these disorders are also discussed.

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“…This could, however, in part be due to the fact that retrospective data were used, in which the IBD was underdiagnosed and thus not sufficiently monitored [27]. Two patients with cyclic neutropenia and Crohn ileocolitis were described, who remained in complete remission during GCSF therapy [9]. In GSD-1b no complete remission of the IBD was found, despite the increase in neutrophil counts.…”

Section: Discussionmentioning

confidence: 99%

Granulocyte colony-stimulating factor in glycogen storage disease type 1b. Results of the European Study on Glycogen Storage Disease Type 1

Visser

Rake

Labrune

et al. 2002

European Journal of Pediatrics

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Cyclic Neutropenia in Crohn's Ileocolitis: Efficacy of Granulocyte Colony-Stimulating Factor

Cited by 33 publications

References 17 publications

An Open-Label Pilot Study of Granulocyte Colony-Stimulating Factor for the Treatment of Severe Endoscopic Postoperative Recurrence in Crohn’s Disease

An Open-Label Pilot Study of Granulocyte Colony-Stimulating Factor for the Treatment of Severe Endoscopic Postoperative Recurrence in Crohn’s Disease

Phagocyte dysfunction and inflammatory bowel disease

Granulocyte colony-stimulating factor in glycogen storage disease type 1b. Results of the European Study on Glycogen Storage Disease Type 1

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