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Intractable colitis associated with chronic granulomatous disease
Journal of Medical Microbiology
|October 13, 2006
Summary
This case study highlights a fatal outcome in a patient with chronic granulomatous disease (CGD) due to a varicella-zoster virus (VZV) infection. It emphasizes avoiding corticosteroids in CGD-associated colitis to prevent severe complications.
Area of Science:
- Immunology
- Gastroenterology
- Infectious Diseases
Background:
- Autosomal recessive chronic granulomatous disease (CGD) can present with gastrointestinal manifestations, including unclassified colitis.
- Corticosteroids are a primary treatment for inflammatory bowel disease (IBD), but their use in CGD requires careful consideration due to potential risks.
Observation:
- A 20-year-old Japanese male with CGD, treated with corticosteroids for intractable colitis, developed disseminated varicella-zoster virus (VZV) infection.
- The patient experienced recurrent bloody diarrhea, with episodes achieving remission via intravenous corticosteroid therapy.
- Colonic biopsy revealed macrophages with lipofuscin deposits, indicative of CGD-associated colitis.
Findings:
- The patient succumbed to multiple organ failure secondary to disseminated intravascular coagulation, triggered by disseminated VZV infection.
- Despite VZV inoculation at age 5, the patient developed a severe, disseminated VZV infection later in life.
Implications:
- Differentiating secondary IBD in CGD from idiopathic IBD is crucial for appropriate patient management.
- Immunosuppressive therapies, particularly corticosteroids, should be avoided in CGD-associated colitis due to increased risk of severe infections and complications.
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