Intractable colitis associated with chronic granulomatous disease.
The case of a 20-year-old Japanese man, diagnosed as having autosomal recessive chronic granulomatous disease (CGD), who was being treated with corticosteroids for intractable unclassified colitis, is described. He died from multiple organ failure following disseminated intravascular coagulation secondary to disseminated varicella-zoster virus (VZV) infection. He was diagnosed as an index case of CGD when 2 years old, was inoculated against VZV at the age of 5 years and had had an unremarkable course for 19 years. He was admitted to hospital because of a third episode of recurrent bloody diarrhoea. Clinical remission for each episode was achieved by intravenous corticosteroid therapy. Unclassified colitis associated with CGD was diagnosed based on a colonic biopsy demonstrating characteristic macrophages with lipofuscin deposits. From a treatment viewpoint, idiopathic inflammatory bowel disease (IBD) should be differentiated from secondary IBD occurring in CGD, in which immunosuppressive drugs including corticosteroids, still the mainstay of IBD treatment, should be avoided.
Arimura
Yoshiaki
Y
Goto
Akira
A
Yamashita
Kentaro
K
Endo
Takao
T
Ikeda
Hideyuki
H
Tanaka
Kaori
K
Tsutsumi
Hiroyuki
H
Shinomura
Yasuhisa
Y
Imai
Kohzoh
K
D000328:Adult; D003092:Colitis / Q000150:complications* / Q000188:drug therapy / Q000821:virology; D004279:DNA, Viral / Q000737:chemistry / Q000235:genetics; D017809:Fatal Outcome; D006105:Granulomatous Disease, Chronic / Q000150:complications* / Q000188:drug therapy / Q000821:virology; D006562:Herpes Zoster / Q000150:complications; D014645:Herpesvirus 3, Human / Q000235:genetics / Q000254:growth & development; D006801:Humans; D008297:Male; D016133:Polymerase Chain Reaction; D012150:Polymorphism, Restriction Fragment Length; D011241:Prednisone / Q000009:adverse effects / Q000627:therapeutic use; D014775:Virus Activation
D004279:DNA, Viral; D011241:Prednisone