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The nature of colitis in chronic granulomatous disease
Michela G Schäppi1, Nigel J Klein, Keith J Lindley
1Departments of Gastroenterology, dagger Immunology, and double dagger Histopathology, Institute of Child Health and Great Ormond Street Hospital, London, UK.
Background:
Patients with chronic granulomatous disease (CGD) may have gastrointestinal manifestations, commonly colitis. The etiology, prevalence, and inflammatory process of CGD colitis are unclear.
Objectives:
To characterize the inflammatory process of CGD colitis and to compare it with other inflammatory bowel disorders.
Methods:
Colonic mucosal biopsies from 8 CGD patients were immunostained for eosinophils, neutrophils, macrophages, and adhesion molecules (ICAM; VCAM, E-selectin) and compared with normal and diseased controls (allergic colitis, ulcerative colitis, and melanosis coli). Cell types were counted and expressed as cell/mm2.
Results:
The inflammatory infiltrate in CGD colitis differed from the normal controls by an increase in eosinophils (110; 48-176 [median and range] versus 14.5; 3-30; P < 0.005) and macrophages (291.5; 203-480 versus 38.5; 27-64; P < 0.005). There was a paucity of neutrophils compared to ulcerative colitis (10; 0-101 versus 315.5; 78-688; P < 0.005). Expression of HLA-DR was increased in the epithelium and vascular endothelium in CGD compared with normal controls. Patterns of expression of the adhesion molecules differed significantly in CGD from those in other inflammatory bowel diseases: intracellular adhesion molecule-1 was more strongly expressed in the lamina propria, vascular adhesion molecule-1 was more patchily expressed, and E-selectin was present only in the small vessels.
Conclusions:
The mechanism of inflammation and profile of inflammatory mediators in CGD colitis differs from that in other inflammatory bowel diseases.