Rs. Walmsley et al., ANTINEUTROPHIL CYTOPLASM AUTOANTIBODIES AGAINST BACTERICIDAL PERMEABILITY-INCREASING PROTEIN IN INFLAMMATORY BOWEL-DISEASE/, Gut, 40(1), 1997, pp. 105-109
Background-Bactericidal/permeability-increasing protein (BPI), a const
ituent of primary neutrophil granules, is a potent natural antibiotic
and an antineutrophil cytoplasm antibody (ANCA) antigen in cases of va
sculitis in which the target antigen is neither myeloperoxidase (MPG)
nor proteinase-3 (PR3). Aim-To investigate BPI as a possible target an
tigen for ANCAs in inflammatory bowel disease. Methods-ANCAs were dete
cted by routine immunofluorescence (IIF) and solid phase enzyme Linked
immunosorbent assay (ELISA) performed for antibodies to the purified
neutrophil granule proteins; MPO, PR3, cathepsin-G, lactoferrin, and B
PI in serum samples from 88 patients with inflammatory bowel disease (
36 with Crohn's disease, 52 with ulcerative colitis). Thirty patients
with bacterial enteritis acted as controls. Results-Significantly more
patients with ulcerative colitis were ANCA positive by IIF (60%) than
patients with Crohn's disease (28%) or infectious enteritis (23%) (p<
0.001). IgG anti-BPI antibodies were present in 29% of patients with u
lcerative colitis, 14% of patients with Crohn's disease, and 23% of pa
tients with infectious enteritis, occurring in 44% of those patients w
ith inflammatory bowel disease who were ANCA positive by IIF. Antibodi
es to other ANCA antigens were rare. The presence of ANCAs was not rel
ated to either disease activity or extent; presence of anti-BPI antibo
dies was significantly related to both a lower serum albumin concentra
tion (p=0.001) and a higher erythrocyte sedimentation rate (p=0.02) in
patients with ulcerative colitis, and to colonic involvement in patie
nts with Crohn's disease (p=0.01). Conclusion-BPI is a significant min
ority Cambridge target antigen for ANCAs in inflammatory bowel disease
that seems related to colonic Crohn's disease and disease activity in
ulcerative colitis. Anti-BPI antibodies occur in infectious enteritis
.