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Pseudomonas-induced lung damage in cystic fibrosis correlates to bactericidal-permeability increasing protein (BPI)-autoantibodies

Carlsson, A; Eriksson, Leif LU ; Erwander, I; Wieslander, J and Segelmark, Mårten LU (2003) In Clinical and Experimental Rheumatology 21(Suppl. 32). p.95-100
Abstract
Objective. Lung damage is the most common cause of death in cystic fibrosis (CF). It is induced by bacterial colonization and inflammatory activity perpetuates its course. Autoantibodies directed against BPI (bactericidal permeability increasing protein), called BPI-ANCA, have recently been associated with cystic fibrosis. Here we confirm this association and evaluate the relation between ANCA and total IgG level as they relate to bacterial colonization, pulmonary function, and musculoskeletal symptoms. Methods. BPI-ANCA, MPO-ANCA, and PR3-ANCA were measured with ELISA in 46 adult patients with CF Total IgG was determined by immunoturbidimetry. Results were correlated to bacterial colonization, lung function and musculoskeletal symptoms.... (More)
Objective. Lung damage is the most common cause of death in cystic fibrosis (CF). It is induced by bacterial colonization and inflammatory activity perpetuates its course. Autoantibodies directed against BPI (bactericidal permeability increasing protein), called BPI-ANCA, have recently been associated with cystic fibrosis. Here we confirm this association and evaluate the relation between ANCA and total IgG level as they relate to bacterial colonization, pulmonary function, and musculoskeletal symptoms. Methods. BPI-ANCA, MPO-ANCA, and PR3-ANCA were measured with ELISA in 46 adult patients with CF Total IgG was determined by immunoturbidimetry. Results were correlated to bacterial colonization, lung function and musculoskeletal symptoms. Results. BPI-ANCA was found in 33 patients. In the whole group, both BPI-ANCA and total IgG were inversely correlated to lung function, but in patients chronically colonized with Pseudomonas aeruginosa (P. aeruginosa), BPI-ANCA alone was correlated to lung damage (p = 0.01). Median lung function, measured as forced expiratory volume in I second, in P. aeruginosa colonized patients with high levels of BPI-ANCA was 43% of the predicted value. In BPI-ANCA negative, the corresponding figure was 83%. In patients not colonized with P. aeruginosa, this relation was less evident. No correlation between ANCA and musculoskeletal symptoms was seen. Conclusion. P. aeruginosa induced lung damage in CF patients is associated with the presence of BPI-ANCA. P. aeruginosa colonized patients without BPI-ANCA have almost normal lung function. We suggest that BPI-ANCA discriminate P. aeruginosa colonized CF patients with severe lung damage from those whose disease is less destructive. Vasculitis like symptoms in CF are not ANCA associated. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
antineutrophil cytoplasmatic, cystic fibrosis, Pseudomonas aeruginosa, immunoglobulin G, antibodies, spirometry
in
Clinical and Experimental Rheumatology
volume
21
issue
Suppl. 32
pages
95 - 100
publisher
Pacini
external identifiers
  • wos:000187627400014
  • scopus:0348143305
ISSN
1593-098X
language
English
LU publication?
yes
id
98041526-53a8-4d39-823f-ebc201b99158 (old id 291119)
alternative location
http://www.clinexprheumatol.org/pdf/vol21/s32/s32_pdf/14carlsson.pdf
date added to LUP
2007-08-27 14:23:07
date last changed
2018-05-29 10:57:47
@article{98041526-53a8-4d39-823f-ebc201b99158,
  abstract     = {Objective. Lung damage is the most common cause of death in cystic fibrosis (CF). It is induced by bacterial colonization and inflammatory activity perpetuates its course. Autoantibodies directed against BPI (bactericidal permeability increasing protein), called BPI-ANCA, have recently been associated with cystic fibrosis. Here we confirm this association and evaluate the relation between ANCA and total IgG level as they relate to bacterial colonization, pulmonary function, and musculoskeletal symptoms. Methods. BPI-ANCA, MPO-ANCA, and PR3-ANCA were measured with ELISA in 46 adult patients with CF Total IgG was determined by immunoturbidimetry. Results were correlated to bacterial colonization, lung function and musculoskeletal symptoms. Results. BPI-ANCA was found in 33 patients. In the whole group, both BPI-ANCA and total IgG were inversely correlated to lung function, but in patients chronically colonized with Pseudomonas aeruginosa (P. aeruginosa), BPI-ANCA alone was correlated to lung damage (p = 0.01). Median lung function, measured as forced expiratory volume in I second, in P. aeruginosa colonized patients with high levels of BPI-ANCA was 43% of the predicted value. In BPI-ANCA negative, the corresponding figure was 83%. In patients not colonized with P. aeruginosa, this relation was less evident. No correlation between ANCA and musculoskeletal symptoms was seen. Conclusion. P. aeruginosa induced lung damage in CF patients is associated with the presence of BPI-ANCA. P. aeruginosa colonized patients without BPI-ANCA have almost normal lung function. We suggest that BPI-ANCA discriminate P. aeruginosa colonized CF patients with severe lung damage from those whose disease is less destructive. Vasculitis like symptoms in CF are not ANCA associated.},
  author       = {Carlsson, A and Eriksson, Leif and Erwander, I and Wieslander, J and Segelmark, Mårten},
  issn         = {1593-098X},
  keyword      = {antineutrophil cytoplasmatic,cystic fibrosis,Pseudomonas aeruginosa,immunoglobulin G,antibodies,spirometry},
  language     = {eng},
  number       = {Suppl. 32},
  pages        = {95--100},
  publisher    = {Pacini},
  series       = {Clinical and Experimental Rheumatology},
  title        = {Pseudomonas-induced lung damage in cystic fibrosis correlates to bactericidal-permeability increasing protein (BPI)-autoantibodies},
  volume       = {21},
  year         = {2003},
}