BPI-ANCA and Long-Term Prognosis among 46 Adult CF Patients: A Prospective 10-Year Follow-Up Study.
(2012) In Clinical & Developmental Immunology 2012.- Abstract
- Introduction. Anti-neutrophil cytoplasmic antibodies specific for bactericidal/permeability-increasing protein (BPI-ANCA) are frequent in CF patients and mainly develop in response to infection with Pseudomonas aeruginosa. It is not known to what extent BPI-ANCA correlates to prognosis. Objectives. To evaluate the prognostic value of IgA-BPI-ANCA, measured at the beginning of the study, for transplantation-free survival. Methods. A cohort of 46 adult, nontransplanted CF patients was generated, 1995-1998, and characterized using Leeds criteria, lung function, and IgA-BPI-ANCA levels measured by ELISA. The cohort was followed until December 2009, using the combined endpoint of death or lung transplantation. Results. Lung function and... (More)
- Introduction. Anti-neutrophil cytoplasmic antibodies specific for bactericidal/permeability-increasing protein (BPI-ANCA) are frequent in CF patients and mainly develop in response to infection with Pseudomonas aeruginosa. It is not known to what extent BPI-ANCA correlates to prognosis. Objectives. To evaluate the prognostic value of IgA-BPI-ANCA, measured at the beginning of the study, for transplantation-free survival. Methods. A cohort of 46 adult, nontransplanted CF patients was generated, 1995-1998, and characterized using Leeds criteria, lung function, and IgA-BPI-ANCA levels measured by ELISA. The cohort was followed until December 2009, using the combined endpoint of death or lung transplantation. Results. Lung function and IgA-BPI-ANCA, but not Leeds criteria, were significantly associated with adverse outcome. No patient with normal lung function at baseline reached endpoint. Within 10 years 8/11 with high BPI-ANCA reached an endpoint compared to 3/17 ANCA-negative patients. A similar result was seen within the Leeds I group where 7 out of 9 BPI-ANCA-positive patients reached endpoint, compared to none of the 5 patients without BPI-ANCA. Conclusions. IgA-BPI-ANCA is associated with adverse outcome among Pseudomonas aeruginosa infected CF patients, suggesting that BPI-ANCA is a biomarker of an unfavourable host-pathogen interaction. (Less)
    Please use this url to cite or link to this publication:
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- author
- 						Lindberg, Ulrika
				LU
	; 						Carlsson, Malin
				LU
	; 						Löfdahl, Claes-Göran
				LU
	 and 						Segelmark, Mårten
				LU
				  
- organization
- publishing date
- 2012
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Clinical & Developmental Immunology
- volume
- 2012
- article number
- 370107
- publisher
- Hindawi Limited
- external identifiers
- 
                - wos:000313484400001
- pmid:23346184
- scopus:84872782274
 
- ISSN
- 1740-2530
- DOI
- 10.1155/2012/370107
- language
- English
- LU publication?
- yes
- id
- 46d5b3d0-fa2a-4081-87cc-0d6fe1eb5d50 (old id 3438388)
- alternative location
- http://www.ncbi.nlm.nih.gov/pubmed/23346184?dopt=Abstract
- date added to LUP
- 2016-04-01 10:05:48
- date last changed
- 2025-10-14 13:02:27
@article{46d5b3d0-fa2a-4081-87cc-0d6fe1eb5d50,
  abstract     = {{Introduction. Anti-neutrophil cytoplasmic antibodies specific for bactericidal/permeability-increasing protein (BPI-ANCA) are frequent in CF patients and mainly develop in response to infection with Pseudomonas aeruginosa. It is not known to what extent BPI-ANCA correlates to prognosis. Objectives. To evaluate the prognostic value of IgA-BPI-ANCA, measured at the beginning of the study, for transplantation-free survival. Methods. A cohort of 46 adult, nontransplanted CF patients was generated, 1995-1998, and characterized using Leeds criteria, lung function, and IgA-BPI-ANCA levels measured by ELISA. The cohort was followed until December 2009, using the combined endpoint of death or lung transplantation. Results. Lung function and IgA-BPI-ANCA, but not Leeds criteria, were significantly associated with adverse outcome. No patient with normal lung function at baseline reached endpoint. Within 10 years 8/11 with high BPI-ANCA reached an endpoint compared to 3/17 ANCA-negative patients. A similar result was seen within the Leeds I group where 7 out of 9 BPI-ANCA-positive patients reached endpoint, compared to none of the 5 patients without BPI-ANCA. Conclusions. IgA-BPI-ANCA is associated with adverse outcome among Pseudomonas aeruginosa infected CF patients, suggesting that BPI-ANCA is a biomarker of an unfavourable host-pathogen interaction.}},
  author       = {{Lindberg, Ulrika and Carlsson, Malin and Löfdahl, Claes-Göran and Segelmark, Mårten}},
  issn         = {{1740-2530}},
  language     = {{eng}},
  publisher    = {{Hindawi Limited}},
  series       = {{Clinical & Developmental Immunology}},
  title        = {{BPI-ANCA and Long-Term Prognosis among 46 Adult CF Patients: A Prospective 10-Year Follow-Up Study.}},
  url          = {{https://lup.lub.lu.se/search/files/1561814/3910464.pdf}},
  doi          = {{10.1155/2012/370107}},
  volume       = {{2012}},
  year         = {{2012}},
}