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BPI-ANCA and long-term prognosis among 46 adult CF patients: a prospective 10-year follow-up study
Lund University and Skåne University Hospital, Lund, Sweden.
Lund University, Sweden.
Lund University and Skåne University Hospital, Lund, Sweden.
Linköping University, Department of Medical and Health Sciences, Nephrology. Linköping University, Faculty of Health Sciences. Östergötlands Läns Landsting, Heart and Medicine Centre, Department of Nephrology UHL.
2012 (English)In: Clinical and Development Immunology, no 370107Article in journal (Refereed) Published
Abstract [en]


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.


To evaluate the prognostic value of IgA-BPI-ANCA, measured at the beginning of the study, for transplantation-free survival.


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.


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.


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

Place, publisher, year, edition, pages
Hindawi Publishing Corporation, 2012. no 370107
National Category
Medical and Health Sciences
URN: urn:nbn:se:liu:diva-87623DOI: 10.1155/2012/370107ISI: 000313484400001OAI: diva2:589772
Available from: 2013-01-19 Created: 2013-01-19 Last updated: 2013-02-19

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Segelmark, Mårten
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