INDIVIDUAL TEST 536

BPI-ANCA (IgG, IgA)  

Indication

Suspicion of cystic fibrosis, control of disease activity or assessment of prognosis

Sample material

Serum

• Minim. volume: 0,5 mL

Transport

• Within Sweden: room temperature
• International: cold

Method

ELISA

Reference interval

BPI IgA: <53 units negative, 53-67 borderline

BPI IgG: <38 units negative, 38-50 borderline

Result

Results are reported as negative, borderline or positive with a value.

Interpretation

Antibodies against Bactericidal/permeability-increasing protein

(BPI)-ANCA are present in various inflammatory states but have only clinical significance in cystic fibrosis. In case of cystic fibrosis, the presence of BPI-ANCA indicates infection with pseudomonas, and high levels are associated with poor lung

function.

References

• Sedivá A et al. Microbes Infect. 2003. Antineutrophil cytoplasmic antibodies directed against bactericidal/permeability-increasing protein detected in children with cystic fibrosis inhibit neutrophil-mediated killing of Pseudomonas aeruginosa. PMID: 12593970
• Carlsson M et al. Clin Exp Rheumatol. 2003. Pseudomonas-induced lung damage in cystic fibrosis correlates to bactericidal-permeability increasing protein (BPI)-autoantibodies. PMID: 14740434
• Lindberg U et al. J Immunol Res. 2015. BPI-ANCA Provides Additional Clinical Information to Anti-Pseudomonas Serology: Results from a Cohort of 117 Swedish Cystic Fibrosis Patients. PMID: 26273683
• Theprungsirikul J et al. J Transl Autoimmun. 2021 Killing three birds with one BPI: Bactericidal, opsonic, and anti-inflammatory functions. PMID: 34142075.
• Iwuji K et al. Sci Rep. 2023 Clinical significance of BPI-ANCA in patients with cystic fibrosis: a single center prospective study. PMID: 37875496

Last updated: 2025-12-05