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Bactericidal Permeability Increasing Protein (BPI)-ANCA markiert chronische entzündliche Darmerkrankungen und entzündliche hepatobiliäre Erkrankungen

Bactericidal permeability increasing protein (BPI)-ANCA — a marker of chronic inflammatory bowel diseases and inflammatory hepatobiliary diseases

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Zusammenfassung

□ Hintergrund

Bactericidal permeability increasing protein (BPI) ist ein antibakteriell wirksames Granulozytenprodukt, das Zielantigen für Antineutrophilenzytoplasma-Antikörper (ANCA) sein kann. Die klinischen Assoziationen von Autoantikörpern gegen BPI (BPI-ANCA) sind weitgehend unklar.

□ Patienten und Methoden

Es wurden 587 Seren von Patienten mit chronischen entzündlichen Darmerkrankungen, entzündlichen hepatobiliären Erkrankungen, primären systemischen Vaskulitiden und anderen entzündlichen rheumatischen Erkrankungen mittels indirektem Immunfluoreszenztest und monospezifischem ELISA auf BPI-ANCA untersucht.

□ Ergebnisse

Die Prävalenz von BPI-ANCA betrug 43% bei der Colitis ulcerosa, 23% beim Morbus Crohn, 35% bei der primären sklerosierenden Cholangitis, 25% bei der primären biliären Leberzirrhose und 29% bei autoimmunen Hepatitiden. Die Prävalenz bei einem Spektrum von systemischen Vaskulitiden, entzündlichen Gelenkerkrankungen und Kollagenosen lag bei lediglich 3 bis 11%. Im Gegensatz zu PR3-ANCA und MPO-ANCA zeigte BPI-ANCA keine Assoziation zu einem bestimmten Färbemuster im Immunfluoreszenztest auf äthanol- und formalinfixierten Granulozyten.

□ Schlußfolgerung

Diese Studie zeigt, daß BPI-ANCA neben PR3-ANCA und MPO-ANCA die dritte immundiagnostisch nutzbare ANCA-Spezifität mit einem begrenzten Spektrum von klinischen Assoziationen ist. Die diagnostische und prognostische Relevanz von BPI-ANCA bei den beschriebenen Erkrankungen wird derzeit prospektiv untersucht.

Summary

□ Background

Bactericidal permeability increasing protein (BPI) is an antibacterial product of neutrophilic granulocytes that can serve as target antigen for antineutrophil cytoplasmic antibodies (ANCA). The clinical associations of autoantibodies angainst BPI (BPI-ANCA) are essentially unclear.

□ Patients and Methods

587 sera from patients with chronic inflammatory bowel diseases, inflammatory hepatobiliary diseases, primary systemic vasculitides and other rheumatological diseases were examined for BPI-ANCA by monospecific ELISA and a standard indirect immunofluorescence test for ANCA.

□ Results

The prevalence of BPI-ANCA was 43% in ulcerative colitis, 23% in Crohn’s disease, 35% in primary sclerosing cholangitis, 25% in primary biliary cirrhosis and 29% in autoimmune hepatitides. In a spectrum of systemic vasculitides, inflammatory joint diseases and collagen vascular diseases the prevalence was only 3 to 11%. In contrast to PR3-ANCA and MPO-ANCA, BPI-ANCA was not associated with a particular pattern of fluorescence in the immunofluorescence test on ethanol- and formalin-fixed neutrophils.

□ Conclusion

This study shows that BPI-ANCA is the third ANCA specificity, besides PR3-ANCA and MPO-ANCA, with a limited spectrum of clinical associations. The diagnostic and prognostic relevance of BPI-ANCA in the above clinical conditions is being examined prospectively.

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Authors and Affiliations

  1. Poliklinik für Rheumatologie an der Medizinischen Universität Lübeck und Akademisches Lehrkrankenhaus der Medizinischen Universität Lübeck an der Rheumaklinik Bad Bramstedt GmbH, Deutschland

    Wolfgang L. Gross

  2. Rheumaklinik Bad Bramstedt, Oskar-Alexander-Straße 26, D-24572 Bad, Bramstedt, Deutschland

    Armin Schnabel

Authors
  1. Armin Schnabel
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  2. Elena Csernok
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  3. Hendrik Schultz
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  4. Markus Stoffel
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  5. Christian Herzberg
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  6. Stephan F. Carroll
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  7. Wolfgang L. Gross
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Herrn Professor Dr. Hans-Peter Schuster zum sechzigsten Geburtstag

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Schnabel, A., Csernok, E., Schultz, H. et al. Bactericidal Permeability Increasing Protein (BPI)-ANCA markiert chronische entzündliche Darmerkrankungen und entzündliche hepatobiliäre Erkrankungen. Med. Klin. 92, 389–393 (1997). https://doi.org/10.1007/BF03042568

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