Lysosomal Proteins as Autoantigens of Clinical Relevance

  • W. L. Gross
  • B. K. Flesch
  • E. Csernok
Conference paper

Abstract

Ten years ago autoantibodies directed against the cytoplasm of polymorphonuclear leukocytes (PMN) and monocytes were found in a small number of patients with non classified vasculitis [1, 2]. In 1985 a Dutch-Danish collaboration study reported on the high specificity of a subtype of these autoantibodies (anticytoplasmic antibodies, ACPA) for Wegener’s granulomatosis (WG) and on the high correlation between ACPA titer and disease activity [3]. These findings were soon corroborated by us [4] and later by other groups and were subsequently extended in a prospective study [5]. ACPA were later redesignated as antineutrophil cytoplasmic autoantibodies (ANCA) because a second fluorescence pattern with association to a morphologically defined subgroup of vasculitis was clearly demonstrated [6]. ANCA screening is routinely performed by indirect immunofluorescence (IIF). Using this technique three different staining patterns can be differentiated: the classic “cytoplasmic” pattern (cANCA), which is preferentially associated with WG; the perinuclear pattern (pANCA), which is associated with necrotizing and crescentic glomerulonephritis; and xANCA, which represents a peculiar mixture of the cANCA and pANCA patterns and is thought to be associated with chronic inflammatory bowel disease [7] (Fig. 1).

Keywords

Ulcerative Colitis Glomerular Basement Membrane Chronic Granulomatous Disease Chronic Inflammatory Bowel Disease Crescentic Glomerulonephritis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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Copyright information

© Springer-Verlag Berlin Heidelberg 1993

Authors and Affiliations

  • W. L. Gross
  • B. K. Flesch
  • E. Csernok

There are no affiliations available

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