Detection of immune deposits in skin lesions of patients with Wegener's granulomatosis.
BACKGROUND
Wegener's granulomatosis (WG) is considered a pauci-immune systemic vasculitis based on the absence of immune deposits in renal biopsies of patients with active disease. In animal models of antineutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis, immune deposits along the glomerular capillary wall are present at early stages of lesion development. These deposits are degraded rapidly, resulting in "pauci-immune" lesions.


OBJECTIVE
To test the hypothesis that immune deposits can also be detected in early lesions of patients with WG, thereby initiating an inflammatory reaction that, in time, is augmented in the presence of ANCA, resulting in pauci-immune lesions later on.


METHODS
The presence of immune deposits in skin biopsies taken within 48 hours of lesion development was investigated. Direct immunofluorescence was used to examine 32 skin biopsies for the presence of immune deposits (IgG, IgA, IgM, C3c). When possible, a comparison was made between the immunofluorescence findings in renal and skin biopsies taken at the same time.


RESULTS
Four of 11 biopsies taken at initial presentation and four of 21 biopsies taken at the onset of a relapse of WG showed IgG and/or IgA containing immune deposits in the subepidermal blood vessels. All nine renal biopsies showed pauci-immune glomerulonephritis, irrespective of the presence (n=5) or absence (n=4) of immune deposits in the skin biopsy.


CONCLUSIONS
A substantial number of skin biopsies showed immune deposits during active disease. These results could support the hypothesis that immune complexes may trigger vasculitic lesions in WG.
Brons
R H
RH
Department of Clinical Immunology, University Hospital Groningen, Groningen, The Netherlands.
de Jong
M C
MC
de Boer
N K
NK
Stegeman
C A
CA
Kallenberg
C G
CG
Tervaert
J W
JW
D000328:Adult; D000368:Aged; D000936:Antigen-Antibody Complex / Q000032:analysis*; D001706:Biopsy; D015932:Complement C3c / Q000032:analysis; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007668:Kidney / Q000276:immunology; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012867:Skin / Q000276:immunology* / Q000473:pathology; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000209:etiology
D000936:Antigen-Antibody Complex; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D015932:Complement C3c