Extended report
Detection of immune deposits in skin lesions of patients with
Wegener's granulomatosis
R H Bronsa, M C J M de Jongb, N K de Boerc, C A Stegemand, C G M Kallenberga, J W Cohen Tervaerta d
a Department
of Clinical Immunology, University Hospital Groningen, Groningen, The
Netherlands, b Department of Dermatology,
University Hospital Groningen, c Department of Pathology, University Hospital
Groningen, d Department
of Nephrology, University Hospital Groningen
Correspondence to: Professor C G M Kallenberg, Department of Clinical Immunology, University Hospital Groningen, Hanzeplein 1, 9713 GZ Groningen, The Netherlands c.g.m.kallenberg{at}int.azg.nl
Accepted for publication 14 May 2001
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.
OBJECTIVETo
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.
METHODSThe 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.
RESULTSFour 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.
CONCLUSIONA
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.
© 2001 by Annals of the Rheumatic Diseases
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