Nephrology Dialysis Transplantation, Vol 14, Issue 7 1723-1731, Copyright © 1999 by Oxford University Press
M Andresdottir, K Assmann, A Hoitsma, R Koene and J Wetzels
Background: Dense deposit disease (DDD) is an uncommon
cause of end-stage renal disease (ESRD). As a consequence, information on
the outcome of renal transplantation in patients with DDD comes from a
series with a limited number of patients. Methods: We
present the histological and clinical data of 13 adult patients with DDD,
who received their first allograft in our centre in the period between 1983
and 1994. Results: Renal transplant biopsies were
performed in 11 patients, at 2.9 months after transplantation (median;
range 0.1-13.8 months). The indication for taking the biopsy was in all
instances a raised serum creatinine level. Five patients also had a
significant proteinuria. In only one patient, light microscopy showed
alterations in the capillary walls suggestive of a recurrence of DDD.
However, by immunofluorescence or electron microscopy, we found glomerular
deposits compatible with a recurrence of DDD in all 11 patients. Three
patterns of glomerular C3 deposition were found: globular depositions only
in the mesangium; mesangial accumulation with linear deposits in the
capillary wall; and prominent linear presence in the capillary wall with
only a few mesangial granules. The findings by electron microscopy matched
the immunofluorescence results. The linear C3 accumulation in the capillary
wall was visible ultrastructurally as electron-dense ribbon-like
transformation of the glomerular basement membrane. Mesangial C3 deposits
were seen ultrastructurally as local electron-dense deposits in the
mesangium. Four patients showed a pronounced glomerular influx of
neutrophils, accompanied by crescents in three patients. In these three
latter patients, the recurrence of DDD was the only histological lesion. In
the other patients, the recurrence was merely a coincidence, the biopsy
demonstrating an additional histological lesion (three chronic vascular
rejection, two acute rejection, one ischaemic necrosis and two cyclosporin
A toxicity). Eight patients with a recurrence of DDD have progressed to
ESRD at an average of 14 months (range 0.2-38 months) after
transplantation. The recurrence was the sole cause of graft loss in the
three patients with crescents. The patients in whom the C3 deposits were
confined to the mesangium appeared to have a better prognosis.
Conclusions: The histological recurrence rate of DDD
is high. The histological picture is quite diverse, and in most patients
abnormalities are only found by immunofluorescence and electron microscopy.
Up to one-quarter of the patients with DDD lost their grafts because of a
recurrence. Key words: dense deposit disease;
histology; outcome; recurrence; renal transplantation
ORIGINAL ARTICLES
Renal transplantation in patients with dense deposit disease: morphological characteristics of recurrent disease and clinical outcome
Division of Nephrology and Department of Pathology, Department of Medicine, University Hospital Nijmegen, PO Box 9101, 6500 HB Nijmegen, The Netherlands; Corresponding author
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