Renal transplantation in patients with dense deposit disease: morphological characteristics of recurrent disease and clinical outcome

Background. Dense deposit disease (DDD) is an uncommon cause of end-stage r enal disease (ESRD). As a consequence, information on the outcome of renal transplantation in patients with DDD comes from series with a limited numbe r 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 b etween 1983 and 1994. Results. Renal transplant biopsies were performed in 11 patients, at 2.9 mo nths after transplantation (median; range 0.4-13.8 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, lig ht microscopy showed alterations in the capillary walls suggestive of a rec urrence of DDD. However, by immunofluorescence or electron microscopy, we f ound glomerular deposits compatible with a recurrence of DDD in all 11 pati ents. Three patterns of glomerular C3 deposition were found: globular depos itions only in the mesangium; mesangial accumulation with linear deposits i n the capillary wall; and prominent linear presence in the capillary wall w ith only a few mesangial granules. The findings by electron microscopy matc hed the immunofluorescence results. The linear C3 accumulation in the capil lary wall was visible ultrastructurally as electron-dense ribbon-like trans formation of the glomerular basement membrane. Mesangial C3 deposits were s een ultrastructurally as local electron-dense deposits in the mesangium. Fo ur patients showed a pronounced glomerular influx of neutrophils, accompani ed by crescents in three patients. In these three latter patients, the recu rrence 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 wi th 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 cau se 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 p rognosis. Conclusions, The histological recurrence rate of DDD is high. The histologi cal picture is quite diverse, and in most patients abnormalities are only f ound by immunofluorescence and electron microscopy. Up to one-quarter of th e patients with DDD lost their grafts because of a recurrence.