Introduction: Dense deposit disease (DDD) is caused by the dysregulation of the alternative complement pathway.DDD exhibits histological findings of intense C3 deposition greater than other immunoreactants on immunofluorescence microscopy and electrondense deposits of the glomerular basement membrane lamina densa forming a sausage-like appearance on electron microscopy.The progression to end-stage kidney disease (ESRD) is within several years for a large portion of the patient population.Not only so, the rate of recurrence in kidney transplant recipients are high, ranging 80 to 100% in various studies, with cases noting recurrence within days to weeks post transplantation.Methods: 32-year-old female with history of ESRD due to biopsy proven dense deposit disease (DDD), s/p living related donor kidney transplant (mother) on 6/29/2016.Post transplant course was complicated by early recurrent DDD at 1-month post-transplant.Factor H autoantibody was elevated to 53 units/ml and C3 nephritic factor was high.The patient underwent apheresis and was started on eculizumab q2wk infusions, in addition to immunosuppression.Unfortunately missed a few infusion doses, developed AKI, continued on eculizumab.Repeat kidney biopsy was performed to rule out rejection, showed recurrent dense deposit disease as shown below with modest C3 staining, no active crescents, evidence of acute tubular injury with 24% glomerular obsolescence and no evidence of rejection.
Deshpande et al. (Wed,) studied this question.