Abstract: SA-PO214
Eculizumab Treatment in Recurrent Dense Deposit Disease
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Vu, Duy T., University of Oklahoma Heath sciences center, Yukon, Oklahoma, United States
- Ghata, Joe, OUHSC, Oklahoma City, Oklahoma, United States
Introduction
Dense deposit disease (DDD) is a rare complement-mediated glomerular disease typically affecting children and young adults. About 50% of affected patients develop end stage renal disease by 10 years. Treatment decisions are primarily based on case reports, expert opinion, etc. Terminal complement proteins are thought to be involved in the pathogenesis of DDD. Eculizumab, an anti-C5 antibody, prevents the formation of the terminal complement complex, however the efficacy with this drug has been mixed.
Case Description
We present a 23 years old female with DDD diagnosed at age 10. She underwent cadaveric renal transplant at age 19. A year later, she developed biopsy proven recurrent DDD in her renal allograft. She also had elevation in the C5-9 complex (328 ng/mL), creatinine (1.65 mg/dL), and protein/creatinine ratio (6.1 grams). She was started on Eculizumab 900 mg weekly injection for 4 weeks and then 1200 mg every two weeks for 16 months. Biochemical markers normalized after 1 dose without adverse events. She was in clinical remission from DDD for 18 months when her creatinine (baseline 1-1.2 mg/dL) rose and peaked at 2.59 mg/dL with nephrotic range proteinuria (pro/cr ratio was 12 grams). Biopsy of her renal allograft again revealed recurrent DDD. She had elevated plasma Ba fragment and soluble C5b-9 and low C3, C4, and C5 which are consistent with abnormal activation of the alternative pathway. The alternative pathway functional assay was low and is consistent with the consumption of the above complements. The complement Bb level was borderline elevated. The C3Nef, C4Nef, C5Nef, and Nef activity assay were all negative. Complement factor I, factor H, and properdin levels were normal. In summary, these results support the recurrence of DDD. Subsequently, the patient was given Eculizumab 1200 mg every two weeks with rapid improvement in peripheral edema, creatinine, and proteinuria.
Discussion
Our patient suffered from DDD, a rare and incurable kidney disease, but demonstrated quick and effective response to eculizumab. Eculizumab induced remission in our patient. The natural history of this disease has a high recurrence rate which was evident in our patient. However, it is promising that she demonstrated clinical response despite relapse. This case suggests eculizumab may be effective in treating recurrent DDD and preserving the renal allograft.