Abstract: PUB181
AKI After Eculizumab Interruption in a Case of C3 Glomerulopathy
Session Information
Category: Glomerular Diseases
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Elshirbeny, Mostafa Fottoh, Hamad Medical Corporation, Doha, Ad Dawhah, Qatar
- Hussain, Mohammed Ezzat, Hamad Medical Corporation, Doha, Ad Dawhah, Qatar
- Abuhelaiqa, Essa, Hamad Medical Corporation, Doha, Ad Dawhah, Qatar
- Nauman, Awais, Hamad Medical Corporation, Doha, Ad Dawhah, Qatar
Introduction
<div style="direction: ltr;">C3 glomerulopathy (C3G) is a newly recognized rare disease characterized by predominantly glomerular deposition of complement C3. Treatment with the C5 complement inhibitor eculizumab may be a therapeutic option but due to rarity of the disease, predicting tools of the outcomes remain largely unknown.</div> <div style="direction: ltr;"> </div>
Case Description
<div style="direction: ltr;">Here we report 25-year old female patient who was referred to nephrology clinic with renal impairment, hematuria, and proteinuria. Kidney biopsy results revealed membranoproliferative changes with predominant C3 deposits, suggestive of C3 glomerulopathy. Genetic testing revealed two unrelated mutation in C3 gene, likely not related to C3G. Patient was responding well to oral steroid and MMF with remission of proteinuria and normalized serum creatinine. She was relapsed again, 1 year later with hematuria and nephrotic proteinuria. Steroids and MMF were resumed with no response then started on eculizumab, after which she achieved partial remission with reduction in serum creatinine and urine protein. During next 2 years, patient missed eculizumab in 2 occasions. The first, when she missed one dose followed by mild rise in serum creatinine which improved after eculizumab resuming and few months later she missed two doses then presented with severe AKI requiring dialysis. Was started on steroids and eculizumab was resumed with no improvement in kidney function and patient still dialysis dependent. </div>
Discussion
<div style="direction: ltr;">Despite looks like safe and valuable therapeutic option in patients with C3G but the response to eculizumab is heterogeneous and when to discontinue the therapy still unsolved problem as transient interruption of the therapy sometimes complicated with AKI which may be severe enough to end with ESRD like this case. </div>