Abstract: SA-PO237
IgA Dominant Membranoproliferative Glomerulonephritis
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
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Bechtold, Lance, Banner University Med Center Tucson, Tucson, Arizona, United States
- Aranas, Jennifer, University of Arizona, Tucson, Arizona, United States
- Alsamkari, Mhd kussay, Banner, Tempe, Arizona, United States
- Bracamonte, Erika R., University of Arizona, Tucson, Arizona, United States
- Koppula, Sireesha, University of Arizona, Tucson, Arizona, United States
Introduction
IgA Nephropathy is widely accepted as the most common cause of glomerulonephritis worldwide. Far less common and somewhat controversial is IgA Dominant Membranoproliferative Glomerulonephritis. Most mentions of this unique pathology are described in case studies with suggested etiologies including: cirrhosis, burn victims, and children.
Case Description
We present a case of a 74 year old woman who presented with acute encephalopathy after one month of cough and URI like symptoms. She had recently returned from a family gathering where she was exposed to several sick contacts. The patient had prior history of CKD-3b, hypertension, and carcinoid tumor, for which she was on active treatment with octreotide for several years. Initial labs were significant for acute kidney injury with serum creatinine of 2.67mg/dL and urine protein to creatinine ratio of 18,134mg/g. She was initially treated with IV fluids and antibiotics with no improvement to her renal function. She underwent renal biopsy which demonstrated the characteristic features of MPGN on light and electron microscopy along with a notable predominance of IgA on immunofluorescence microscopy. She eventually required hemodialysis and pulse dose IV steroids followed by tapering course of oral steroids. Given the paucity of reported cases, along with the lack of proven treatment strategies and the issue of her active carcinoid tumor, our treatment options were limited to steroids. She was referred for re-evaluation and was cleared of carcinoid. She then began a trial of oral cyclophosphamide (CPA). After two weeks of treatment, she developed issues of recurrent E.coli UTI and later development of community acquired pneumonia, forcing us to stop CPA. She continued on hemodialysis for a total of three months before renal recovery allowed her to stop dialysis. At last follow up, the patient had returned to her baseline serum creatinine of 1.2 mg/dL and had improvement of her proteinuria with urine protein/creatinine ratio of 5121mg/g. She continued to have intermittent episodes of frank hematuria. The patient had not undergone repeat biopsy. She was pending infectious clearance to resume her course of CPA.
Discussion
Given the lack of proven treatment modalities, we felt that this case presented an opportunity to share a reasonable and effective treatment option for future patients.