Abstract: SA-PO256
A Case of Concurrent IgA Nephropathy and Microscopic Polyangiitis
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
- Soltanpour, Kristine, Upstate University Hospital , Syracuse, New York, United States
- Caza, Tiffany Nicole, SUNY Upstate Medical University, Clay, New York, United States
- Khanna, Apurv, Upstate University Hospital , Syracuse, New York, United States
Introduction
IgA nephropathy is the most common cause of glomerulonephritis worldwide. It is rarely seen to concur with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. This is a case of a elderly female patient with an overlap of microscopic polyangiitis and IgA nephropathy responsible for acute kidney injury which responded to immunotherapy.
Case Description
An 82 year old lady with a history of hypertension and diabetes mellitus presented to the hospital for evaluation of AKI detected on routine blood work. Her creatinine was 5.3 mg/dL from a baseline of 1.2 mg/dL and she had hematuria for over 1 year. She has a past medical history of multiple autoimmune diseases, including thyroid disease, vitiligo, keratoconjunctivitis sicca, arthritis, and myopathy, and remote pancreatic adenocarcinoma status-post Whipple procedure. Review of systems was positive for fatigue, chills, arthralgias, myalgias, back pain, and dysuria. Physical exam was unremarkable. UA was significant for glycosuria (50 mg/dL), hemoglobinuria (3+), hematuria (34 RBC/hpf), and proteinuria (3.0 grams). Serology shown a positive ANA, positive ANCA with anti-MPO specificity, and hypocomplementemia (C3 48 mg/dL, C4 9 mg/dL).
A renal biopsy revealed crescentic glomerulonephritis, glomerular IgA-dominant immune complex deposition, and peritubular capillaritis in the setting of advanced arterio-nephrosclerosis. A severe, active glomerulonephritis with fibrous and fibrocellular crescents was present, concurrent with low grade immune complex deposition, which was IgA-dominant and was restricted to the mesangium. This suggested that the ANCA-associated process was predominant, with concurrent IgA nephropathy.
The patient was treated with Cytoxan and pulse steroid therapy. The patient had an acute drop in hemoglobin to 6.5 mg/dL with respiratory distress with increased oxygen requirements, and was found to have an alveolar hemorrhage. The patient was given oxygen, red cell transfusions, and plasmapheresis. There were no additional complications and the patient had improved respiratory and renal function at follow up.
Discussion
Although IgA nephropathy and ANCA-associated vasculitis do not frequently overlap clinically, recognition of concurrence is critical, as these cases are associated with aggressive disease with rapidly progressive glomerulonephritis, yet have good outcomes with immunosuppressive therapy.