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Kidney Week

Abstract: SA-PO894

Recurrent Dialysis-Dependent Rapidly Progressive Glomerulonephritis

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Stewart, Alexandra, Brooke Army Medical Center, Fort Sam Houston, Texas, United States
  • Dande, Gabriela, The University of Texas Health Science Center at San Antonio, San Antonio, Texas, United States
Introduction

IgA nephropathy is the most common primary glomerulonephritis. Clinical presentations and kidney outcomes are variable, ranging from asymptomatic microscopic hematuria to nephrotic syndrome with rapidly progressive glomerulonephritis (less than 10 percent).

Case Description

A 69-year-old male with PMHx of microscopic hematuria with negative urologic evaluation and no prior nephrology evaluation, T2DM, HTN, small vessel disease with history of CVA was admitted to the hospital after a fall with rib fractures and a L hemopneumothorax complicated by MRSA empyema s/p thoracotomy. After 3 weeks, he developed nephrotic syndrome with anuric renal failure and volume overload.
Physical exam was notable for elevated blood pressures and anasarca. Urine microscopy revealed 40-50 RBC/hpf with at least 30% dysmorphic. Serologic workup was negative for monoclonal protein with normal FLC ratio, vasculitis panel negative for ANCA, negative HIV/ RPR/ Hepatitis B and C, and normal complements. Had 6 g proteinuria on 24 hour urine collection.
Prednisone was started at 1 mg/kg/day and renal biopsy was obtained with findings of IgA nephropathy (Oxford score: M1, E1, S0, T0, C1) and diabetic glomerulosclerosis with extensive foot process effacement. Initially had good response to steroids and diuretics, but developed worsening volume overload and was started on hemodialysis. Required a few sessions of HD with renal recovery and was discharged home on prednisone.
1 week after discharge, patient presented again to the hospital for symptomatic hypoglycemia and was found to have worsening renal failure. He was again started on dialysis for uremic symptoms and another renal biopsy was obtained. Biopsy findings were consistent with RPGN with IgA nephropathy (Oxford score: M1, E1, S0, T0, C2) and <10% IF/TA. Despite pulse steroids and 4 cycles of cyclophosphamide, he had no evidence of renal recovery and declared ESKD after 3 months of HD; CYC therapy was discontinued and he was started on a rapid steroid taper.

Discussion

Rapid progression to ESKD in uncommon in patients with IgAN and prognosis is generally poor. Treatment has not been evaluated in randomized trials; and observational trials have used a treatment approach similar to those in idopathic RPGN. We hope to demonstrate the challenges and the need for early intervention/ treatment approach in RPGN in IgAN.