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Kidney Week

Abstract: TH-PO0538

An Unusual Case of Crescentic IgAN That Exemplifies the Complexity of Diagnosis and Management

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Prabhakar, Sharma S., Texas Tech University Health Sciences Center School of Medicine, Lubbock, Texas, United States
  • Anwar, Muhammad Sajeel, Texas Tech University Health Sciences Center School of Medicine, Lubbock, Texas, United States
Introduction

Ig A nephropathy (IgAN) is the most common primary glomerulopathy worldwide but its presentation, course and prognosis is very varied and the co-existance with other renal diseases often complicates the diagnosis and management. Here we present a case that exemplifies such complexity and confusion .

Case Description

A 47-year-old female presented with chronic low back pain with vaginal bleeding (from a large uterine fibroid). Past medical history included chronic hypertension, heavy NSAIDs use, renal stones with episodic painful hematuria, thromboembolic events on anticoagulants but no diabetes. On admission she had mild oliguric AKI (baseline Scr 0.7 mg/dl 6 months ago), mild bilateral hydronephrosis for which ureteric stents were placed. She developed rapid renal failure (needing dialysis) and nephrotic proteinuria. The workup for secondary glomerular diseases was all negative. A renal biopsy showed crescentic IgA nephropathy with significant interstitial inflammation. ANCA vasculitis was considered and ruled out. The patient received IV pulse followed by high dose oral steroids and IV monthly cyclophosphamide. Due to severe toxicity, corticosteroids had to be rapidly tapered in 6 weeks. A month later, the patient underwent hysterectomy and stent removal. Two months later renal functions normalized completely. A repeat renal biopsy was normal except for a few mesangial IgA deposits. Currently patient is in complete remission and taking target release budesonide.

Discussion

Although the biopsy showed IgAN, the presentation was so atypical, leading to other diagnostic consderations like NSAIDs-AKI and renal vasculitis. Associated comorbidity and drug toxicity complicated the management limiting our therapeutic choices. We conclude that a liberal approach to performing renal biopsy and being innovative in therapeutic decisions, can significantly improve the prognosis of IgAN.