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Abstract: TH-PO526

Beguiling but Harmful: IgA Nephropathy Manifests

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Rivera, Maria Eugenia, Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
  • Vega-Colon, Jesus Daniel, Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
  • Rivera Gonzalez, Alexis, Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
  • Ocasio Melendez, Ileana E., Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
  • Andujar-Rivera, Krystahl Z., Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
Introduction

IgA nephropathy (IgAN) is the most common pattern of primary glomerular disease worldwide and remains a leading cause of CKD. Given its prevalence, attention should be paid to risk factors of progression.

Case Description

A 22-year-old female with atopic dermatitis, complains of 6-month history of worsening bilateral leg edema associated with frothy dark urine. Denied recent skin, respiratory or gastrointestinal infections. No use of NSAID’s or other nephrotoxins. No family history of renal disease. Toxic habits were remarkable for marihuana smoking. Examination revealed blood pressure 166/88 mmHg and pitting edema +2 up to shins. Laboratory results notable for anemia, BUN 25.7 mg/dL, creatinine 2.64 mg/dL, albumin 2.2 g/dL and bicarbonate 21.8 mmol/L and uric acid 5.9 mg/dL. Urinalysis with proteinuria, hematuria, granular casts and fat oval bodies. Urine protein-creatinine ratio of 8,300 mg/g, consistent with nephrotic range proteinuria. Testing for HIV, Hepatitis B and C, ANA, complements C3/C4, and serum free light chains were normal. Renal biopsy showed mesangial proliferation with crescent formation by light microscopy with IgA staining in IF. EM showed mesangial and subendothelial deposits with foot processes effacement. There was moderate glomerulosclerosis and interstitial fibrosis.
Treatment was started with prednisone 60 mg daily, furosemide, low sodium diet, losartan and atorvastatin. A month after diagnosis, patient presented with worsening renal function and edema. Despite medical treatment, patient continued to deteriorate requiring kidney replacement therapy. Unfortunately, patient did not recover and was discharged home dependent of hemodialysis.

Discussion

IgAN may present in a myriad of ways. From progressive CKD to nephritic syndrome, and all the spectrum in between. After diagnosis, one fourth of patients will progress to ESKD within 20-25 years of presentation. Our patient presented with nephrotic syndrome and rapidly progressive glomerulonephritis with multiple markers for poor prognosis like hypertension, proteinuria above 1 gm/day, and histologic predictors of disease progression such as chronic fibrotic disease, tubular atrophy and interstitial fibrosis on biopsy. Overall, the prognosis of IgAN may be difficult to predict but identifying these risk factors early may guide clinicians and patients.