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Abstract: TH-PO726

Nephrotic Syndrome due to AA Amyloidosis Associated with Cystic Fibrosis: A Case Report

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Freire Filho, Washington Alves, Universidade de Sao Paulo Hospital das Clinicas, São Paulo, São Paulo, Brazil
  • Maia, Tassila Gomes, Universidade de Sao Paulo Hospital das Clinicas, São Paulo, São Paulo, Brazil
  • Campos, Diogo, Universidade de Sao Paulo Hospital das Clinicas, São Paulo, São Paulo, Brazil
  • Salgado, Isabela Cavalcante, Universidade de Sao Paulo Hospital das Clinicas, São Paulo, São Paulo, Brazil
  • de Menezes, Liudmila G R, Universidade de Sao Paulo Hospital das Clinicas, São Paulo, São Paulo, Brazil
  • Cavalcante, Livia Barreira, Universidade de Sao Paulo Hospital das Clinicas, São Paulo, São Paulo, Brazil
  • Yu, Luis, Universidade de Sao Paulo Hospital das Clinicas, São Paulo, São Paulo, Brazil
Introduction

Cystic fibrosis (CF) is an autosomal recessive and multisystemic disease that predominantly involves the respiratory and digestive tracts. New drugs for treatment and early recognition have recently increased survival of the patients, leading to a chronic inflammation environment, introducing renal AA amyloidosis as a possible complication.

Case Description

A 28-year-old male with cystic fibrosis, mild malnutrition and recurrent pneumonia was admitted for treatment of infectious decompensation and investigation of anasarca that started 1 month earlier. He underwent antibiotic treatment with resolution of the infectious condition. Concerning the edemigenic syndrome, echocardiogram and abdominal ultrasound indicated normal results; spot urine revealed 6.41g of protein and no hematuria or pyuria. Urine protein-creatinine ratio in isolated sample was 7.5g/g; 24-hour proteinuria: 4.56g; serum albumin of 1.8g/dL; LDL 191mg/dL; triglycerides 248mg/dL. Serologies for hepatitis B, hepatitis C and HIV, and both FAN and ANCA resulted negative. Urinary protein electrophoresis showed no alteration. Renal biopsy presented mesangial expansion with amorphous eosinophilic pale material, PAS negative, with birefringence on analysis under polarized light in sections stained with Congo red (fig1). Amyloid A antigen test was performed, which resulted positive in arteriole walls. Gathering all of this information, the patient obtained a presumptive diagnosis of AA amyloidosis secondary to CF.

Discussion

CF may rarely complicate with AA amyloidosis. Amyloid deposits occur mainly in the liver, spleen and kidneys. This complication reflects a history of recurrent infections and successive inflammatory insults. AA amyloidosis presents itself as a poor prognostic factor and patients with CF and 24h-proteinuria > 1g or nephrotic syndrome should mandatorily perform a renal biopsy.