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Kidney Week

Abstract: SA-PO881

Unexplained Chylous Pleural Effusions Diagnosed by Kidney Biopsy

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Jose, Aju, Northwestern University McGaw Medical Center, Chicago, Illinois, United States
  • Ludwig, Amy, Northwestern University McGaw Medical Center, Chicago, Illinois, United States
  • Ludwig, John Travis, University of Michigan, Ann Arbor, Michigan, United States
Introduction

Amyloidosis is a group of diseases characterized by extracellular protein deposition of beta-sheet fibrils. Renal amyloidosis is found in about 2% kidney biopsies and is usually secondary to systemic disorders. Here we describe an atypical case of renal AA amyloidosis presenting with chylous pleural effusions and ascites.

Case Description

Patient is a 47 year old paraplegic male with neurogenic bladder, chronic indwelling urinary catheter, chronic sacral wounds, history of osteomyelitis and malnutrition. History of presenting illness included insidious onset of shortness of breath which started 18 months prior to presentation. He was evaluated by his primary care physician who found bilateral pleural effusions and was sent to a thoracic surgeon. He had multiple serial thoracentesis underwent VATS with talc pleurodesis which was unsuccessful. Bilateral pleurX catheters were placed for symptom management. He was referred to our center due to increased drainage from his catheters, anasarca and worsening shortness of breath and consideration of lymphangiogram with interventional radiology. Labs were significant for serum albumin < 1.5 and pleural fluid triglycerides > 135 consistent with chylous efusion and UPCR > 40 g/g. Lymphangiogram showed a patent thoracic duct with no evidence of leak. Renal Biopsy showed changes of amyloidotic nephropathy. Laser dissection and tandem mass spectroscopy confirmed AA amyloid. Pleural fluid cultures revealed VRE and he was treated with daptomycin and ciprofloxacin. Supportive care was provided with nutrition management, wound care and diuresis. Drainage from left pleurX catheter decreased and was eventually removed. He continued to require frequent paracentesis. He was discharged with plans for close follow up but unfortunately passed away 14 days after discharge.

Discussion

Chylous pleural effusions and ascites is a rare presentation of amyloidosis, in this case diagnosed on kidney biopsy. This diagnosis is challenging and requires a high index of suspicion. AA amyloidosis is a rare but serious complication from underlying chronic inflammatory disorders with a sustained acute phase reactant response. Renal dysfunction with proteinuria is the most common manifestation of AA amyloidosis, with high mortality (median survival of 133 months after diagnosis)1. Management typically consists of targeting the underlying inflammatory response.