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Abstract: TH-PO522

A Case of Fibrillary Glomerulonephritis Presenting With Asthma Responsive to Corticosteroids

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Sharifi, Bobak, University of Virginia, Charlottesville, Virginia, United States
  • Cavanaugh, Corey J., University of Virginia, Charlottesville, Virginia, United States
  • Pourafshar, Negiin, Georgetown University, Washington, District of Columbia, United States
Introduction

Fibrillary glomerulonephritis (FGN) is a rare cause of rapidly progressive glomerulonephritis (RPGN). The typical FGN clinical presentation includes renal insufficiency, hematuria, proteinuria and nephrotic syndrome. Rarely extrarenal manifestations are seen. Herein we describe a case of FGN presenting with asthma.

Case Description

A 51-year-old female with a history of celiac disease, tobacco abuse, and Hashimoto’s thyroiditis was admitted twice in a two-week period for refractory lower extremity edema, petechial rash, wheezing, and fevers. She was initially admitted to the medicine service for asthma exacerbation and bacterial pneumonia, which dramatically improved with antibiotics and corticosteroids. Unfortunately, she re-presented 2 weeks later, with recurrence of dyspnea and wheezing. Her creatinine rose to 2.5mg/dL, above a baseline of 0.8mg/dL over two weeks. Chest CT on admission showed ground glass opacities that resolved on repeat scan in three weeks. Her urinalysis was significant for large blood and protein to creatinine ratio of 5.8g/g. She had hypoalbuminemia 2.2g/dL and edema. Serologic workup including SPEP, HIV, Hepatitis C, ANCA, and Anti-GBM were negative, only ANA was positive at 1:80. Kidney biopsy was performed, and electron microscopy revealed randomly arranged fibrils ranging from 15-23nm in diameter, immunofluorescence with linear deposition of IgG, C3, fibrin, kappa and lambda light chains. A single crescent was noted in one of seventeen glomeruli. Stains for DNAJB9 showed diffuse granular staining of both the mesangial and capillary loop basement membrane within the glomeruli. She was treated with prednisone (1mg/Kg) to target asthma and glomerular disease with a 3-month taper, losartan, and an SGLT2 inhibitor. Her proteinuria decreased from 5.8g/g to 1g/g at one year follow up for partial remission. She quit smoking completely and her pulmonary symptoms resolved. Creatinine fell to 1.8mg/dL and remained stable for 2 years.

Discussion

FGN has a poor prognosis and there are no randomized controlled trials to guide optimal therapy. Our case highlights the difficulty in recognition and management of patients with RPGN as multiple etiologies with unusual presentations can provide dilemmas in management. This atypical presentation of RPGN with asthma and improvement with steroids and smoking cessation is particularly unique.