AN UNUSUAL CASE OF ACUTE HYPOXIC RESPIRATORY FAILURE IN A PATIENT WITH FIBRILLARY GLOMERULONEPHRITIS

Abstract

SESSION TITLE: Fellows Critical Care Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease (incidence <1%) characterized by immune-complex mediated GN with amyloid-like fibrils which are IgG positive and Congo red negative(1). We present a case of refractory respiratory failure with co-existing FGN. CASE PRESENTATION: A 58-year-old woman with hypertension and chronic hematuria/ proteinuria (presumed IgA nephropathy but no biopsy) presented to the hospital with fever and dyspnea for 2 days. Due to moderate hypoxemia she required invasive mechanical ventilation (IMV). CXR showed bilateral diffuse airspace opacities. Lab work up showed proteinuria, hematuria, elevated CRP and normal creatinine. ANCA panel, complements, anti-GBM antibody, anti-SSA/SSB, RF and anti Scl-70 were negative. BAL showed no evidence of DAH, neutrophilic alveolitis and sterile cultures. Echocardiogram was normal. Despite lung protective IMV, PEEP adjustment as per ARDS net and adjuvant therapies (inhaled nitric oxide, steroids and paralytics), severe hypoxemia persisted. She developed AKI requiring renal replacement therapy. She received appropriate antibiotics for ESBL E. coli UTI and C. diff. colitis. Kidney biopsy showed FGN. She underwent plasma exchange, intravenous immunoglobulin therapy followed by rituximab per management protocol used in other pulmonary renal syndromes. Due to worsening of severe ARDS refractory to medical therapy, VV-ECMO was initiated in week 4. She was unable to be weaned off ECMO support (>30 days) which lead to her death. DISCUSSION: FGN was first described in 1977(2). Fibrils characterizing FGN are confined to glomeruli and stain intensely by IF for IgG, C3, ?, and ?, strongly suggesting that the fibrils are composed of a complex of antibodies and antigens as seen in our case(3). It was described in association with underlying malignancy, hepatitis C, diabetes or autoimmune disease. Nephrotic range proteinuria, hematuria and hypertension are usual presentations. Recent discovery of major fibril component, DNA-J heat-shock protein family member B9 (DNAJB9), has made the diagnosis of FGN by immunohistochemistry possible(4,5). Nearly half of patients with FGN progress to ESRD within 4 years. Benefit from immunosuppressive therapy is unestablished. Transplant is not curative as recurrence is not uncommon. Very few cases of extra-renal manifestations of FGN have been reported. Only cases associated with pulmonary hemorrhage were successfully treated with steroids(6). CONCLUSIONS: Our case reports a rare extra-renal manifestation of FGN causing refractory respiratory failure. Further research to identify exact pathogenesis and successful treatment is imperative. Reference #1: 1. Fibrillary Glomerulonephritis: Clinicopathological features and atypical cases from a multi-institutional cohort, CJASN Dec 2019, 14 (12) 1741-50 2. Nephrotic syndrome associated with amyloid-like glomerular deposits. Nephron. 1977;18:301-308 Reference #2: 3. Fibrillary Glomerulonephritis: An update, Kidney Int Rep. 2019 Jul; 4(7): 917-922 4. DNA J heat shock protein family B member 9 is a novel biomarker for fibrillary GN. J Am Soc Nephrol. 2018;29:51–56 Reference #3: 5. DNA J homolog subfamily B member 9 is a putative autoantigen in fibrillary GN. J Am Soc Nephrol. 2018; 29:231–239 6. Fibrillary Glomerulonephritis: Another cause of bleeding lung. Case reports, ATS 2017 conference DISCLOSURES: No relevant relationships by Moises Cossio, source=Web Response No relevant relationships by Domingo Franco-Palacios, source=Web Response No relevant relationships by Kushagra Gupta, source=Web Response No relevant relationships by Shahzad Hussain, source=Web Response No relevant relationships by Venkateswara Kollipara, source=Web Response No relevant relationships by Toribiong Uchel, source=Web Response