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Abstract: PO1522

A Case of Necrotizing Crescentic Glomerulonephritis due to ANCA Vasculitis and Fibrillary Glomerulonephritis

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation


  • Nasim, Munahal, Nishtar Medical College and Hospital, Multan, Punjab, Pakistan
  • Babar, Faizan, Nephrology Associates Lexington, Lexington, Kentucky, United States

Fibrillary Glomerulonephritis is a rare disorder thought to be idiopathic in nature. Cases of fibrillary GN have been assoicated with malignancy, monoclonal gammopathy, autoimmune disease, or infection. We present a unique case of fibrillary GN with pauc-immune cresentic GN due to Myleoperoxidase antibody (MPO) vasculitis.

Case Description

A 77 year old lady with history of well controlled diabetes, hypertension, congenital deafness, presented with weakness, fatigue & weight loss for four weeks. She was noted to have severe anemia with Hemoglobin of 6.6 g/dl, and presumed acute kidney injury. Her labs on admission were remarkable for creatinine 2.5mg/dl (unclear baseline creatinine), eGFR 25ml/min, potassium 5.3, Bicarbonate 16, Sodium 128. Urinalysis showed large blood and 1+ protein. The serological workup was positive for P ANCA 1:640, C ANCA 1:640, MPO ab and negative for PR-3 ANA, SPEP, Free light chain ratio, HIV, Hepatitis panel. Endocscopy and Colonoscopy was negative for any obvious bleeding. Patient underwent renal biopsy which showed 7 out of 15 glomeruli with early fibrocellular crescents with fibrinoid necrosis, along with healing phase of necrotiizing arteritis. Immunoflourescence didn't show any preferrential staning for immunoglobulin, kappa or labmda. Electron microscopy showed messangial mild non branching randomly arrayed thick fibrils with no immune complex type deposits. EM findings were confirmed by positive DNAJB9 stain. Patient received treatment with pulse dose steriods, two doses of rituximab 1 gm 14 days apart and continued on prednisone for slow taper with good renal response. Due to the findings of Fibrillary GN addition workup for lymphoproliferative disorder was done Whole body CT scan was negative but flow cytometry testing still pending.


We present a unique case of fibrillary GN and pauci-immune crescentic GN with positive MPO antibodies. The significance of fibirillary deposits in this setting is unclear and usually not seen with pauci-immune crescentic GN. Fibrillary GN is a very rare diagnosis mostly thought to be idiopathic in nature. This unique presentation of fibrillary GN with ANCA vasculitis questions the current pathogensis fibrillary GN and overall renal prognosis in association with glomerular pathologies like ANCA vasclitis