Abstract: SA-PO250
Eosinophilic Granulomatosis with Polyangiitis - Renal Biopsy Can Aid in Guiding Therapy
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Kamboj, Mayanka, University of florida, Gainesville, Florida, United States
- Chamarthi, Gajapathiraju, University of florida, Gainesville, Florida, United States
- Ruchi, Rupam, University of florida, Gainesville, Florida, United States
Introduction
Eosinophilic granulomatosis with polyangiitis (EGPA), a rare systemic vasculitis; can cause end organ damage with eosinophilic infiltration of the extravascular tissue and necrotizing vasculitis. Patients with renal involvement usually develop rapidly progressive glomerulonephritis (GN).
Case Description
61-year-old female with 1.5-year history of asthma, recurrent sinusitis with nasal polyposis presented with fever. Lab work showed leukocytosis (WBC 23,400/mm3 with 29% eosinophils), serum creatinine (SCr) 0.7 mg/dL, microscopic hematuria, proteinuria and positive antineutrophil cytoplasmic antibody (ANCA) 1:320 with myeloperoxidase specificity. Therapy with glucocorticoids was initiated. She developed acute kidney injury, initially atrribited to volume depletion; when SCr worsened to 1.9 mg/dL and improved to 1.3 mg/dL with IV fluids. Renal biopsy showed segmental necrotizing pauci-immune GN with crescent formation. Treatment with cyclophosphamide (CYC) (10mg/kg) was initiated. On 3 week follow up, SCr worsened to 2.7 mg/dL when CYC was escalated to 15mg/kg/dose for the next 3 doses. Her SCr improved and stabilized at 2 mg/dL over the next 2 months.
Discussion
Renal function as measured by SCr can be relatively preserved in crescentic ANCA vasculitis. Biopsy showing renal involvement affected the decision to initiate therapy with CYC in this patient. The rapidity of decline in renal function despite treatment foretells that the patient could have had a worse renal outcome with delay in appropriate immunosuppressive (IS) therapy. Our case emphasizes the importance of doing a renal biopsy in patients with systemic features of EGPA and active urine sediment even with normal relatively preserved SCr, not only for definitive diagnosis but also guide clinicians to pursue aggressive early IS therapy and improve renal prognosis.
Glomerulus showing a cellular crescent ( methanamine silver staining)