Abstract: PUB202
ANCA Vasculitis: Detecting It Early
Session Information
Category: Trainee Case Report
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Patel, Kirtan, The Christ Hospital Physicians Spine, Cincinnati, Ohio, United States
- Austin, Joe N., The Christ Hospital Physicians Spine, Cincinnati, Ohio, United States
Introduction
Renal limited vasculitis (RLV) is part of the spectrum of antineutrophil cytoplasmic autoantibody (ANCA) vasculitis and includes granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). GPA and MPA present with kidney and pulmonary involvement. RLV tend to present later in the disease course given the lack of extrarenal symptoms that are seen with GPA and MPA. They can progress into having GPA and MPA with development of extrarenal manifestations. Here we present a case in which renal limited vasculitis is diagnosed early in its course.
Case Description
54 year Hispanic male with no medical history was admitted with atrial fibrillation, acute heart failure, hyperthyroidism and melena. Patient had no history of recurrent sinus or respiratory infections. On exam, patient had a saddler nose deformity from an accident as a child and lower extremity edema. Creatinine (Cr) was 0.54 on admit and was also 0.54 in the emergency room one week prior. Urinalysis had large blood with >100 RBC/HPF and 30mg/dL protein. Patient was diuresed aggressively for 3 days with a furosemide drip and with a mild bump in creatinine from 0.73 to 1.16.
The patient’s renal function deteriorated even after losartan and furosemide were discontinued and nephrology was consulted when Cr was 2.23. Evaluation showed ongoing microscopic hematuria. Serologic studies were ordered and renal biopsy was performed. Biopsy showed pauci-immune necrotizing glomerulonephritis (GN) with crescents. C3 and C4 were low and C-ANCA and anti-proteinase 3 were strongly positive. Despite induction therapy with Solumedrol followed by weekly rituximab x 4 doses, renal function continued to decline. Due to aggressive clinical course, he was started on plasma exchange x 7 treatments. Despite interventions, he became oligo-anuric and required initiation of dialysis. Although he has had some recovery of renal function and is no longer oligo-anuric, he remains dialysis-dependent.
Discussion
Renal limited vasculitis is confirmed by kidney biopsy, and commonly shows necrotizing GN with crescents in the early stages. As the disease progresses, the lesions become more sclerotic. Untreated glomerulonephritis can progress to end stage renal disease within weeks. Treatment requires pulse methylprednisolone with 500 to 1000mg/day for three days followed by oral prednisone along with cyclophosphamide or rituximab. In rapidly decline in Cr continues, plasmapheresis is warranted.