Abstract: FR-PO136
Granulomatosis with Polyangiitis (GPA) Presenting with Granulomatous Nephritis but No Glomerulonephritis
Session Information
- AKI: Diagnosis and Outcomes
October 25, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Abdali, Mohamed, Detroit Medical Center, Detroit, Michigan, United States
- Koneru, Praveena, John D Dingell VA Medical Center, Detroit, Michigan, United States
- Shah, Mili Jay, John D Dingell VA Medical Center, Detroit, Michigan, United States
- Mohanty, Madhumita J., John D Dingell VA Medical Center, Detroit, Michigan, United States
Introduction
We report a patient with worsening renal function and positive ANCA antibody with atypical clinical and histological findings
Case Description
A 83-year-old male with a history of hypertension and working diagnosis of sarcoidosis with recurrent uveitis, skin lesions with biopsy consistent with possible sarcoidosis, and pulmonary nodules, was being treated with mycophenolate mofetil ( He was previously on methotrexate). He had an increase in plasma creatinine from 2.2 mg/dl in December 2023 to 4.5 mg/dl in March 2024. His blood pressure was normal, and his examination was unremarkable. He had no proteinuria, hematuria, or urinary casts. Renal ultrasound showed normal sized kidneys with no hydronephrosis. Plasma calcium and complement C3 and C4 were normal. ANA, dsDNA, rheumatoid factor, hepatitis C antibody, hepatitis B surface antigen and QuantiFERON TB Gold Plus were negative. Angiotensin converting enzyme was not high. ANCA immunofluorescence titer was negative but serine protease 3, IgG was elevated at 47 AU/ml (normal: 0-14). He had a kidney biopsy in March 2024 which showed granulomatous nephritis with non-caseating granulomas in the interstitium with possible small artery /arteriole disrupted by granulomatous inflammation. Granulomas were somewhat loose and not clearly defined. There was no definitive glomerulonephritis, glomerular necrosis or crescents. There were occasional neutrophil casts. There was patchy interstitial infiltrate in 40% of cortex with mononuclear and plasma cells in some regions and neutrophil and eosinophils in some regions. There was no evidence of histoplasmosis or AFB in the biopsy. The differential included GPA and sarcoidosis. Loose organization of granulomas, neutrophil casts, interstitial infiltrate with neutrophils and eosinophils and possible destructive arteriolitis all favored GPA over sarcoidosis. The patient was treated with prednisone and rituximab with improvement of plasma creatinine to 2.1 mg/dl.
Discussion
ANCA vasculitis may lead to worsening renal function without glomerular involvement. Renal involvement of ANCA vasculitis should be considered in patients with positive ANCA even in the absence of hematuria, proteinuria or urinary casts and early renal biopsy should be considered in these patients so that treatment may be started before the onset of irreversible renal injury