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Kidney Week

Abstract: TH-PO576

Looking at ANCA Vasculitis

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Authors

  • Aslam, Saher, University of Nebraska Medical Center, Omaha, Nebraska, United States
  • Chaudhry, Haris H., Creighton University School of Medicine, Omaha, Nebraska, United States
  • Magliulo, Eric, University of Nebraska Medical Center, Omaha, Nebraska, United States
  • Foster, Kirk W., University of Nebraska Medical Center, Omaha, Nebraska, United States
  • Naranjo, Felipe Sebastian, University of Nebraska Medical Center, Omaha, Nebraska, United States
Introduction

Anti-neutrophilic cytoplasmic antibody (ANCA) associated vasculitis is a group of autoimmune diseases that can cause inflammation and damage to small blood vessels throughout the body. Inflammatory eye disease is described in 50% to 60% of patients with ANCA-positive vasculitis, and for 8% to 16% of patients, it is an initial manifestation.

Case Description

A 74-year-old man with a history of gout, chronic sinusitis, and chronic kidney disease (G3a) presented to the clinic with worsening kidney function and proteinuria without hematuria. He was seen by Ophthalmology a few months prior for scleritis and was treated with a short course of NSAIDs, with the improvement of his symptoms. The initial workup was unremarkable. Kidney biopsy showed a lesion of focal segmental sclerosis with obliteration of the capillary lumen, mild interstitial fibrosis, and tubular atrophy, immunofluorescence was negative and electron microscopy with rare small dense deposits. The patient was started on SGLT-2 inhibitors. Renal function continued to deteriorate. The patient's condition was complicated by acute bronchitis, diffuse anterior scleritis, and acute kidney injury, laboratory work was significant for positive ANCA antibodies with elevated PR3 antibodies. A repeat kidney biopsy revealed necrotizing crescentic glomerulonephritis. He was treated with IV steroids, rituximab and ultimately required dialysis.

Discussion

Renal involvement is a significant feature of ANCA vasculitis and can lead to acute kidney injury or chronic renal failure if left untreated. Clinicians should consider ANCA vasculitis in the differential diagnosis of patients presenting with scleritis, especially in those with renal dysfunction even with partial or complete resolution in ocular symptoms. Early diagnosis and treatment are essential in preventing irreversible organ damage and improving patient outcomes.

Biopsy with PAS stain showing crescent formation consistent with ANCA vasculitis