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Kidney Week

Abstract: FR-PO0767

Methimazole-Induced ANCA-Associated Vasculitis (AAV): Lessons from Two Cases

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Ojaimi, Nadim, University of Maryland Medical Center, Baltimore, Maryland, United States
  • Patel, Ami Mahendrakumar, University of Maryland Medical Center, Baltimore, Maryland, United States
Introduction

Antithyroid drug–induced ANCA-associated vasculitis is a rare complication that may mimic infection or primary AAV. We report two methimazole-associated cases.

Case Description

Case 1: A 50-year-old woman recently off methimazole developed cough, pulmonary nodules, active urine sediment, and AKI. PR3-ANCA was positive; infectious work-up including bronchospcopy, MTB DNA, viral respiratory panel, and HIV were negative. Biopsy demonstrated necrotizing crescentic GN involving 50% of glomeruli.
Case 2: A 50-year-old man on methimazole developed DAH, respiratory failure, and dialysis-requiring AKI. p-ANCA/MPO were positive; other serologies were negative. Biopsy showed pauci-immune GN with arteritis. He improved after withdrawal and immunosuppression, stopping dialysis.

Discussion

Methimazole-induced AAV can range from renal-limited disease to severe pulmonary-renal syndrome with DAH and dialysis-requiring AKI. Although MPO-ANCA is typical, PR3 positivity may occur. Biopsy, methimazole withdrawal, and prompt immunosuppression are key.

Acknowledgment

Dr Autumn Larocque-Pathology.

Serologies and features
Case #Case 1Case 2
ANCA ProfilePR3-ANCA positive,PR3 4.3; MPO negativep-ANCA 1:320; MPO positive165; PR3 negative
Renal InvolvementActive urinary sediment; Cr peak 2.24 mg/dLAKI requiring CRRT/iHD; Cr peak 11 mg/dL
Urine studiesActive sediment: >20 RBCs/hpf, 11–20 WBCs/hpf; UPCR 0.7–1 g/g; UACR 376 mg/g.UA w 6-10 rbcs/hpf; UPCR 0.7 g/g
Pulmonary involvementBilateral pulmonary nodules and chronic upper respiratory symptoms concerning for pulmonary-renal involvementDiffuse alveolar hemorrhage with hypoxemic respiratory failure
Biopsy findingsNecrotizing and crescentic GN involving 50% of glomeruli; mild IFTA ~20%Severe pauci-immune ANCA vasculitis with fibrinoid necrosis, crescents, necrotizing arteritis, and mild to focally moderate IFTA

Figure 1. Case 1 urine microscopy and kidney biopsy. Urine microscopy shows an RBC cast, upper left. H&E section shows an RBC cast, lower left, 60×. Jones stain shows a segmental necrotizing lesion, right, 40×.

Figure 2. Case 2 kidney biopsy. Left: vessel with fibrinoid necrosis, arrowhead, H&E, 20×. Right: glomerular crescent, arrowhead, with fibrinoid necrosis, arrow, PAMS, 20×.