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Abstract: TH-PO671

Unusual Presentation of ANCA-Associated Vasculitis with Full-House Pattern Immunostaining

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Monk, Brian, The University of Mississippi Medical Center, Jackson, Mississippi, United States
  • Al-Sawajneh, Suhaib F M, The University of Mississippi Medical Center, Jackson, Mississippi, United States
  • Yen, Timothy E., The University of Mississippi Medical Center, Jackson, Mississippi, United States
  • Hassanein, Mohamed, The University of Mississippi Medical Center, Jackson, Mississippi, United States
  • Atari, Mohammad, The University of Mississippi Medical Center, Jackson, Mississippi, United States
Introduction

Pauci-immune glomerulonephritis is the most common cause of rapidly progressive glomerulonephritis (RPGN) mainly secondary to antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Typically, renal involvement lacks the presence of immune deposits. Here we present a case of AAV-induced RPGN with a full-house pattern immunofluorescence (IF).

Case Description

A 35-year-old male with a history of uncorrected congenital heart disease presented to the hospital with lower extremitiy edema. Evaluation revealed AKI with serum creatinine 5.43 mg/dL, urine analysis with >100 RBCs (0-4), +50 protein with sub-nephrotic range proteinuria. Autoimmune workup was positive for ANA 1:160 (speckled pattern), low C3 70mg/dL (90-180 mg/dL), positive PR3-ANCA >8U (<0.4U), and P-ANCA, but negative MPO-ANCA and C-ANCA. Anti-dsDNA, anti-histone antibodies, anti-smith, anti-GBM, viral panel, and protein electrophoresis all were normal. Kidney biopsy showed necrotizing crescentic glomerulonephritis with variable para-medullary inflammation strongly suggesting ANCA vasculitis. IF showed a full-house pattern (IgG, IgM, IgA, C3, C1q, kappa, and lambda). Treatment with Rituximab and pulse steroids followed by steroids taper was initiated. Unfortunately, the patient passed away.

Discussion

Renal involvement by AAV typically has no or few immune deposits. Full-house immunofluorescence has been rarely reported in the setting of ANCA vasculitis and it is unusual. Although histopathology in our patient is suggestive of AAV, the presence of full house pattern on IF with positive serum ANA is concerning for possible overlap syndrome where lupus and ANCA vasculitis accompany. Despite the absence of anti-dsDNA and anti-smith, still, lupus cannot be ruled out. This case is one of a few reported cases having AAV with RPGN presentation and concomitant full-house pattern in kidney biopsy, possibly representing lupus/AAV overlap.