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Abstract: PO1752

A Case of Granulomatosis with Polyangiitis Complicated by Renal Mass-Like Lesion

Session Information

Category: Trainee Case Report

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Kaikoi, Daichi, Department of Nephrology and Laboratory Medicine, Kanazawa University, Kanazawa, Ishikawa, Japan
  • Sato, Koichi, Department of Nephrology and Laboratory Medicine, Kanazawa University, Kanazawa, Ishikawa, Japan
  • Ogura, Hisayuki, Department of Nephrology and Laboratory Medicine, Kanazawa University, Kanazawa, Ishikawa, Japan
  • Miyagawa, Taro, Department of Nephrology and Laboratory Medicine, Kanazawa University, Kanazawa, Ishikawa, Japan
  • Toyama, Tadashi, Department of Nephrology and Laboratory Medicine, Kanazawa University, Kanazawa, Ishikawa, Japan
  • Kitajima, Shinji, Department of Nephrology and Laboratory Medicine, Kanazawa University, Kanazawa, Ishikawa, Japan
  • Hara, Akinori, Department of Nephrology and Laboratory Medicine, Kanazawa University, Kanazawa, Ishikawa, Japan
  • Iwata, Yasunori, Department of Nephrology and Laboratory Medicine, Kanazawa University, Kanazawa, Ishikawa, Japan
  • Sakai, Norihiko, Department of Nephrology and Laboratory Medicine, Kanazawa University, Kanazawa, Ishikawa, Japan
  • Shimizu, Miho, Department of Nephrology and Laboratory Medicine, Kanazawa University, Kanazawa, Ishikawa, Japan
  • Wada, Takashi, Department of Nephrology and Laboratory Medicine, Kanazawa University, Kanazawa, Ishikawa, Japan
Introduction

Granulomatosis with polyangiitis (GPA) is a multiorgan systemic disease. Some cases of GPA may mimic IgG4-related disease (IgG4-RD) on histologic examination. Here we report a case of GPA complicated by renal mass-like lesion with infiltration of IgG4-positive plasma cells.

Case Description

A 76-year-old woman was diagnosed with otitis media with effusion 6 years before admission, and scleritis 3 years before admission. She developed nasal leaks and nasal bleeding a year before admission, and high fever and general malaise a month before admission. She visited nearby hospital and was detected a mass-like lesion in the right nasal cavity. Contrast-enhanced computed tomography (CT) of the head revealed an enhanced soft-tissue from the right middle meatus to the nasal septum and cervical lymphadenopathy. Serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) was positive (271.7 U/mL) with high C-reactive protein (CRP) level (29.7 mg/dL). Urinalysis findings showed minor proteinuria (0.1 g/gCr), but elevated tubular injury markers such as urinary beta 2-microglobulin. So, she admitted to our department. Contrast-enhanced CT of the abdomen revealed a 47-mm large mass-like lesion in the right kidney, and CT-guided renal biopsy was performed. Cellular to fibro-cellular crescent and fibrinoid necrosis were observed in the glomerulus. In the interstitium, granulomas with multinucleated giant cells and infiltration of IgG4-positive (IgG4+) plasma cells were observed. In addition, cell infiltration into the arteriolar wall and the rupture of lamina elastica were observed. From these findings with small vessel vasculitis, we diagnosed her as GPA with infiltration of IgG4+ plasma cells. After two courses of methylprednisolone pulse therapy, we added two courses of cyclophosphamide pulse therapy. With improved symptoms and serum data (PR3-ANCA level reduced from 266.8 to 39.0 U/mL), mass-like lesions in nasal turbinate and right kidney diminished.

Discussion

We experienced a case of GPA complicated by renal mass-like lesion. Renal biopsy revealed a coexistence of microvasculitis and infiltration of IgG4+ plasma cells. Further investigation will be required to clarify the role of IgG4+ cells in the pathogenesis of GPA.