Emergence of Smoldering ANCA-associated Glomerulonephritis during the Clinical Course of Mixed Connective Tissue Disease and Sjögren's Syndrome

Chikayuki Morimoto; Yoshihide Fujigaki; Yoshifuru Tamura; Tatsuru Ota; Shigeru Shibata; Kurumi Asako; Hirotoshi Kikuchi; Hajime Kono; Fukuo Kondo; Yutaka Yamaguchi; Shunya Uchida

doi:10.2169/internalmedicine.9844-17

CASE REPORTS

Emergence of Smoldering ANCA-associated Glomerulonephritis during the Clinical Course of Mixed Connective Tissue Disease and Sjögren's Syndrome

Chikayuki Morimoto, Yoshihide Fujigaki, Yoshifuru Tamura, Tatsuru Ota, Shigeru Shibata, Kurumi Asako, Hirotoshi Kikuchi, Hajime Kono, Fukuo Kondo, Yutaka Yamaguchi, Shunya Uchida

Author information

Chikayuki Morimoto
Department of Internal Medicine, Teikyo University School of Medicine, Japan
Yoshihide Fujigaki
Department of Internal Medicine, Teikyo University School of Medicine, Japan
Central Laboratory, Teikyo University School of Medicine, Japan
Yoshifuru Tamura
Department of Internal Medicine, Teikyo University School of Medicine, Japan
Tatsuru Ota
Department of Internal Medicine, Teikyo University School of Medicine, Japan
Shigeru Shibata
Department of Internal Medicine, Teikyo University School of Medicine, Japan
Kurumi Asako
Department of Internal Medicine, Teikyo University School of Medicine, Japan
Hirotoshi Kikuchi
Department of Internal Medicine, Teikyo University School of Medicine, Japan
Hajime Kono
Department of Internal Medicine, Teikyo University School of Medicine, Japan
Fukuo Kondo
Department of Pathology, Teikyo University School of Medicine, Japan
Yutaka Yamaguchi
Department of Pathology, Teikyo University School of Medicine, Japan
Shunya Uchida
Department of Internal Medicine, Teikyo University School of Medicine, Japan

Keywords: ANCA-associated glomerulonephritis, mixed connective tissue disease, Sjögren's syndrome

JOURNAL OPEN ACCESS

2018 Volume 57 Issue 12 Pages 1757-1762

DOI https://doi.org/10.2169/internalmedicine.9844-17

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Abstract

A 67-year-old woman presented with hematuria and proteinuria 16 and 11 months ago, respectively. She had been followed up as mixed connective tissue disease and Sjögren's syndrome for over 19 years. Blood chemistry showed no elevated serum creatinine or C-reactive protein but did reveal myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) of 300 U/dL. A kidney biopsy showed pauci-immune focal necrotizing glomerulonephritis. She was treated with prednisolone and rituximab, resulting in normal urinalysis and decreased MPO-ANCA. The complication of ANCA-associated glomerulonephritis should not be overlooked when abnormal urinalysis findings appear in the course of connective tissue disease, irrespective of the presence of rapidly progressive glomerulonephritis.

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