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Renal-limited vasculitis with elevated levels of multiple antibodies

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Abstract

Renal-limited vasculitis (RLV) is a type of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis that presents with crescentic glomerulonephritis with no other organ involvement. Although several studies reported patients with crescentic glomerulonephritis who were dual positive for proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA or ANCA and anti-glomerular basement membrane (GBM) antibody, patients positive for all three antibodies, i.e., triple-positive patients, were rarely reported. We herein report the case of a male with pauci-immune type crescentic glomerulonephritis positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibody. Renal biopsy led to the definitive diagnosis of RLV with pauci-immune-type crescentic glomerulonephritis. Fluorescence immunostaining showed no linear deposition of IgG on GBM, indicating no involvement of anti-GBM associated diseases. Intensive therapy, including prednisolone, plasma exchange, and intravenous cyclophosphamide, was effective. We report the case of triple-positive patient with crescentic glomerulonephritis, who was successfully treated with glucocorticoid, plasma exchange, and cyclophosphamide, suggesting that treatment for RLV in the patient with serological triple antibodies positivity in the absence of linear IgG deposition could benefit from the combination therapy regimen for plasma exchange and primary induction of remission against microscopic polyangiitis.

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Abbreviations

ANCA:

Anti-neutrophil cytoplasmic antibody

PR3-ANCA:

Proteinase 3 anti-neutrophil cytoplasmic antibody

MPO-ANCA:

Myeloperoxidase anti-neutrophil cytoplasmic antibody

GBM:

Glomerular basement membrane

RLV:

Renal-limited vasculitis

MPA:

Microscopic polyangiitis

RPGN:

Rapidly progressive glomerulonephritis

APTT:

Activated partial thromboplastin time

IVCY:

Intravenous high-dose cyclophosphamide therapy

PSC:

Primary sclerotic cholangitis

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Acknowledgements

We gratefully acknowledge Y. Mizukami, A. Morita, and M. Ozone in the Department of Nephrology, Graduate School of Medicine, Kyoto University for secretarial assistance.

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Authors and Affiliations

  1. Department of Nephrology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo, Kyoto, 606-8507, Japan

    Noriaki Sato, Hideki Yokoi, Hirotaka Imamaki, Eiichiro Uchino, Kaoru Sakai, Takeshi Matsubara, Tatsuo Tsukamoto & Motoko Yanagita

  2. Department of Nephrology, Hirakata Kohsai Hospital, Federation of National Public Service Personnel Mutual Aid Associations, Hirakata, Japan

    Hirotaka Imamaki

  3. Department of Nephrology and Dialysis, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan

    Tatsuo Tsukamoto

  4. Department of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Japan

    Sachiko Minamiguchi

Authors
  1. Noriaki Sato
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  2. Hideki Yokoi
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Corresponding author

Correspondence to Hideki Yokoi.

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The authors declare that they have no competing interests.

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All relevant ethics for the case report and its publication were obtained.

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Sato, N., Yokoi, H., Imamaki, H. et al. Renal-limited vasculitis with elevated levels of multiple antibodies. CEN Case Rep 6, 79–84 (2017). https://doi.org/10.1007/s13730-017-0248-3

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  • DOI: https://doi.org/10.1007/s13730-017-0248-3

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