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Long-term observations of clinicopathological characteristics and outcome of Japanese patients with pauci-immune crescentic glomerulonephritis

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Abstract

Background

The clinicopathological characteristics and outcome with pauci-immune crescentic glomerulonephritis (CreGN) are presumed to vary over time. We examined the characteristics and outcome of Japanese patients with CreGN according to the treatment periods.

Patients and methods

From 1968 to 2011, we examined a total of 102 patients diagnosed with pauci-immune CreGN by renal biopsy. The patients were divided into three groups according to the treatment periods—Group I (1968–1988, n = 18), Group II (1989–2001, n = 37; when the nationwide survey of rapidly progressive glomerulonephritis [RPGN] was performed in Japan), and Group III (2002–2011, n = 47; after publication of the Japanese guideline for RPGN).

Results

There were no significant differences in blood pressure, renal function or anti-neutrophil cytoplasmic antibody titer between groups. On the other hand, the rate of crescent formation and degree of interstitial inflammatory cell infiltration were decreased in Group III. Serum creatinine (<3.0, 3.0–6.0, ≥6.0 mg/dL) and crescent formation (<30, 30–50, 50–80, ≥80 %) were significant renal prognostic factors in Group III [serum creatinine: hazard ratio (HR) 4.79, 95 % confidence interval (CI) 1.43–16.1, P = 0.011; crescent formation: HR 2.86, 95 % CI 1.06–7.73, P = 0.039]. Furthermore, renal survival rate of patients with crescent formation <50 % and patient survival rate of patients with serum creatinine <3 mg/dL were improved in Group III.

Conclusion

Patients with CreGN were diagnosed in the early phase of crescent formation and outcome has improved in recent years.

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Acknowledgments

We thank the physicians and staff members at the collaborative institutions for participating in this study and for providing data. This work was financially supported in part by Grants-in-Aid for Progressive Renal Diseases Research, Research on Intractable Disease, and for Intractable Vasculitis from the Ministry of Health, Labour and Welfare of the Japanese Government.

Conflict of interest

The authors declare that no conflicts of interest exist.

Author information

Authors and Affiliations

  1. Division of Blood Purification, Kanazawa University Hospital, Kanazawa, Japan

    Kiyoki Kitagawa & Kengo Furuichi

  2. Division of Nephrology, Kanazawa University Hospital, 13-1 Takara-machi, Kanazawa, 920-8641, Japan

    Kiyoki Kitagawa, Kengo Furuichi, Tadashi Toyama, Shinji Kitajima, Yasunori Iwata & Takashi Wada

  3. Department of Disease Control and Homeostasis, Kanazawa University, Kanazawa, Japan

    Kiyoki Kitagawa, Kengo Furuichi, Yasuyuki Shinozaki, Tadashi Toyama, Shinji Kitajima, Akinori Hara, Norihiko Sakai & Shuichi Kaneko

  4. Department of Laboratory Medicine, Kanazawa University, Kanazawa, Japan

    Yasunori Iwata & Takashi Wada

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  1. Kiyoki Kitagawa
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  2. Kengo Furuichi
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  3. Yasuyuki Shinozaki
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  10. Takashi Wada
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Kanazawa Study Group for Renal Diseases and Hypertension

Corresponding author

Correspondence to Takashi Wada.

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Kitagawa, K., Furuichi, K., Shinozaki, Y. et al. Long-term observations of clinicopathological characteristics and outcome of Japanese patients with pauci-immune crescentic glomerulonephritis. Clin Exp Nephrol 17, 858–865 (2013). https://doi.org/10.1007/s10157-013-0798-4

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  • DOI: https://doi.org/10.1007/s10157-013-0798-4

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