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Validation of a renal risk score in a cohort of ANCA-associated vasculitis patients with severe kidney damage

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A Correction to this article was published on 13 March 2020

This article has been updated

Abstract

Objective

To validate the renal risk score in a cohort of patients with advanced kidney damage.

Methods

A total of 72 patients with biopsy-proven ANCA glomerulonephritis with >12 months of follow-up were studied. The renal risk score was calculated and evaluated by survival analysis for time of renal survival. Cohort-specific clinical, histopathologic, and post-treatment factors associated with renal survival were determined by Cox regression analysis.

Results

Kidney biopsies were classified as focal, crescentic, mixed, and sclerotic classes in 6 (8%), 4 (6%), 25 (35%), and 37 (51%) patients, respectively. The 1-, 3-, and 5-year renal survival rates were 79%, 73%, and 68%, respectively. Patients were segregated by the risk score in low- (18%), medium- (47%), and high-risk (35%) groups. Patients in the low-risk group had 36-, 60-, and 84-month renal survival of 100%; those in the medium risk 85% (95% CI 72–92), 81% (95% CI 66–95), and 76% (95% CI 60–92), respectively; and those in the high risk 37% (95% CI 17–57), 26% (95% CI 7–45), and 18% (95% CI 1–36), respectively. Six (43%) of the 14 patients in the high-risk group recovered renal function after the initial episode, and 2 (14%) remained dialysis-free. Other parameters associated with renal survival included age, proteinuria, general symptoms, cellular crescents, glomerulosclerosis, tubulointerstitial lesions, best post-treatment eGFR, and renal relapses.

Conclusions

We validated the renal risk score as a prognostic tool in a cohort with predominantly mixed and sclerotic histologic categories. Since patients in the high-risk group still benefited from immunosuppressive therapy, this score should be used in conjunction with other predictive parameters to aid therapeutic decisions.

Key Points

• The ANCA renal risk score is validated in a cohort with advanced kidney damage.

• Patients in the high-risk group still benefited from immunosuppressive therapy.

• Parameters not included in the risk score are associated with renal survival and may be useful.

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Change history

  • 13 March 2020

    The footnote of Figure 2 in the published original version of the above article went missing and the correct figure is presented in this article.

Abbreviations

AAV :

ANCA-associated vasculitides

ANCA :

Anti-neutrophil cytoplasmic antibody

BVAS/GPA :

Birmingham Vasculitis Activity Score for Granulomatosis with Polyangiitis

eGFR :

Estimated glomerular filtration rate

ESR :

Erythrocyte sedimentation rate

FFS :

Five-Factor Score

GPA :

Granulomatosis with polyangiitis

hsCRP :

High-sensitive C-reactive protein

IF/TA :

Interstitial fibrosis/tubular atrophy

I.V :

Intravenous

MPA :

Microscopic polyangiitis

MPO-ANCA :

Myeloperoxidase ANCA

PR3-ANCA :

Proteinase 3 ANCA

RLV :

Renal-limited vasculitis

RRT :

Renal replacement therapy

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Funding

The authors declare that no specific funding was received to carry out the work described in this manuscript.

Author information

Authors and Affiliations

  1. Department of Nephrology and Mineral Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico, Mexico

    Juan M. Mejía-Vilet

  2. Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Sección XVI, Tlalpan, 14000, Mexico, CP, Mexico

    Eduardo Martín-Nares, Manuel A. Sedano-Montoya & Andrea Hinojosa-Azaola

  3. Facultad de Medicina, Universidad Autónoma de Baja California campus Mexicali, Mexicali, Mexico

    Mayra L. Cano-Verduzco & Abril A. Pérez-Arias

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  1. Juan M. Mejía-Vilet
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Contributions

JMMV, EMN, and AHA designed the study; JMMV, EMN, MLCV, AAPA, MASM, and AHA participated in data acquisition; JMMV, EMN, AAPA, and AHA analyzed and interpreted data; JMMV, EMN, MLCV, AAPA, MASM, and AHA drafted and revised the manuscript.

Corresponding author

Correspondence to Andrea Hinojosa-Azaola.

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Research was conducted in compliance with the Helsinki Declaration. Approval by the local ethical committee was obtained.

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The original version of this article was revised: The publisher regret in the Introduction section of the above published article contained an error. The sentence original reading "...(proportion of normal IF, and estimated glomerular filtration/IF,.." should read as "... (proportion of normal glomeruli, percentage of TA/IF,...". [bold text used to highlight problem area]. The article has been corrected.

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Mejía-Vilet, J.M., Martín-Nares, E., Cano-Verduzco, M.L. et al. Validation of a renal risk score in a cohort of ANCA-associated vasculitis patients with severe kidney damage. Clin Rheumatol 39, 1935–1943 (2020). https://doi.org/10.1007/s10067-020-04936-5

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