Skip to main content

Main menu

  • Home
  • Content
    • Published Ahead of Print
    • Current Issue
    • Podcasts
    • Subject Collections
    • Archives
    • Kidney Week Abstracts
    • Saved Searches
  • Authors
    • Submit a Manuscript
    • Author Resources
  • Trainees
    • Peer Review Program
    • Prize Competition
  • About CJASN
    • About CJASN
    • Editorial Team
    • CJASN Impact
    • CJASN Recognitions
  • More
    • Alerts
    • Advertising
    • Feedback
    • Reprint Information
    • Subscriptions
  • ASN Kidney News
  • Other
    • ASN Publications
    • JASN
    • Kidney360
    • Kidney News Online
    • American Society of Nephrology

User menu

  • Subscribe
  • My alerts
  • Log in
  • My Cart

Search

  • Advanced search
American Society of Nephrology
  • Other
    • ASN Publications
    • JASN
    • Kidney360
    • Kidney News Online
    • American Society of Nephrology
  • Subscribe
  • My alerts
  • Log in
  • My Cart
Advertisement
American Society of Nephrology

Advanced Search

  • Home
  • Content
    • Published Ahead of Print
    • Current Issue
    • Podcasts
    • Subject Collections
    • Archives
    • Kidney Week Abstracts
    • Saved Searches
  • Authors
    • Submit a Manuscript
    • Author Resources
  • Trainees
    • Peer Review Program
    • Prize Competition
  • About CJASN
    • About CJASN
    • Editorial Team
    • CJASN Impact
    • CJASN Recognitions
  • More
    • Alerts
    • Advertising
    • Feedback
    • Reprint Information
    • Subscriptions
  • ASN Kidney News
  • Visit ASN on Facebook
  • Follow CJASN on Twitter
  • CJASN RSS
  • Community Forum
Original ArticlesGlomerular and Tubulointerstitial Diseases
You have accessRestricted Access

Prognostic Factors and Long-Term Outcome with ANCA-Associated Kidney Vasculitis in Childhood

Marta Calatroni, Filippo Consonni, Marco Allinovi, Alessandra Bettiol, Natasha Jawa, Susanna Fiasella, Dritan Curi, Sarah Abu Rumeileh, Leonardo Tomei, Laura Fortunato, Elena Gelain, Davide Gianfreda, Elena Oliva, Guido Jeannin, Chiara Salviani, Giacomo Emmi, Monica Bodria, Renato A. Sinico, Gabriella Moroni, Giuseppe A. Ramirez, Enrica Bozzolo, Enrico Tombetti, Sara Monti, Claudia Bracaglia, Giulia Marucci, Serena Pastore, Pasquale Esposito, Maria G. Catanoso, Barbara Crapella, Giovanni Montini, Rosa Roperto, Marco Materassi, Giovanni M. Rossi, Salvatore Badalamenti, Rae S.M. Yeung, Paola Romagnani, Gian M. Ghiggeri, Damien Noone and Augusto Vaglio
CJASN July 2021, 16 (7) 1043-1051; DOI: https://doi.org/10.2215/CJN.19181220
Marta Calatroni
1Nephrology Unit, Humanitas Clinical and Research Center, Rozzano, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Filippo Consonni
2Department of Health Sciences, University of Florence, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Filippo Consonni
Marco Allinovi
3Nephrology Unit, Careggi Hospital, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Marco Allinovi
Alessandra Bettiol
4Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Natasha Jawa
5Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Susanna Fiasella
6Nephrology and Dialysis Unit, Meyer Children’s Hospital, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Dritan Curi
6Nephrology and Dialysis Unit, Meyer Children’s Hospital, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Sarah Abu Rumeileh
2Department of Health Sciences, University of Florence, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Leonardo Tomei
2Department of Health Sciences, University of Florence, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Laura Fortunato
6Nephrology and Dialysis Unit, Meyer Children’s Hospital, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Elena Gelain
6Nephrology and Dialysis Unit, Meyer Children’s Hospital, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Davide Gianfreda
7Nephrology Unit, Fondazione Panico Hospital, Tricase, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Elena Oliva
8Nephrology Unit, Riuniti Hospital Marche, Ancona, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Guido Jeannin
9Nephrology Unit, Spedali Civili Hospital, Brescia, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Chiara Salviani
9Nephrology Unit, Spedali Civili Hospital, Brescia, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Giacomo Emmi
4Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Monica Bodria
10Laboratory of Molecular Nephrology and Division of Nephrology and Transplantation, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Giannina Gaslini Institute, Genoa, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Renato A. Sinico
10Laboratory of Molecular Nephrology and Division of Nephrology and Transplantation, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Giannina Gaslini Institute, Genoa, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Gabriella Moroni
11Nephrology Unit, University of Milano-Bicocca, Monza, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Giuseppe A. Ramirez
12Nephrology Unit, IRCCS Ca 'Granda Foundation Maggiore Policlinico Hospital, Milan, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Enrica Bozzolo
12Nephrology Unit, IRCCS Ca 'Granda Foundation Maggiore Policlinico Hospital, Milan, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Enrico Tombetti
12Nephrology Unit, IRCCS Ca 'Granda Foundation Maggiore Policlinico Hospital, Milan, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Sara Monti
13Unit of Immunology, Allergy, Rheumatology and Rare Disease, San Raffaele Hospital, Milan, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Claudia Bracaglia
14Department of Rheumatology, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Pavia, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Giulia Marucci
14Department of Rheumatology, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Pavia, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Serena Pastore
15Division of Rheumatology, IRCCS Bambino Gesù Children’s Hospital, Rome, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Pasquale Esposito
16Institute for Maternal and Child Health, IRCCS Burlo Garofolo Hospital, Trieste, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Maria G. Catanoso
17Unit of Nephrology, Dialysis and Transplantation, Department of Internal Medicine, University of Genoa and Ospedale Policlinico San Martino–IRCCS, Genoa, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Barbara Crapella
18Rheumatology Unit, IRCCS Reggio Emilia Hospital, Reggio Emilia, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Giovanni Montini
18Rheumatology Unit, IRCCS Reggio Emilia Hospital, Reggio Emilia, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Giovanni Montini
Rosa Roperto
19Pediatric Nephrology, Dialysis and Transplant Unit, IRCCS Ca 'Granda Foundation Maggiore Policlinico Hospital, Milan, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Marco Materassi
19Pediatric Nephrology, Dialysis and Transplant Unit, IRCCS Ca 'Granda Foundation Maggiore Policlinico Hospital, Milan, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Giovanni M. Rossi
20Department of Biomedical, Experimental and Clinical Sciences “Mario Serio,” University of Florence, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Salvatore Badalamenti
1Nephrology Unit, Humanitas Clinical and Research Center, Rozzano, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Rae S.M. Yeung
21Nephrology Unit, Parma University Hospital, Parma, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Paola Romagnani
6Nephrology and Dialysis Unit, Meyer Children’s Hospital, Florence, Italy
20Department of Biomedical, Experimental and Clinical Sciences “Mario Serio,” University of Florence, Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Gian M. Ghiggeri
10Laboratory of Molecular Nephrology and Division of Nephrology and Transplantation, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Giannina Gaslini Institute, Genoa, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Gian M. Ghiggeri
Damien Noone
5Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada
22Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Augusto Vaglio
6Nephrology and Dialysis Unit, Meyer Children’s Hospital, Florence, Italy
23Pediatric Translational Research Chair, University of Toronto, Toronto, Ontario, Canada
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data Supps
  • Info & Metrics
  • View PDF
Loading

This article has a correction. Please see:

  • Correction: Prognostic Factors and Long-Term Outcome with ANCA-Associated Kidney Vasculitis in Childhood - December 01, 2021

This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.

Visual Abstract

Figure
  • Download figure
  • Open in new tab
  • Download powerpoint

Abstract

Background and objectives ANCA-associated vasculitis is extremely rare in children. We report the clinicopathologic features, long-term outcomes, and prognostic factors of a large pediatric cohort of patients with ANCA-associated kidney vasculitis.

Design, setting, participants, & measurements This retrospective study included 85 consecutive patients with kidney biopsy specimen–proven ANCA-associated vasculitis from tertiary referral centers in Italy and Canada. Kidney biopsy specimens were categorized as focal, crescentic, sclerotic, or mixed, according to the Berden classification. The prognostic significance of baseline clinical, laboratory, and histologic findings was analyzed with respect to kidney failure or CKD stage 3–5/kidney failure.

Results A total of 53 patients had microscopic polyangiitis (62%), and 32 had granulomatosis with polyangiitis (38%). Rapidly progressive GN was the most frequent presentation (39%); a third of the patients also had nephrotic-range proteinuria. Kidney biopsy specimens were classified as focal in 21% of the patients, crescentic in 51%, sclerotic in 15%, and mixed in 13%. Remission-induction therapies included cyclophosphamide in 78% of patients. A total of 25 patients (29%) reached kidney failure. The median (interquartile range) time to kidney failure or last follow-up was 35 (6–89) months in the whole cohort, and 73 (24–109) months among the patients who did not reach this outcome. Patients whose biopsy specimens showed sclerotic histology had significantly shorter kidney survival (hazard ratio, 11.80; 95% confidence interval, 2.49 to 55.99) and survival free of CKD stage 3–5 (hazard ratio, 8.88; 95% confidence interval, 2.43 to 32.48), as compared with those with focal/mixed histology. Baseline eGFR, low serum albumin, hypertension, central nervous system complications, and sclerotic histology, which reflected severe kidney involvement, were associated with both kidney failure and CKD stage 3–5/kidney failure at unadjusted analysis; no independent prognostic factors emerged at multivariable analysis.

Conclusions Children with ANCA-associated kidney vasculitis often have aggressive presentation; a third of such children progress to kidney failure and this usually occurs early during follow-up. A severe clinical presentation is associated with the development of CKD or kidney failure.

  • ANCA
  • vasculitis
  • pediatric nephrology
  • glomerulopathy
  • antineutrophil cytoplasmic antibody
  • Received December 8, 2020.
  • Accepted April 21, 2021.
  • Copyright © 2021 by the American Society of Nephrology
View Full Text

If you are:

  • an ASN member, select the "ASN Member" login button. 
  • an individual subscriber, login with you User Name and Password.
  • an Institutional user, select the Institution option where you will be presented with a list of Shibboleth federations. If you do not see your federation, contact publications@asn-online.org. 

ASN MEMBER LOGIN

ASN MEMBER LOGIN

Log in using your username and password

Forgot your user name or password?

Log in through your institution

You may be able to gain access using your login credentials for your institution. Contact your library if you do not have a username and password.

Purchase access

Pay Per Article - You may access this article (from the computer you are currently using) for 1 day for US$34.00

In order to get access to the article, you must have an account.  If you have an account, enter your user name and password into the boxes above. If you do not have an account, follow the instructions below to create one.  Once you have purchased the article, you will have access to it for 24 hours.  

Steps for Creating an Account:

Click the "Purchase Access" button.  The page will redisplay with the following message at the top of the screen which contains the link to register for an account.

When you create an account, you will be asked for your name, email address and other information.  Just like commercial web sites, we do need details from you in order to complete your purchase of an article.  

You will then be asked to register a user name, email address and you will need to create a password that is at least eight characters in length. You do not need an ASN Member number to complete the form. As you move through the registration page, you will have to verify you are a person by completing a Captcha request.   Lastly, your first and last name will be required. 

Once your information is successfully saved, the system will redisplay the home page of the journal.  From there, navigate back to the article to purchase.  Select the article and at the bottom of the page, use the credentials you just created to login. The article will be added to your shopping cart.  You can continue to navigate across JASN and CJASN adding to your cart from both journals. When you are ready to complete your purchse, select the Shopping Cart from the upper right hand corner of the page and follow the onscreen instructions. 

PreviousNext
Back to top

Podcast

Subscribe to podcast
Download MP3

In this issue

Clinical Journal of the American Society of Nephrology: 16 (7)
Clinical Journal of the American Society of Nephrology
Vol. 16, Issue 7
July 2021
  • Table of Contents
  • Table of Contents (PDF)
  • About the Cover
  • Index by author
View Selected Citations (0)
Print
Download PDF
Sign up for Alerts
Email Article
Thank you for your help in sharing the high-quality science in CJASN.
Enter multiple addresses on separate lines or separate them with commas.
Prognostic Factors and Long-Term Outcome with ANCA-Associated Kidney Vasculitis in Childhood
(Your Name) has sent you a message from American Society of Nephrology
(Your Name) thought you would like to see the American Society of Nephrology web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Citation Tools
Prognostic Factors and Long-Term Outcome with ANCA-Associated Kidney Vasculitis in Childhood
Marta Calatroni, Filippo Consonni, Marco Allinovi, Alessandra Bettiol, Natasha Jawa, Susanna Fiasella, Dritan Curi, Sarah Abu Rumeileh, Leonardo Tomei, Laura Fortunato, Elena Gelain, Davide Gianfreda, Elena Oliva, Guido Jeannin, Chiara Salviani, Giacomo Emmi, Monica Bodria, Renato A. Sinico, Gabriella Moroni, Giuseppe A. Ramirez, Enrica Bozzolo, Enrico Tombetti, Sara Monti, Claudia Bracaglia, Giulia Marucci, Serena Pastore, Pasquale Esposito, Maria G. Catanoso, Barbara Crapella, Giovanni Montini, Rosa Roperto, Marco Materassi, Giovanni M. Rossi, Salvatore Badalamenti, Rae S.M. Yeung, Paola Romagnani, Gian M. Ghiggeri, Damien Noone, Augusto Vaglio
CJASN Jul 2021, 16 (7) 1043-1051; DOI: 10.2215/CJN.19181220

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Request Permissions
Share
Prognostic Factors and Long-Term Outcome with ANCA-Associated Kidney Vasculitis in Childhood
Marta Calatroni, Filippo Consonni, Marco Allinovi, Alessandra Bettiol, Natasha Jawa, Susanna Fiasella, Dritan Curi, Sarah Abu Rumeileh, Leonardo Tomei, Laura Fortunato, Elena Gelain, Davide Gianfreda, Elena Oliva, Guido Jeannin, Chiara Salviani, Giacomo Emmi, Monica Bodria, Renato A. Sinico, Gabriella Moroni, Giuseppe A. Ramirez, Enrica Bozzolo, Enrico Tombetti, Sara Monti, Claudia Bracaglia, Giulia Marucci, Serena Pastore, Pasquale Esposito, Maria G. Catanoso, Barbara Crapella, Giovanni Montini, Rosa Roperto, Marco Materassi, Giovanni M. Rossi, Salvatore Badalamenti, Rae S.M. Yeung, Paola Romagnani, Gian M. Ghiggeri, Damien Noone, Augusto Vaglio
CJASN Jul 2021, 16 (7) 1043-1051; DOI: 10.2215/CJN.19181220
del.icio.us logo Digg logo Reddit logo Twitter logo Facebook logo Google logo Mendeley logo
  • Tweet Widget
  • Facebook Like

Jump to section

  • Article
    • Visual Abstract
    • Abstract
    • Introduction
    • Materials and Methods
    • Results
    • Discussion
    • Disclosures
    • Funding
    • Supplemental Material
    • Footnotes
    • References
  • Figures & Data Supps
  • Info & Metrics
  • View PDF

More in this TOC Section

Original Articles

  • Association of Polypharmacy with Kidney Disease Progression in Adults with CKD
  • The Effect of Atrasentan on Kidney and Heart Failure Outcomes by Baseline Albuminuria and Kidney Function
  • Collectin11 and Complement Activation in IgA Nephropathy
Show more Original Articles

Glomerular and Tubulointerstitial Diseases

  • Kidney Histopathologic Spectrum and Clinical Indicators Associated with MGRS
  • Chronic Kidney Disease from Polyvinylpyrrolidone Deposition in Persons with Intravenous Drug Use
  • The Cost-Effectiveness of Belimumab and Voclosporin for Patients with Lupus Nephritis in the United States
Show more Glomerular and Tubulointerstitial Diseases

Cited By...

  • No citing articles found.
  • Google Scholar

Similar Articles

Related Articles

  • Correction: Prognostic Factors and Long-Term Outcome with ANCA-Associated Kidney Vasculitis in Childhood
  • PubMed
  • Google Scholar

Keywords

  • ANCA
  • vasculitis
  • pediatric nephrology
  • glomerulopathy
  • Antineutrophil cytoplasmic antibody

Articles

  • Current Issue
  • Early Access
  • Subject Collections
  • Article Archive
  • ASN Meeting Abstracts

Information for Authors

  • Submit a Manuscript
  • Trainee of the Year
  • Author Resources
  • ASN Journal Policies
  • Reuse/Reprint Policy

About

  • CJASN
  • ASN
  • ASN Journals
  • ASN Kidney News

Journal Information

  • About CJASN
  • CJASN Email Alerts
  • CJASN Key Impact Information
  • CJASN Podcasts
  • CJASN RSS Feeds
  • Editorial Board

More Information

  • Advertise
  • ASN Podcasts
  • ASN Publications
  • Become an ASN Member
  • Feedback
  • Follow on Twitter
  • Password/Email Address Changes
  • Subscribe to ASN Journals

© 2022 American Society of Nephrology

Print ISSN - 1555-9041 Online ISSN - 1555-905X

Powered by HighWire