ACR Meeting Abstracts

ACR Meeting Abstracts

  • Meetings
    • ACR Convergence 2024
    • ACR Convergence 2023
    • 2023 ACR/ARP PRSYM
    • ACR Convergence 2022
    • ACR Convergence 2021
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • View and print all favorites
    • Clear all your favorites
  • ACR Meetings

Abstract Number: 2196

Identifying Specific Criteria for Juvenile Systemic Sclerosis: A Comparison of Adult and Pediatric Ratings

Suzanne Li1, Natalia Vasquez-Canizares2, Clare Pain3, Marinka Twilt4, Amra Adrovic5, Abdulrahman Alrasheed6, Simone Appenzeller7, ADELE CIVINO8, narendra bagri9, Patricia Costa Reis10, Muserref Cucueoglu11, Fatma Dedeoglu12, samundeeswari Deepak13, Jianghong Deng14, Dalia El-Ghoneimy15, Ivan Foeldvari16, Fernando Garcia-Rodriguez17, Marija Jelusic18, Ankur Jindal19, Ozgur Kasapcopur20, Maria Katsicas21, Archana Khan22, Raju Khubchandani23, Meiping Lu24, Hanna Lythgoe25, edoardo marrani26, Giorgia Martini27, Takako Miyamae28, Tomo Nozawa29, Seza Ozen30, Lidia Rutkowska-Sak31, Sunil Sampath13, Dieneke Schonenberg-Meinema32, Hongmei Song33, Betul Sozeri34, Hayakazu Sumida35, Maria Teresa Terreri36, Kathryn Torok37, Seyma Turkmen38, Dilara Unal38, Gong Yinv39, Emily Willis25, Rongjun Zheng24 and Brian Feldman40, and Pediatric International Consortium for Scleroderma, 1Hackensack Meridian School of Medicine, Joseph M. Sanzari Children's Hospital, Hackensack, NJ, 2Children's Hospital at Montefiore, Bronx, NY, 3Alderhey Children's NHS Foundation Trust, Liverpool, United Kingdom, 4Alberta Children's Hospital, Calgary, AB, Canada, 5Koc University Hospital, Istanbul, Turkey, 6King Abdullah Specialized Children Hospital, Riyadh, Saudi Arabia, 7Unicamp, Campinas, SP, Brazil, 8Pediatric Rheumatology "Vito Fazzi" Hospital LECCE, LECCE, Italy, 9All India Institute of Medical Sciences, New Delhi, Delhi, India, 10Hospital de Santa Maria, Lisbon, Portugal, 11Hacettepe University, Ankara, Turkey, 12Boston Children's Hospital, Boston, MA, 13THE NEWCASTLE UPON TYNE HOSPITALS NHS FOUNDATION TRUST, Newcastle upon Tyne, United Kingdom, 14Beijing Children's Hospital, Beijing, China, 15Children's Hospital, Ain Shams University, Cairo, Egypt, 16Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, 17Universidad Autonoma de Nuevo Leon, Monterrey, NL, Mexico, 18University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, 19Postgraduate Institute of Medical Education and Research, Chandigarh, India, 20Istanbul University-Cerrahpasa, Cerrahpasa Medical School, istanbul, Turkey, 21Hospital Garrahan, Buenos Aires, Argentina, 22SRCC Children's Hospital, Mumbai, India, Mumbai, India, 23The SRCC Children's Hospital, Mumbai, India, 24Department of Rheumatology Immunology and Allergy, Children's Hospital of Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China (People's Republic), 25Royal Manchester Children's Hospital, Manchester, United Kingdom, 26Dipartimento Neurofarba, Università di Firenze, Firenze, Italy, 27University of Udine University Hospital Santa Maria della Misericordia, Udine, Italy, 28Tokyo Women's Medical University, Tokyo, Japan, 29Yokohama City University, Yokohama, Japan, 30Department of Pediatrics, Hacettepe University, Ankara, Turkey, 31National Institute of Geriatrics, Rheumatology and Rehabilitation, Warszawa, Poland, 32Amsterdam University Medical Center, Amsterdam, Netherlands, 33Peking Union Medical College Hospital, Beijing, China, 34University of Health Sciences, Istanbul, Turkey, 35The University of Tokyo, Tokyo, Japan, 36UNIFESP, São Paulo, SP, Brazil, 37Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, 38University of Health Sciences, Umraniye Training and Research Hospital, Ankara, Turkey, 39Children's Hospital Affiliated to Fudan University, Shanghai, China (People's Republic), 40Division of Rheumatology, The Hospital for Sick Children; Departments of Pediatrics and Medicine, Faculty of Medicine; The Institute for Health Policy, Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada

Meeting: ACR Convergence 2024

Keywords: classification criteria, Pediatric rheumatology, Scleroderma, Systemic

  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print
Session Information

Date: Monday, November 18, 2024

Title: Pediatric Rheumatology – Clinical Poster III

Session Type: Poster Session C

Session Time: 10:30AM-12:30PM

Background/Purpose: Despite the high morbidity and mortality risk associated with juvenile systemic sclerosis (jSSc), evidence to guide management is limited.  No jSSc clinical trials have been conducted, and relying on adult SSc recommendations is suboptimal because of major disease differences including for subtype and morbidity patterns. JSSc is a rare disease, and existing SSc classification criteria (CC) have been found to have suboptimal sensitivity for jSSc.  Our Pediatric International Consortium for Scleroderma (PICS) group is working to develop high performing jSSc CC to enable jSSc treatment studies to be conducted.  This study was focused on identifying potential criteria for jSSc CC. 

Methods: PICS members were asked rate the appropriateness of items to serve as a criteria for jSSc CC from 1 (low) to 9 (high) in a web-based survey.  Queried features included the 23 items rated by adult SSc experts during their development of the 2013 ACR/EULAR adult SSc CC (van den Hoogen et al DOI 10.1002/art.38098).  The median appropriateness score of PICS respondents was calculated.  Items were ranked in order of 1) the median appropriateness rating and 2) average score.  Ranking by PICS members was compared to the previously reported adult expert ranking (Fransen et al DOI 10.1002/acr.20679).  Responses were also analyzed based upon number of jSSc patients seen. 

Results: Thirty-eight attending PICS members (mostly pediatric rheumatologists) from 18 countries answered the survey (84% response rate).  Respondents had been practicing for a median of 15-20 years, and saw a median of 11-15 jSSc patients.  Several differences were found between PICS and adult SSc expert ratings.  PICS members rated the 4 vasculopathy-related items higher than adult experts, placing them among the top 7 criteria and including one as the top criteria.  Adult experts rated only 2 among the top 7 criteria, and rated presence of skin thickening as the top criteria (Table 1).  Renal crisis, tendon friction rubs, and positivity of several SSc-associated autoantibodies were rated lower by PICS members than adult experts, except for the overlap associated autoantibody anti-PM/Scl, which was rated much higher.     

We compared the appropriateness scores of respondents stratified by number of jSSc patients seen.  Group A had seen 21 to > 60 jSSc patients, B 11-20 patients, and C 1-10 patients.  Most items were rated similarly (Figure 1).   Pulmonary arterial hypertension (PAH) was rated lower by Group A than B or C, and Group A had also seen PAH more commonly than other groups.  Tendon friction rubs were less commonly seen by Group C than A or B.  Most other items were reported to be similarly prevalent across groups.

Conclusion: We found several differences in the rating of potential criteria items for SSc CC between adult and pediatric rheumatologists who specialize in SSc.  A major difference was the higher rating of vasculopathy-related items by pediatric rheumatologists. PICS members had similar ratings for most features Irrespective of the number of jSSc patients they saw.  Our findings support the appropriateness of developing jSSc-specific CC, and suggest that modifications of the 2013 ACR/EULAR adult SSc CC could improve its performance for jSSc.

Supporting image 1

Table 1

Supporting image 2

Figure 1


Disclosures: S. Li: Boehringer-Ingelheim, 2, Merck/MSD, 3; N. Vasquez-Canizares: Boehringer-Ingelheim, 2; C. Pain: None; M. Twilt: None; A. Adrovic: None; A. Alrasheed: None; S. Appenzeller: None; A. CIVINO: None; n. bagri: None; P. Costa Reis: None; M. Cucueoglu: None; F. Dedeoglu: UpToDate, 9; s. Deepak: None; J. Deng: None; D. El-Ghoneimy: None; I. Foeldvari: Boehringer-Ingelheim, 1, Eli Lilly, 6, miirsubishi, 2; F. Garcia-Rodriguez: None; M. Jelusic: None; A. Jindal: None; O. Kasapcopur: None; M. Katsicas: None; A. Khan: None; R. Khubchandani: None; M. Lu: None; H. Lythgoe: None; e. marrani: None; G. Martini: None; T. Miyamae: None; T. Nozawa: None; S. Ozen: Novartis, 2, 6, SOBI, 2, 6; L. Rutkowska-Sak: None; S. Sampath: None; D. Schonenberg-Meinema: None; H. Song: None; B. Sozeri: None; H. Sumida: None; M. Terreri: None; K. Torok: None; S. Turkmen: None; D. Unal: None; G. Yinv: None; E. Willis: None; R. Zheng: None; B. Feldman: None.

To cite this abstract in AMA style:

Li S, Vasquez-Canizares N, Pain C, Twilt M, Adrovic A, Alrasheed A, Appenzeller S, CIVINO A, bagri n, Costa Reis P, Cucueoglu M, Dedeoglu F, Deepak s, Deng J, El-Ghoneimy D, Foeldvari I, Garcia-Rodriguez F, Jelusic M, Jindal A, Kasapcopur O, Katsicas M, Khan A, Khubchandani R, Lu M, Lythgoe H, marrani e, Martini G, Miyamae T, Nozawa T, Ozen S, Rutkowska-Sak L, Sampath S, Schonenberg-Meinema D, Song H, Sozeri B, Sumida H, Terreri M, Torok K, Turkmen S, Unal D, Yinv G, Willis E, Zheng R, Feldman B. Identifying Specific Criteria for Juvenile Systemic Sclerosis: A Comparison of Adult and Pediatric Ratings [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/identifying-specific-criteria-for-juvenile-systemic-sclerosis-a-comparison-of-adult-and-pediatric-ratings/. Accessed .
  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print

« Back to ACR Convergence 2024

ACR Meeting Abstracts - https://acrabstracts.org/abstract/identifying-specific-criteria-for-juvenile-systemic-sclerosis-a-comparison-of-adult-and-pediatric-ratings/

Advanced Search

Your Favorites

You can save and print a list of your favorite abstracts during your browser session by clicking the “Favorite” button at the bottom of any abstract. View your favorites »

All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, academic institutions, private organizations, and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part of editorial news coverage) is under media embargo until 10:00 AM ET on November 14, 2024. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

Violation of this policy may result in the abstract being withdrawn from the meeting and other measures deemed appropriate. Authors are responsible for notifying colleagues, institutions, communications firms, and all other stakeholders related to the development or promotion of the abstract about this policy. If you have questions about the ACR abstract embargo policy, please contact ACR abstracts staff at [email protected].

Wiley

  • Online Journal
  • Privacy Policy
  • Permissions Policies
  • Cookie Preferences

© Copyright 2025 American College of Rheumatology