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

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.

Table 1

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 .

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