Presence of Nailfold Capillary Changes Correlates with More Severe Organ Involvement in Juvenile Systemic Scleroderma. Results of the Juvenile Scleroderma Inception Cohort

Ivan Foeldvari¹, Jens Klotsche², Kathryn Torok³, Ozgur Kasapcopur⁴, Amra Adrovic⁵, Brian Feldman⁶, Jordi Anton⁷, Sindhu R. Johnson⁸, Flavio Sztajnbok⁹, Maria Teresa Terreri¹⁰, Ana Sakamoto¹¹, Valda Stanevica¹², Dieneke Schonenberg-Meinema¹³, Ekaterina Alexeeva¹⁴, Maria Katsicas¹⁵, Raju Khubchandani¹⁶, Sujata Sawhney¹⁷, vanessa smith¹⁸, Eslam Al-Abadi¹⁹, Simone Appenzeller²⁰, Tadey Avcin²¹, Mikhail Kostik²², Thomas Lehman²³, Hana Malcova²⁴, edoardo marrani²⁵, Clare Pain²⁶, Anjali Patwardhan²⁷, Walter Alberto Sifuentes-Giraldo²⁸, Natalia Vasquez-Canizares²⁹, Sima Abu Alsaoud³⁰, Patricia Costa Reis³¹, Mahesh Janarthanan³², Dana Nemcova³³, Maria Jose Santos³⁴ and Nicola Helmus³⁵, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁴Istanbul University-Cerrahpasa, Cerrahpasa Medical School, istanbul, Turkey, ⁵Cerrahpasa Medical School, Istanbul, Turkey, ⁶Division 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, ⁷Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, ⁸University of Toronto, Toronto, Canada, ⁹UFRJ/UERJ, SAO PAULO, Brazil, ¹⁰UNIFESP, São Paulo, SP, Brazil, ¹¹Federal University of São Paulo (UNIFESP), São Paulo, SP, Brazil, ¹²Childrens Clinical University Hospital, Zemgales priekšpilseta, Riga, Latvia, ¹³Emma Children’s Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands, ¹⁴National Medical Research Center of Children's Health, Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Moscow, Russia, ¹⁵Hospital Garrahan, Buenos Aires, Argentina, ¹⁶SRCC Childrens Hospital, Mumbai, India, ¹⁷Sir Ganga Ram Hospital, Sector 37 noida, Uttar Pradesh, India, ¹⁸Ghent University Hospital, Gent, Belgium, ¹⁹Birmingham Women’s and Children’s Hospital NHSFT, Birmingham, United Kingdom, ²⁰Unicamp, Campinas, SP, Brazil, ²¹University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia, ²²Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ²³Hospital for Special Surgery, New York, NY, ²⁴Motol University Hospital, Prague, Czech Republic, ²⁵Dipartimento Neurofarba, Università di Firenze, Firenze, Italy, ²⁶Alderhey Children's NHS Foundation Trust, Liverpool, United Kingdom, ²⁷University of Missouri-Columbia, Department of Child Health, 404 N Keene Street, Columbia MO 65210, Columbia, ²⁸Hospital Universitario Ramon y Cajal, Madrid, Spain, ²⁹Children’s Hospital at Montefiore, Bronx, NY, ³⁰Caritas baby Hospital, East Jerusalem, Israel, ³¹Hospital de Santa Maria, Lisbon, Portugal, ³²SRI RAMACHANDRA INSTITUTE OF HIGHER EDUCATION AND RESEARCH, Chennai, Tamil Nadu, India, ³³MD, Prague, Czech Republic, ³⁴Hospital Garcia de Orta and Centro Académico de Medicina de Lisboa, Lisboa, Portugal, ³⁵Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany

Meeting: ACR Convergence 2024

Keywords: Pediatric rheumatology, Systemic sclerosis

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: Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence in 3 in 1 000 000 children. Positive nailfold capillaroscopy (NF+) finding correlate with more severe disease in adult systemic scleroderma [1]. There is currently no data if this correlation does exist in jSSc.

Methods: Baseline data was extracted from patients enrolled in the juvenile scleroderma inception cohort that had nailfold capillaroscopy performed at inclusion [2] until 1^st of December 2023. NF was performed by dermatoscope and/or high resolution video nailfold capillaroscopy. We compared patients with NF+ and NF- findings from the baseline visit using chi-square test.

Results: 237 patients were included in the analysis, 185 (78%) of them were female. 126 (70%) had diffuse subtype. 183/237 patients (77%) were in the NF+ group. 71% in the NF+ group were Caucasian compared to 85% in the NF- group (p=0.051). Median disease duration was 2.3 years in the NF+ and 3.2 years in the NF- patients. Median age at onset of the first non-Raynaud´s was around 11 years in both groups. More patients in the NF+ group were ANA positive (95% compared to 79%, p< 0.001). There was no difference in the anti-Scl70 or anti-centromere distribution.
NF+ patients had significantly more frequent Raynaud phenomenon (96% compared to 78%, p< 0.001); history of digital ulcerations (59% compared to 27%, p< 0.001); abnormal high resolution CT findings of the lung (49% compared to 30%, p=0.034); overall gastrointestinal involvement (49% compared to 20%, p< 0.001); oesophageal involvement (47% compared to 19%, p< 0.001); musculoskeletal involvement (71% compared to 41%, p=0.003); presence of joints with decreased range (63% versus 45%, p=0.022) and presence of muscle weakness (25% compared to 3%, p=0.002). No significant differences were demonstrated in involvement of other organ systems such as skin, cardiac or renal. (see table 1)

Conclusion: In a jSSc cohort there were significantly more patients affected within various organ systems in those with nailfold capillary changes at enrollment compared to those without. Future studies should assess whether these differences persist over time.

[1] Vanhaecke A, Cutolo M, Distler O, et al. Nailfold capillaroscopy in SSc: innocent bystander or promising biomarker for novel severe organ involvement/progression? Rheumatology (Oxford). 2022 Nov 2;61(11):4384-4396.

[2] Foeldvari I, Klotsche J, Kasapcopur O, et al. Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. Arthritis Care Res (Hoboken). 2022 Oct;74(10):1575-1584.

Table 1

Disclosures: I. Foeldvari: Boehringer-Ingelheim, 1, Eli Lilly, 6, miirsubishi, 2; J. Klotsche: None; K. Torok: None; O. Kasapcopur: None; A. Adrovic: None; B. Feldman: None; J. Anton: None; S. Johnson: None; F. Sztajnbok: None; M. Terreri: None; A. Sakamoto: None; V. Stanevica: None; D. Schonenberg-Meinema: None; E. Alexeeva: Johnson & Johnson, 6, Novartis, 6, Roche, 6; M. Katsicas: None; R. Khubchandani: None; S. Sawhney: None; v. smith: Argenx, 2, BKC Moving Media Makers, 6, Boehringer-Ingelheim, 2, 5, 6, Janssen, 2, 5, 6, WebMDD Global LLC, 2; E. Al-Abadi: None; S. Appenzeller: None; T. Avcin: None; M. Kostik: None; T. Lehman: None; H. Malcova: None; e. marrani: None; C. Pain: None; A. Patwardhan: None; W. Sifuentes-Giraldo: None; N. Vasquez-Canizares: Boehringer-Ingelheim, 2; S. Abu Alsaoud: None; P. Costa Reis: None; M. Janarthanan: None; D. Nemcova: None; M. Santos: None; N. Helmus: None.

To cite this abstract in AMA style:

Foeldvari I, Klotsche J, Torok K, Kasapcopur O, Adrovic A, Feldman B, Anton J, Johnson S, Sztajnbok F, Terreri M, Sakamoto A, Stanevica V, Schonenberg-Meinema D, Alexeeva E, Katsicas M, Khubchandani R, Sawhney S, smith v, Al-Abadi E, Appenzeller S, Avcin T, Kostik M, Lehman T, Malcova H, marrani e, Pain C, Patwardhan A, Sifuentes-Giraldo W, Vasquez-Canizares N, Abu Alsaoud S, Costa Reis P, Janarthanan M, Nemcova D, Santos M, Helmus N. Presence of Nailfold Capillary Changes Correlates with More Severe Organ Involvement in Juvenile Systemic Scleroderma. Results of the Juvenile Scleroderma Inception Cohort [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/presence-of-nailfold-capillary-changes-correlates-with-more-severe-organ-involvement-in-juvenile-systemic-scleroderma-results-of-the-juvenile-scleroderma-inception-cohort/. Accessed .

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