gms | German Medical Science

Deutscher Rheumatologiekongress 2024

52. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh)
34. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)
38. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh)

18.09. - 21.09.2024, Düsseldorf

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Presence of nailfold capillary changes correlates with more severe organ involvement in juvenile systemic scleroderma. Results of the juvenile scleroderma inception cohort

Meeting Abstract

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  • Ivan Foeldvari - Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg
  • Jens Klotsche - German Rheumatism Research Center, Berlin
  • Kathryn Torok - jSSc collaborative group, Hamburg
  • Ozgur Kasapcopur - jSSc collaborative group, Hamburg
  • Amra Adrovic - jSSc collaborative group, Hamburg
  • Brian Feldman - jSSc collaborative group, Hamburg
  • Jordi Anton - jSSc collaborative group, Hamburg
  • Sindhu Johnson - jSSc collaborative group, Hamburg
  • Flavio Sztajnbok - jSSc collaborative group, Hamburg
  • Maria Teresa Terreri - jSSc collaborative group, Hamburg
  • Ana Paula Sakamoto - jSSc collaborative group, Hamburg
  • Valda Stanevicha - jSSc collaborative group, Hamburg
  • Dieneke Schonenberg - jSSc collaborative group, Hamburg
  • Ekaterina Alexeeva - jSSc collaborative group, Hamburg
  • Maria Katsicas - jSSc collaborative group, Hamburg
  • Raju Khubchandani - jSSc collaborative group, Hamburg
  • Sujata Sawhney - jSSc collaborative group, Hamburg
  • Vanessa Smith - jSSc collaborative group, Hamburg
  • Eslam Al-Abadi - jSSc collaborative group, Hamburg
  • Simone Appenzeller - jSSc collaborative group, Hamburg
  • Tadey Avcin - jSSc collaborative group, Hamburg
  • Mikhail Kostik - jSSc collaborative group, Hamburg
  • Thomas Lehman - jSSc collaborative group, Hamburg
  • Hana Malcova - jSSc collaborative group, Hamburg
  • Edoardo Marrani - jSSc collaborative group, Hamburg
  • Clare Pain - jSSc collaborative group, Hamburg
  • Anjali Patwardhan - jSSc collaborative group, Hamburg
  • W.-Alberto Sifuentes-Giraldo - jSSc collaborative group, Hamburg
  • Natalia Vasquez-Canizares - jSSc collaborative group, Hamburg
  • Sima Abu Al-Saoud - jSSc collaborative group, Hamburg
  • Patricia Costa Reis - jSSc collaborative group, Hamburg
  • Mahesh Janarthanan - jSSc collaborative group, Hamburg
  • Dana Nemcova - jSSc collaborative group, Hamburg
  • Maria Jose Santos - jSSc collaborative group, Hamburg
  • Christina Battagliotti - jSSc collaborative group, Hamburg
  • Lillemor Berntson - jSSc collaborative group, Hamburg
  • Blanca Bica - jSSc collaborative group, Hamburg
  • Jürgen Brunner - jSSc collaborative group, Hamburg
  • Despina Eleftheriou - jSSc collaborative group, Hamburg
  • Liora Harel - jSSc collaborative group, Hamburg
  • Gerd Horneff - jSSc collaborative group, Hamburg
  • Daniela Kaiser - jSSc collaborative group, Hamburg
  • Tilmann Kallinich - jSSc collaborative group, Hamburg
  • Dragana Lazarevic - jSSc collaborative group, Hamburg
  • Kirsten Minden - jSSc collaborative group, Hamburg
  • Farzana Nuruzzaman - jSSc collaborative group, Hamburg
  • Siri Opsahl Hetlevik - jSSc collaborative group, Hamburg
  • Mihaela Spirchez - jSSc collaborative group, Hamburg
  • Yosef Uziel - jSSc collaborative group, Hamburg
  • Nicola Helmus - Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg

Deutsche Gesellschaft für Rheumatologie. Deutsche Gesellschaft für Orthopädische Rheumatologie. Gesellschaft für Kinder- und Jugendrheumatologie. Deutscher Rheumatologiekongress 2024, 52. Kongress der Deutschen Gesellschaft für Rheumatologie und Klinische Immmunologie (DGRh), 34. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR), 38. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh). Düsseldorf, 18.-21.09.2024. Düsseldorf: German Medical Science GMS Publishing House; 2024. DocKI.12

doi: 10.3205/24dgrh119, urn:nbn:de:0183-24dgrh1195

Veröffentlicht: 18. September 2024

© 2024 Foeldvari et al.
Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). Lizenz-Angaben siehe http://creativecommons.org/licenses/by/4.0/.

Gliederung

Text

Introduction: 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 1st 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 [Tab. 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.

Gliederung

References

1.
Vanhaecke A, Cutolo M, Distler O, Riccieri V, Allanore Y, Denton CP, Hachulla E, Ingegnoli F, Deschepper E, Avouac J, Jordan S, Launay D, Melsens K, Pizzorni C, Sulli A, Vasile M, Herrick AL, Smith V. Nailfold capillaroscopy in SSc: innocent bystander or promising biomarker for novel severe organ involvement/progression? Rheumatology (Oxford). 2022 Nov 2;61(11):4384-96. DOI: 10.1093/rheumatology/keac079 Externer Link
2.
Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Terreri MT, Sakamoto AP, Stanevicha V, Sztajnbok F, Anton J, Feldman B, Alexeeva E, Katsicas M, Smith V, Avcin T, Marrani E, Kostik M, Lehman T, Sifuentes-Giraldo WA, Vasquez-Canizares N, Appenzeller S, Janarthanan M, Moll M, Nemcova D, Patwardhan A, Santos MJ, Sawhney S, Schonenberg-Meinema D, Battagliotti C, Berntson L, Bica B, Brunner J, Costa-Reis P, Eleftheriou D, Harel L, Horneff G, Kaiser D, Kallinich T, Lazarevic D, Minden K, Nielsen S, Nuruzzaman F, Uziel Y, Helmus N, Torok KS. Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. Arthritis Care Res (Hoboken). 2022 Oct;74(10):1575-84. DOI: 10.1002/acr.24609 Externer Link