Background: 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.

Objectives: To assess the difference in patients with jSSc with normal (NF-) and pathologic NF(NF+) findings at the time of inclusion in the cohort.

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.

This project was supported by an unrestricted grant from “Joachim Herz Stiftung”

REFERENCES: [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.

Acknowledgements: NIL.

Disclosure of Interests: None declared.