Session Information
Session Type: Poster Session B
Session Time: 10:30AM-12:30PM
Background/Purpose: Juvenile systemic sclerosis(jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. In adult systemic sclerosis is interstitial lung disease (ILD) one of the main leading causes of mortality. High resolution computer tomography (HRCT) is the gold standard to assess ILD. To find risk factors or predictive factors for ILD in jSSc has a significant clinical importance.
Methods: We extracted data from patients till March 2025, who had an HRCT evaluation at the time of inclusion into the juvenile scleroderma inception cohort(jSScIC)(1). We compared the clinical presentation of patients with positive (HRCT+) and negative (HRCT-) findings for ILD.
Results: 216 patients had an HRCT result at time of the inclusion in the cohort. 76 of them were HRCT+. In both groups around 70% of the patients had diffuse subset. Median age at the time of the first non-Raynaud´s presentation was 10.8 and 11.3 in the HRCT+ versus HRCT- group. Median disease duration was around 2 years. Significantly more patients had decreased FVC< 80% (44% versus 25%, p=0.011) and DLCO< 80% (62% versus 36%, p=0.004) in the HRCT + then HRCT- group. Median MRSS was lower in the HRCT+ then HRCT- group (8.5 versus 12.5, p=0.031). Lower number of patients had sclerodactyly in the HRCT+ then HRCT- group (68% versus 81%, p=0.039). Nailfold capillary changes occurred more often in the HRCT+ then in HRCT- group (88% versus 73%, p=0.042). In the HRCT+ group pulmonary hypertension occurred more frequently (14% versus 2%, p=0.001). Oesophageal involvement was more frequent in the HRCT+ group (58% versus 34%, p=0.001). We could not find any significant difference regarding antibody profile, cardiac, renal and musculoskeletal involvement.
Conclusion: We found a remarkable clinical pattern associated with HRCT+, with increased capillary changes, more frequent oesophageal involvement and pulmonary hypertension and lower MRSS. This pattern may help to predict ILD. We must remark that we had no central reading of the HRCT imaging. We need more patients and more long term observation period to prove our findings. 1. Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Terreri MT, Sakamoto AP, et al. Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. Arthritis Care Res (Hoboken). 2022;74(10):1575–84.
To cite this abstract in AMA style:
Foeldvari I, Torok K, Kasapcopur O, Adrovic A, Sztajnbok F, Terreri M, Sakamoto A, Anton J, Khubchandani R, Johnson S, Özomay Baykal G, Feldman B, Schonenberg-Meinema D, Sawhney S, Smith V, Al-Abadi E, Appenzeller S, Hajek S, Katsicas M, Kostik M, Li S, Malcova H, Patwardhan A, Sifuentes-Giraldo W, Vasquez-Canizares N, Avcin T, Costa Reis P, Hetlevik S, Janarthanan M, Marrani E, Pain C, Santos M, Stanevica V, Abu Alsaoud S, Alexeeva E, Battagliotti C, Berntson L, bica b, Brunner J, Eleftheriou D, Harel L, Kallinich T, Lazarevic D, Minden K, Nemcova D, Nielsen S, Nuruzzaman F, Sparchez M, Uziel Y, Helmus N. A clear pattern of clinical presentation in juvenile systemic scleroderma is associated with interstitial lung disease [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/a-clear-pattern-of-clinical-presentation-in-juvenile-systemic-scleroderma-is-associated-with-interstitial-lung-disease/. Accessed .« Back to ACR Convergence 2025
ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-clear-pattern-of-clinical-presentation-in-juvenile-systemic-scleroderma-is-associated-with-interstitial-lung-disease/