Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Several adult publications looked at the differences between limited (ljSSc) and diffuse subtype (djSSc). There is few data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort is a prospective standardized register for patients with jSSc.
Methods: Patients with jSSc were included worldwide to the jSSc cohort. Patients fulfilled the ACR criteria for jSSc. We compared the demographics and clinical features of the ljSSc and djSSc patients.
Results: Up till now 80 patients were enrolled, 58 (72.5%) with djSSc and 22 (27.5%) with ljSSc. 10% in djSSc and 23% in ljSSc showed overlap features. Disease duration at time of inclusion in the cohort was 3.7 years in the djSSc and 3.0 years in ljSSc. 83% in the djSSc and 81% in the ljSSc group were female. The mean age at onset of Raynaud symptoms was 9.0 years in the jdSSc and 10.4 years in ljSSc group and the mean age at onset of the non-Raynaud symptoms was 9.4 in djSSc and 10.9 ljSSc. At the time of inclusion the mean of modified Rodnan Skin Score was 18.2 in the djSSc and 9.1 in ljSSc. Anti-Scl 70 positivity was found in 30.4% of djSSc and 33.3% in ljSSc. Only 2 patients in the djSSc group and 2 in the ljSSc group presented anticentromere positive. Capillary changes occurred in 62.1% in the djSSc and 54.5% in ljSSc . History of active ulceration was present in 56% in the djSSc and in18% in ljSSc. Active ulcerations were present in 21.1% in the djSSc and 4.5% in the ljSSc. The mean of 6 Minute walk test was 392 m in the djSSc group and 504 m in the ljSSc group. 15.5% of djSSc and 41% of ljSSc had cardiac involvement. Pulmonary hypertension occurred in 8% in djSSc and 13% in ljSSc. 56% in djSSc and 31% in ljSSc group had signs of interstitial lung disease on imaging. Renal involvement occurred in 7% in djSSc and 4.5 % in ljSSc. None had systemic hypertension. 38% of djSSc and 18% of ljSSc had gastrointestinal involvement. 57% in djSSc and 73% in ljSSc had musculoskeletal involvement. Patient global disease activity measured by VAS was 44.2 in the djSSc and 46.3 in ljSSc. Patient global disease damage was 42.6 in the djSSc and 33.8 in ljSSc. Physician global disease activity measured by VAS was 40.3 in the djSSc and 25 in the ljSSc and physician global disease damage measured by VAS was 35.4 in the djSSc and 15.0 in the ljSSc.Mean CHAQ score was 0.4 in both groups.
Conclusion: Patients with djSSc were younger at onset; have more often capillary changes, active ulcerations and gastrointestinal involvement, and less pulmonary hypertension and musculoskeletal involvement. They also present moresevere disease and more disease damage. Features of the pediatric subtypes of jSSc differ from adults with SSc, especially the high proportion of patients with diffuse subtype.
To cite this abstract in AMA style:Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Stanevicha V, Terreri MT, Alexeeva E, Katsicas MM, Cimaz R, Kostik M, Lehman TJA, Sifuentes-Giraldo WA, Smith V, Sztajnbok F, Avcin T, Santos MJ, Nemkova D, Battagliotti C, Eleftheriou D, Harel L, Janarthanan M, Kallinich T, Anton J, Minden K, Nielsen SM, Torok KS, Uziel Y, Helmus N. Is There a Difference in the Presentation of Diffuse and Limited Subtype in Childhood? Results from the Juvenile Scleroderma Inception Cohort [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/is-there-a-difference-in-the-presentation-of-diffuse-and-limited-subtype-in-childhood-results-from-the-juvenile-scleroderma-inception-cohort/. Accessed October 21, 2021.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/is-there-a-difference-in-the-presentation-of-diffuse-and-limited-subtype-in-childhood-results-from-the-juvenile-scleroderma-inception-cohort/