Date: Monday, October 22, 2018
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children(1). There is limited data published regarding the clinical presentation of jSSc. The juvenile systemic scleroderma inception cohort is a multinational cohort with a prospective standardized assessment of the patients. We present the clinical characteristics of the patients at time of the inclusion in the cohort.
The juvenile scleroderma inception cohort is a prospective multicenter registry of patients with jSSc, who fullfill the adult classification criteria, are less than 16 years old at disease onset, and are less then 18 years of age at the time of inclusion in the cohort. We evaluated the patients characteristics at time of inclusion in the cohort.
One hundred and nine jSSc patients were enrolled and analyzed as of April 15th, 2018. The majority are female (79%) and Caucasian (90%). Diffuse cutaneous subtype was the most common (80%), with 15% having overlap features. The mean age of onset of Raynauds was 9.7 years in the diffuse subtype (djSSc) and 10.5 years in the limited subtype (ljSSc) (p=0.62). The mean age of non-Raynauds was 10.2 in the djSSc and 11.2 in the ljSSc (p=0.52). Mean disease duration at time of inclusion was 3.4 in the djSSc and 2.5 in the ljSSc group. Approximately 80% of all jSSc patients received DMARDs. ANA positivity was around 86% in both groups. Anti-Scl70 was 32% in djSSc and 39% in the ljSSc group. Anticentromere positivity was 4% in the djSSc and 11% in the ljSSc group(p=0.28). Mean Modified Rodnan skin score was 18.2 in the djSSc and 7.7 in the ljSSc (p=0.001). Gottron papulae were significantly more common the djSSc with 27% compared to 7% in the ljSSc group. History of ulceration was significantly more common in the djSSc with 57% compared to 29% in the ljSSc group (p=0.010). FVC<80% occured in 34% in the djSSc and 21% in the ljSSc group(p=0.295). DLCO <80% occured 44% in the djSSc and 43% in the ljSSc group. Pulmonary hypertension assessed by ultrasound occured 7% in both groups. No hypertension or renal crisis was reported or observed. Gastointestinal involvement occured 35% in the djSSc and 24% in the ljSSc (p=0.28). Number of swollen joints were observed around 20% in both groups. Muscle weakness wtih joint contractures were present in 12% in the djSSc and 28% in the ljSSc group. Tendon friction rub was present in 4 % in djSSc and 7% in the ljSSc group. djSSc patients had significantly worse scores for physician global disease activity (VAS 0-100), 41.6 compared to 30.7 ( p=0.041) and for physician global disease damage (VAS 0-100) 38.2 compared to 18.9 ( p=0.023).
In this large cohort of jSSc patients there are surprisingly not many significant differences between djSSc and ljSSc. According to the physician global, the djSSc patients have significantly more severe disease.
- Beukelman T, Xie F, Foeldvari I. Assessing the Prevalence of Juvenile Systemic Sclerosis in Childhood Using Administrative Claims Data from the United States. Journal fo Scleroderma and Related Disorders. 2018;3.
To cite this abstract in AMA style:Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Torok KS, Stanevicha V, Sztajnbok F, Terreri MT, Alexeeva E, Anton J, Katsicas MM, Smith V, Avcin T, Cimaz R, Kostik M, Lehman TJA, Sifuentes-Giraldo WA, Appenzeller S, Janarthanan M, Nemkova D, Santos MJ, Battagliotti C, Berntson L, Bica B, Brunner J, Costa Reis P, Eleftheriou D, Harel L, Horneff G, Kallinich T, Lazarevic D, Minden K, Nielsen SM, Uziel Y, Helmus N. Update from the Juvenile Scleroderma Inception Cohort [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 10). https://acrabstracts.org/abstract/update-from-the-juvenile-scleroderma-inception-cohort/. Accessed April 9, 2020.
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