Session Type: ACR Concurrent Abstract Session
Session Time: 11:00AM-12:30PM
Juvenile systemic sclerosis (jSSc) patients with overlap features seems to have a better long term outcome1. There is currently no data, where the clinical characteristics of patients with and without overlap features were compared.
Patients, fulfilling the PRES jSSc-classification criteria were included. Organ involvement was assessed using the standardized assessment. Patients were classified to diffuse (dcjSSc) and limited (lcjSSc) subtype and grouped as patients with and without overlap features.
Till April 2016 eighty patients were enrolled. We present data at time point of the enrollment. 11 of the patients had overlap features (oJSSc). 6 of 58 (10%) in the dcjSSc and 5 of 22 (23%) in the lcjSSc. Most patients were Caucasian. Female/male ratio differed in the groups, in the nojSSc 3.9:1 and in the ojSSc 11:1 ( p=0.1). Mean disease duration was 3.6 years in the non-overlap features group (nojSSc) and 3.2 years in the ojSSc group. Patients with nojSSc were 35% Scl-70 positive and ojSSc group none (p=0.032). Decreased DLCO occurred in 48% in the nojSSc and in 100% in the ojSSc group (p=0.088). Renal involvement was more frequent in the ojSSc group with 18% compared to 4% the nojSSc (p=0.078). Swollen joints occurred in 63% in the ojSSc and in 29% in the nojSSc group (p<0.001). Muscle weakness combined with joint contractures occurred in 43% in the ojSSc and in 8% in the nojSSc group (p=0.011).
Patients with overlap features have distinct characteristics compared with patients with nojSSc. This charactheristics were no Scl-70 positivity, higher portion of interstial lung disease, renal involvement, arthritis and muscle involvement accompanied by joint contractures. The future larger patient data collection in our cohort will help to make the differences more accentuated.
1. Foeldvari I, Nihtyanova SI, Wierk A, Denton CP. Characteristics of Patients with Juvenile Onset Systemic Sclerosis in an Adult Single-center Cohort. J Rheumatol 2010;37:2422- 6.
To cite this abstract in AMA style:Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Stanevicha V, Terreri MT, Alexeeva E, Katsicas MM, Smith V, Cimaz R, Kostik M, Lehman TJA, Anton J, Sifuentes-Giraldo WA, Sztajnbok F, Avcin T, Janarthanan M, Santos MJ, Nemkova D, Battagliotti C, Eleftheriou D, Harel L, Kallinich T, Minden K, Nielsen SM, Torok KS, Uziel Y, Stevens A, Pilkington C, Helmus N. Are Patients with Overlap Features Different from Patients without? Results from the Juvenile Systemic Scleroderma Cohort Www.Juvenile-Scleroderma.Com [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/are-patients-with-overlap-features-different-from-patients-without-results-from-the-juvenile-systemic-scleroderma-cohort-www-juvenile-scleroderma-com/. Accessed September 20, 2021.
« Back to 2017 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/are-patients-with-overlap-features-different-from-patients-without-results-from-the-juvenile-systemic-scleroderma-cohort-www-juvenile-scleroderma-com/