Date: Monday, November 6, 2017
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
In adult systemic scleroderma patients male have a more sever course. This issue was never evaluated in a larger juvenile scleroderma cohort.
Patients with jSSc were included worldwide to the jSScI cohort. We compared the demographics and clinical characteristics of male and female dcjSSc patients at the time of the enrollment into the jSSci.
Sixty-two dcjSSc patients were enrolled Up till April 2016. 11/62 (18%) of the patients were male (M) and 51/62 female (F) (82%). The mean age of the onset of Raynaud symptoms was 8.0 in M and 9.4 years in the F patients and the non-Raynaud symptoms was 8.2 in M and 10.0 in F patients. At the time of the enrollment the mean MRSS was 24.3 in M and 17.3 in F patients. Anti-Scl 70 positivity was found in 4/11 (36.4%) in M and 14/49 (28.6%) in F patients. Anticentromere positivity occurred in 2/11 (18.2%) in M and 0/23 (0%) in F patients (p=0.035). Capillary changes were present in 8/11(73%) of the M and 30/51 (59%) of F patients, but 36% of M and F had already history of ulcerations. 7/11 (64%) of the M and 21/51 (41%) of the F patients had cardiopulmonary involvement. pulmonary hypertension occurred in F patients (n=6). 7/11 (64%) of M and 11/51 (22%) of F patients had signs of interstitial lung disease (p=0.005). No renal crisis was observed till enrollment. 37% in both sexes had gastrointestinal involvement. Patient global disease damage was on a VAS (0- 100) 56.9 in M and in 38.4 in F (p=0.014) and patient global disease activity was 58.8 in M and 41.9 in M (p=0.024). Physician global of disease activity on a VAS was 58.9 in M and 36.9 in F (p=0.004) and physician global disease damage was 60.2 in M and 31.2 in F (p=0.001).
Male patients had a significantly more severe disease course at time point of enrollment . This finding overlaps to the observation in adult cohorts.
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. Male Patients Have a More Severe Course As Female Patients with Diffuse Juvenile Systemic Scleroderma? Results from the Juvenile Scleroderma Inception Cohort Www.Juvenile-Scleroderma.Com [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/male-patients-have-a-more-severe-course-as-female-patients-with-diffuse-juvenile-systemic-scleroderma-results-from-the-juvenile-scleroderma-inception-cohort-www-juvenile-scleroderma-com/. Accessed September 24, 2021.
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