Article
Is there a Difference in the Presentation of male and female Patients with diffuse subtype of juvenile systemic Sclerosis? Results from the juvenile Scleroderma Inception Cohort www.juvenile-scleroderma.com
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Published: | September 4, 2017 |
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Background: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Several adult publications looked at the differences between the clinical presentation of male and female patients with Systemic Sclerosis. There is a rarity of data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort (http://www.juvenile-scleroderma.com/) is a prospective standardized register for patients with jSSc.
Methods: Patients with jSSc were included worldwide to the juvenile scleroderma inception cohort. We compared the demographics and clinical characteristics of male and female patients.
Results: Up till now 88 patients were enrolled, 62 (70%) with djSSc 11/62 (18%) of the patients were male (M) and 51/62 female (F) (82%). The mean disease duration at the time of inclusion was 3.5 in M and 3.6 in F patients. 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 inclusion the mean modified Rodnan Skin Score 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%) (p=0.035) in M and 0/23 (0%) in F patients. 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. Only 6 F patients had pulmonary hypertension. 7/11 (64%) of M and 11/51 (22%) of F patients had signs of interstitial lung disease (p=0.005). Renal involvement was 2/11 (18%) in M and 3/51 (6%) in F patients. 37% in both sexes had gastrointestinal involvement. 9/11 (82%) of M and 26/50 (52%) in F patients had musculoskeletal 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).
Conclusion: We present the data of the first 62 diffuse subtype patients with jSSc included in our cohort. Male Patients presented a significantly more severe disease similar to adult male patients.