AB0764 Comparison of disease characteristics in patients with juvenile-onset and adult-onset progressive systemic sclerosis

Objective: To characterize the clinical and laboratory profile of juvenile-onset compared to adult-onset systemic sclerosis in a large Brazilian cohort. Methods: Retrospective analysis of a cohort of 1016 systemic sclerosis patients followed at the Scleroderma Outpatient Clinic from two referral university centers in Brazil. Patients were classified as systemic sclerosis according to the 1980 American College of Rhaumatology (ACR) criteria. Juvenile-onset systemic sclerosis was defined if age at onset was <16 years. Results: Thirty-one (3.1%) patients were classified as juvenile-onset systemic sclerosis. These patients were predominantly females (90.3%), Caucasians (71.0%), and presented diffuse systemic sclerosis (51.6%), with mean age at onset of 12.71 years. Compared to the adult-onset patients, juvenile onset was associated with diffuse systemic sclerosis (p < 0.001), calcinosis (p < 0.001), myositis (p = 0.050), and lower frequency of interstitial lung disease (p = 0.050), pulmonary hypertension (p = 0.035), and esophageal (p = 0.005) involvement. Conclusion: Juvenile-onset systemic sclerosis characterized a distinct clinical pattern in this large series of systemic sclerosis patients, since it was predominantly associated with diffuse systemic sclerosis without significant organ involvement.

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Characteristics of Patients with Juvenile Onset Systemic Sclerosis in an Adult Single-center Cohort

The Journal of Rheumatology ◽

10.3899/jrheum.100001 ◽

2010 ◽

Vol 37 (11) ◽

pp. 2422-2426 ◽

Cited By ~ 28

Author(s):

IVAN FOELDVARI ◽

SVETLANA I. NIHTYANOVA ◽

ANGELA WIERK ◽

CHRISTOPHER P. DENTON

Keyword(s):

Systemic Sclerosis ◽

Survival Rates ◽

Disease Onset ◽

High Survival ◽

Adult Onset ◽

Single Center ◽

Juvenile Onset ◽

Pulmonary Arterial ◽

The Mean ◽

Onset Group

Objective.Systemic sclerosis (SSc) is a rare connective tissue disease in childhood. We compared the characteristics of adult patients with juvenile-onset SSc (jSSc) from a single-center cohort to an adult-onset group.Methods.Patients with disease onset before the age of 17 years were included in the jSSc cohort, while subjects with SSc onset after age 17 formed the adult-onset cohort.Results.We identified 52 adult subjects with jSSc and compared them to 954 patients with adult-onset SSc. The mean ± SD age at disease onset of the patients with jSSc was 14 ± 2 years, 39 (75%) of them were women, and 24 (46%) had the diffuse cutaneous subset of SSc (dcSSc). There were no differences between the 2 cohorts in terms of sex and disease subset. Overlaps were significantly more frequent among the jSSc cohort (37%) compared to the adult-onset group (18%; p = 0.002). Autoantibody analysis demonstrated significantly more antitopoisomerase I antibody-positive subjects (33% vs 20%; p = 0.034) and significantly fewer anticentromere antibody-positive subjects (2% vs 25%; p < 0.001) in the jSSc cohort. Compared to the adult-onset group at 10 years from disease onset, survival was significantly higher among the subjects with jSSc (98% vs 75%; p = 0.001), pulmonary arterial hypertension had a significantly lower incidence (2% vs 14%; p = 0.032), and there was no difference in terms of pulmonary fibrosis (22% vs 21%) and cardiac scleroderma (3% vs 2%) between the 2 groups.Conclusion.The high survival rates and lower proportion of dcSSc in the adult jSSc cohort may represent a survival bias.

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Juvenile Onset Systemic Sclerosis

Journal of the Royal Society of Medicine ◽

10.1177/014107688407700916 ◽

1984 ◽

Vol 77 (9) ◽

pp. 793-794 ◽

Cited By ~ 8

Author(s):

S M Burge ◽

T J Ryan ◽

R P R Dawber

Keyword(s):

Systemic Sclerosis ◽

Progressive Systemic Sclerosis ◽

Crest Syndrome ◽

Juvenile Onset ◽

Anticentromere Antibody

A case of progressive systemic sclerosis (CREST syndrome), which began in childhood, is described. Juvenile onset CREST syndrome is extremely rare; there are few well documented cases and no published series of children with this form of progressive systemic sclerosis. An anticentromere antibody was recently detected in this case and its significance is discussed.

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