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.
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.
Authors: Ivan Foeldvari; Alan Tyndall; Francesco Zulian; Ulf Müller-Ladner; László Czirjak; Chris Denton; Ottilia Kowal-Bielecka; Dominique Farge Bancel; Marco Matucci-Cerinic Journal: Rheumatology (Oxford) Date: 2012-06-22 Impact factor: 7.580
Authors: E C LeRoy; C Black; R Fleischmajer; S Jablonska; T Krieg; T A Medsger; N Rowell; F Wollheim Journal: J Rheumatol Date: 1988-02 Impact factor: 4.666
Authors: G Martini; F Vittadello; O Kasapçopur; S Magni Manzoni; F Corona; C Duarte-Salazar; D Nemcova; C A Len; S M Garay; S Ullman; F Zulian Journal: Rheumatology (Oxford) Date: 2008-10-14 Impact factor: 7.580
Authors: Christine Peoples; Thomas A Medsger; Mary Lucas; Bedda L Rosario; Carol A Feghali-Bostwick Journal: J Scleroderma Relat Disord Date: 2016-07-23