OBJECTIVE: The aim of the present study was to explore the long-term outcome and clinical characteristics of adult patients with juvenile onset in the EULAR Scleroderma Trials and Research (EUSTAR) cohort and compare them with adult patients with onset between 20 and 40 years of age. METHODS: From the EUSTAR SSc cohort two patient groups were analysed: patients with juvenile SSc (jSSc) who are adults at present, and patients diagnosed between the age of 20 and 40 years (aSSc). Demographic data of the patients, organ involvement and outcome of the disease were examined using the Minimal Essential Data Set database system. RESULTS: From 5000 patients in the EUSTAR cohort, 60 patients (1.2%) with jSSc and 910 patients (18%) with aSSc were selected according the inclusion criteria. In the jSSc group, the mean age of disease onset was 12.4 years (range 2-15.9 years), and in the aSSc group, the mean age was 32 years (range 20-40 years). Disease subsets were similar. The antibody profile was also comparable except for ACAs, which were positive in 5% of the jSSc group and 26.9% of the aSSc group (P<0.005). Organ involvement (lung, kidney, joint, muscle and heart) was similar in the two groups of patients at the time of the last follow-up. CONCLUSION: The subset distribution in the jSSc and aSSc cohorts was found to be similar. Only the frequency of ACAs was significantly lower in the jSSc, which supports the hypothesis that the SSc patients with paediatric onset in the adult cohort may represent a distinct subgroup of the complete cohort of paediatric patients.
OBJECTIVE: The aim of the present study was to explore the long-term outcome and clinical characteristics of adult patients with juvenile onset in the EULAR Scleroderma Trials and Research (EUSTAR) cohort and compare them with adult patients with onset between 20 and 40 years of age. METHODS: From the EUSTAR SSc cohort two patient groups were analysed: patients with juvenile SSc (jSSc) who are adults at present, and patients diagnosed between the age of 20 and 40 years (aSSc). Demographic data of the patients, organ involvement and outcome of the disease were examined using the Minimal Essential Data Set database system. RESULTS: From 5000 patients in the EUSTAR cohort, 60 patients (1.2%) with jSSc and 910 patients (18%) with aSSc were selected according the inclusion criteria. In the jSSc group, the mean age of disease onset was 12.4 years (range 2-15.9 years), and in the aSSc group, the mean age was 32 years (range 20-40 years). Disease subsets were similar. The antibody profile was also comparable except for ACAs, which were positive in 5% of the jSSc group and 26.9% of the aSSc group (P<0.005). Organ involvement (lung, kidney, joint, muscle and heart) was similar in the two groups of patients at the time of the last follow-up. CONCLUSION: The subset distribution in the jSSc and aSSc cohorts was found to be similar. Only the frequency of ACAs was significantly lower in the jSSc, which supports the hypothesis that the SSc patients with paediatric onset in the adult cohort may represent a distinct subgroup of the complete cohort of paediatric patients.
Authors: Percival D Sampaio-Barros; Adriana B Bortoluzzo; Ana Paula T Del Rio; Ana Paula Luppino-Assad; Danieli Co Andrade; João Francisco Marques-Neto Journal: J Scleroderma Relat Disord Date: 2018-04-24
Authors: Brandi E Stevens; Kathryn S Torok; Suzanne C Li; Nicole Hershey; Megan Curran; Gloria C Higgins; Katharine F Moore; C Egla Rabinovich; Samuel Dodson; Anne M Stevens Journal: Arthritis Care Res (Hoboken) Date: 2018-11-08 Impact factor: 4.794
Authors: Anne M Stevens; Sami B Kanaan; Kathryn S Torok; Thomas A Medsger; Maureen D Mayes; John D Reveille; Marisa Klein-Gitelman; Ann M Reed; Tzielan Lee; Suzanne C Li; Gretchen Henstorf; Christine Luu; Tessa Aydelotte; J Lee Nelson Journal: Arthritis Rheumatol Date: 2016-10-06 Impact factor: 10.995
Authors: Anne M Stevens; Kathryn S Torok; Suzanne C Li; Sarah F Taber; Theresa T Lu; Francesco Zulian Journal: Front Immunol Date: 2019-06-25 Impact factor: 7.561
Authors: Marco A Alba; César Velasco; Carmen Pilar Simeón; Vicent Fonollosa; Luis Trapiella; María Victoria Egurbide; Luis Sáez; María Jesús Castillo; José Luis Callejas; María Teresa Camps; Carles Tolosa; Juan José Ríos; Mayka Freire; José Antonio Vargas; Gerard Espinosa Journal: Medicine (Baltimore) Date: 2014-03 Impact factor: 1.889