OBJECTIVES: This study aims to evaluate the demographic and clinical features, laboratory data, treatment modalities, and outcomes of juvenile systemic sclerosis (JSS) and juvenile localized scleroderma (JLS) patients at a referral pediatric rheumatology center in Turkey. PATIENTS AND METHODS: Medical records of a total of 57 patients, including 29 with JSS (1 male, 28 females; mean age 18.3±3.2 years; range 14 to 27 years) and 28 with JLS (6 males, 22 females; mean age 14.4±4.8 years; range 6 to 23 years), diagnosed betweenJanuary 2006 and Mart 2015 and followed-up for at least six months were evaluated in this retrospective longitudinal study. All medical records were retrospectively analyzed for demographic, clinical, and laboratory findings. RESULTS: Mean age at disease onset was 9.9±4.2 years and 7.7±3.9 years for JSS and JLS, respectively. Mean ages at diagnosis and at the time of study were lower in JLS: 9.1±3.5 years vs. 11.7±3.7 years and 14.4±4.8 years vs. 18.3±3.2 years, respectively. Mean disease duration was 7.8±5.2 years and 8.0±4.3 years for JSS and JLS, respectively. Among JSS patients, interstitial lung disease was seen in eight (27%), pulmonary hypertension in three (10%), and arrhythmia in one (3%). One JSS patient (3%) died as a consequence of cardiac sclerosis. Corticosteroids with methotrexate were used in 29 JSS patients (100%) and in 21 JLS patients (75%). Patients with vasculopathy were treated with nifedipine (n=18, 62%) and bosentan (n=12, 41%). Internal organ involvement was treated with high-dose cyclophosphamide (n=10, 34%) or biological agent (n=3, 10%). CONCLUSION: Close monitoring of internal organ involvement is of great importance in preventing disease-related complications in JSS and JLS. Although rare, vital organ involvement has a devastating effect on prognosis. Biological agents represent an option for patients resistant to standard immunosuppressive treatment.
OBJECTIVES: This study aims to evaluate the demographic and clinical features, laboratory data, treatment modalities, and outcomes of juvenile systemic sclerosis (JSS) and juvenile localized scleroderma (JLS) patients at a referral pediatric rheumatology center in Turkey. PATIENTS AND METHODS: Medical records of a total of 57 patients, including 29 with JSS (1 male, 28 females; mean age 18.3±3.2 years; range 14 to 27 years) and 28 with JLS (6 males, 22 females; mean age 14.4±4.8 years; range 6 to 23 years), diagnosed betweenJanuary 2006 and Mart 2015 and followed-up for at least six months were evaluated in this retrospective longitudinal study. All medical records were retrospectively analyzed for demographic, clinical, and laboratory findings. RESULTS: Mean age at disease onset was 9.9±4.2 years and 7.7±3.9 years for JSS and JLS, respectively. Mean ages at diagnosis and at the time of study were lower in JLS: 9.1±3.5 years vs. 11.7±3.7 years and 14.4±4.8 years vs. 18.3±3.2 years, respectively. Mean disease duration was 7.8±5.2 years and 8.0±4.3 years for JSS and JLS, respectively. Among JSS patients, interstitial lung disease was seen in eight (27%), pulmonary hypertension in three (10%), and arrhythmia in one (3%). One JSS patient (3%) died as a consequence of cardiac sclerosis. Corticosteroids with methotrexate were used in 29 JSS patients (100%) and in 21 JLS patients (75%). Patients with vasculopathy were treated with nifedipine (n=18, 62%) and bosentan (n=12, 41%). Internal organ involvement was treated with high-dose cyclophosphamide (n=10, 34%) or biological agent (n=3, 10%). CONCLUSION: Close monitoring of internal organ involvement is of great importance in preventing disease-related complications in JSS and JLS. Although rare, vital organ involvement has a devastating effect on prognosis. Biological agents represent an option for patients resistant to standard immunosuppressive treatment.
Authors: I Foeldvari; M Zhavania; N Birdi; R J Cuttica; S H de Oliveira; P B Dent; R Elborgh; F Falcini; G Ganser; H Girschick; R Häfner; R Joos; W Kuis; P Pelkonen; A M Prieur; K Rostropowicz-Denisiewicz; R Russo; A Savolainen; A Siamopoulou-Mayridou; F Zulian Journal: Rheumatology (Oxford) Date: 2000-05 Impact factor: 7.580
Authors: Giorgia Martini; Ivan Foeldvari; Ricardo Russo; Ruben Cuttica; Anne Eberhard; Angelo Ravelli; Thomas J A Lehman; Sheila Knupp Feitosa de Oliveira; Gordana Susic; Galina Lyskina; Dana Nemcova; Robert Sundel; Fernanda Falcini; Herman Girschick; Ana Paula Lotito; Antonella Buoncompagni; Flavio Sztajnbok; Sulaiman M Al-Mayouf; Ilonka Orbàn; Clodoveo Ferri; Balu H Athreya; Patricia Woo; Francesco Zulian Journal: Arthritis Rheum Date: 2006-12
Authors: Francesco Zulian; Patricia Woo; Balu H Athreya; Ronald M Laxer; Thomas A Medsger; Thomas J A Lehman; Marco Matucci Cerinic; Giorgia Martini; Angelo Ravelli; Ricardo Russo; Ruben Cuttica; Sheila Knupp Feitosa de Oliveira; Christopher P Denton; Franco Cozzi; Ivan Foeldvari; Nicolino Ruperto Journal: Arthritis Rheum Date: 2007-03-15
Authors: Kenneth Scalapino; Thaschawee Arkachaisri; Mary Lucas; Noreen Fertig; David J Helfrich; Aldo V Londino; Virginia D Steen; Thomas A Medsger Journal: J Rheumatol Date: 2006-04-01 Impact factor: 4.666
Authors: F Atzeni; A Bardoni; M Cutolo; N Hunzelmann; T Krieg; G Martini; C Montecucco; T M Olski; M E Secchi; G Valentini; F Zulian; P Sarzi-Puttini Journal: Clin Exp Rheumatol Date: 2006 Jan-Feb Impact factor: 4.473