Abstract:Juvenile systemic sclerosis is an orphan disease of the macro- and microvasculature with an unknown etiology. Although pediatric and adult classification systems have been developed, neither has been validated yet. Recent publications have shown a shift in the subset pattern in juvenile-onset adult patients, with a better outcome in these patients as compared with young adult patients. As a first step in assessing activity, damage, and severity, a pediatric severity score--a modification of the adult Medsger s… Show more
“…Juvenile systemic sclerosis (scleroderma; SSc) is a rare connective tissue disease with an estimated incidence of <1 per million children that carries significant morbidity and mortality . Progressive multisystem organ involvement can occur in juvenile SSc, including respiratory and gastrointestinal fibrosis leading to >10% mortality within the first 5 years of diagnosis .We describe the relationship between esophageal abnormalities on imaging and pulmonary function tests in children with juvenile systemic sclerosis (SSc).Abnormal esophageal peristalsis and/or bolus clearance on upper gastrointestinal (UGI) tests were associated with signs of decreased pulmonary function (decreased forced vital capacity, forced expiratory volume in 1 second, and vital capacity) and increased esophageal diameter on high‐resolution computed tomography (HRCT).Abnormal esophageal findings were not associated with disease duration, frequency of symptoms, or autoantibodies.Neither gastrointestinal nor pulmonary symptoms predicted organ involvement as identified by UGI tests or HRCT.…”
Objective. Juvenile systemic sclerosis (SSc) is a disabling autoimmune condition that affects multiple organs in addition to skin, notably the gastrointestinal and pulmonary systems. The relationship between esophageal abnormalities and pulmonary disease in juvenile SSc is not well understood. We describe associations between radiologic esophageal abnormalities and pulmonary function.Methods. Clinical and radiographic data of children ages >18 years who fulfilled the 2007 Pediatric Rheumatology Provisional Classification Criteria for juvenile SSc between 1994 and 2016 were reviewed. Fluoroscopic upper gastrointestinal (UGI) studies, high-resolution computed tomography (HRCT), and pulmonary function tests (PFTs) within 12 months of presentation to Seattle Children's Hospital were extracted.Results. Twenty-one children with juvenile SSc (67% female, ages 8-17 years) were studied. Esophageal abnormalities, defined as abnormal esophageal peristalsis and/or bolus clearance, were found in 12 patients. Abnormal esophagus on UGI tests was not associated with gastrointestinal or pulmonary symptoms, disease duration, use of medications (proton pump inhibitor or immunosuppressant), or specific autoantibodies. Compared with patients with a normal esophagus on UGI tests, children with an abnormal esophagus had decreased PFTs: mean forced expiratory volume in 1 second 96% versus 78% (P = 0.03), forced vital capacity 94% versus 76% (P = 0.02), and vital capacity 95% versus 76% (P = 0.02). Children with an abnormal esophagus on UGI tests had a larger mean esophageal diameter on HRCT (14.6 mm compared to 8.5 mm; P < 0.01).Conclusion. There was an association between esophageal and pulmonary disease in children with juvenile SSc. Esophageal findings on UGI tests or HRCT, despite lack of symptoms, should raise concern for esophageal dysfunction and prompt heightened surveillance for concurrent lung disease.
“…Juvenile systemic sclerosis (scleroderma; SSc) is a rare connective tissue disease with an estimated incidence of <1 per million children that carries significant morbidity and mortality . Progressive multisystem organ involvement can occur in juvenile SSc, including respiratory and gastrointestinal fibrosis leading to >10% mortality within the first 5 years of diagnosis .We describe the relationship between esophageal abnormalities on imaging and pulmonary function tests in children with juvenile systemic sclerosis (SSc).Abnormal esophageal peristalsis and/or bolus clearance on upper gastrointestinal (UGI) tests were associated with signs of decreased pulmonary function (decreased forced vital capacity, forced expiratory volume in 1 second, and vital capacity) and increased esophageal diameter on high‐resolution computed tomography (HRCT).Abnormal esophageal findings were not associated with disease duration, frequency of symptoms, or autoantibodies.Neither gastrointestinal nor pulmonary symptoms predicted organ involvement as identified by UGI tests or HRCT.…”
Objective. Juvenile systemic sclerosis (SSc) is a disabling autoimmune condition that affects multiple organs in addition to skin, notably the gastrointestinal and pulmonary systems. The relationship between esophageal abnormalities and pulmonary disease in juvenile SSc is not well understood. We describe associations between radiologic esophageal abnormalities and pulmonary function.Methods. Clinical and radiographic data of children ages >18 years who fulfilled the 2007 Pediatric Rheumatology Provisional Classification Criteria for juvenile SSc between 1994 and 2016 were reviewed. Fluoroscopic upper gastrointestinal (UGI) studies, high-resolution computed tomography (HRCT), and pulmonary function tests (PFTs) within 12 months of presentation to Seattle Children's Hospital were extracted.Results. Twenty-one children with juvenile SSc (67% female, ages 8-17 years) were studied. Esophageal abnormalities, defined as abnormal esophageal peristalsis and/or bolus clearance, were found in 12 patients. Abnormal esophagus on UGI tests was not associated with gastrointestinal or pulmonary symptoms, disease duration, use of medications (proton pump inhibitor or immunosuppressant), or specific autoantibodies. Compared with patients with a normal esophagus on UGI tests, children with an abnormal esophagus had decreased PFTs: mean forced expiratory volume in 1 second 96% versus 78% (P = 0.03), forced vital capacity 94% versus 76% (P = 0.02), and vital capacity 95% versus 76% (P = 0.02). Children with an abnormal esophagus on UGI tests had a larger mean esophageal diameter on HRCT (14.6 mm compared to 8.5 mm; P < 0.01).Conclusion. There was an association between esophageal and pulmonary disease in children with juvenile SSc. Esophageal findings on UGI tests or HRCT, despite lack of symptoms, should raise concern for esophageal dysfunction and prompt heightened surveillance for concurrent lung disease.
“…Juvenile scleroderma (JS) is a rare connective tissue disease affecting multiple organs [1]. The published data on JS are sparse [2].…”
Section: Introductionmentioning
confidence: 99%
“…JLS is the most frequent form of scleroderma in childhood [3]. It is estimated that 10% of all patients with scleroderma develop the disease before the age of 8 years, with the mean age of onset at 8.8 years [1,3].…”
Juvenile scleroderma (JS) represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests), Forced vital capacity (FVC) and Carbon monoxide diffusion capacity (DLCO) in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP) and risk factors for pulmonary arterial hypertension (PAH) were made by the measurement of maximum tricuspid insufficiency (TI), end-diastolic pulmonary insufficiency (PI), ratio of acceleration time (AT) to ejection time (ET) (AT/ET), right atrial pressure (RAP) and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years). The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years) and 3.57 years (median 2 years, range 0.5-14.5 years), respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.
“…The disease is considered to be benign but it may cause significant functional and cosmetic deformity. 2,3,5,7,8 Studies on localized scleroderma among adults and children generally report marked diagnostic delay and present a diverse range of treatment modalities. 7,8 Juvenile systemic sclerosis (JSS) is a quite rare condition characterized with multisystemic involvement and poorer prognosis.…”
mentioning
confidence: 99%
“…7,8 Juvenile systemic sclerosis (JSS) is a quite rare condition characterized with multisystemic involvement and poorer prognosis. [1][2][3][4] Many data regarding disease features and treatment options in JS are based on studies among adults. comparison with adults, with the exception of an ongoing multicentric, multinational study among children with systemic sclerosis.…”
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.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
