To establish a new prospective cohort of Korean patients with systemic sclerosis and track the natural history of the disease over time. To generate new hypotheses for further investigation.
Systemic sclerosis (SSc) is a multisystem and heterogeneous disease characterized by an unpredictable course and high morbidity and mortality. The complexity of SSc requires interdisciplinary diagnostic and therapeutic management and result in a growing burden for all health-care systems. For this reasons, researchers are seeking new diagnostic and therapeutic strategies to improve management of these patients. In order to improve clinical care and to develop recommendations for the diagnosis and treatment of SSc, disease registries are used to capture and track key patient information. Therefore, SSc research associations and consortiums play a pivotal role to perform ongoing research and data collection to understanding the disease and support research projects. Currently, several national SSc registries in the UK, Germany, USA, Canada, Brazil, Australia and international registry, EUSTAR (European League Against Rheumatism scleroderma trial and research), have been established. However, the natural history of Asian populations are fully evaluated. Therefore, investigators decided to establish SSc cohort in Korean patients.
Study Type
OBSERVATIONAL
Enrollment
150
Investigators will get clinical information including gender, date of birth, height, weight, family history for systemic sclerosis, education, and symptoms of organ involvement of skin, lung, heart, gastrointestinal tract, kidney, musculoskeletal system and laboratory data. Investigators will evaluate nailbed capillary microscope, hand x-ray, 6 minute walk test, echocardiography, computed tomography of chest, pulmonary function test (FVC%, DLCO%), and electrocardiogram as baseline. Investigators permit replacement of previous results within one year from baseline. Participants in the cohort will be followed up annually and investigators will treat SSc patients according to their routine practice in the best interest of patents. Medications for other comorbid conditions are allowed.
Seoul National University Hospital
Seoul, Seoul, South Korea
Internal organ involvement
Number of participants with internal organ involvement (Interstitial lung disease, Pulmonary arterial hypertension, Cardiac involvement, Gastrointestinal involvement, Renal involvement)
Time frame: 2-year
Interstitial lung disease
Number of participants with ILD (at baseline and and during follow up) by computed tomography of chest
Time frame: 2-year
Interstitial lung disease-progression
Number of participants with progression of ILD from baseline by computed tomography of chest
Time frame: 2-year
Interstitial lung disease-Changes of FVC% from baseline
Changes of FVC% from baseline per year =FVC% at 1 year (0-unlimited%) - FVC% at baseline (0-unlimited%)
Time frame: 2-year
Interstitial lung disease-Changes of DLCO% from baseline
Changes of DLCO% from baseline per year =DLCO% at 1year (0-unlimited%) - DLCO% at baseline (0-unlimited%) * Negative value means improvement from baseline * Positive value means deterioration from baseline
Time frame: 2-year
Pulmonary arterial hypertension by echocardiography
Number of participants with pulmonary arterial hypertension (PAH) by electrocardiography -pulmonary arterial systolic pressure: 0-unlimited (mmHg)
Time frame: 2-year
Pulmonary arterial hypertension by right heart catheterization (optional)
Number of participants with PAH confirmed by right heart catheterization mean pulmonary arterial pressure: 0-unlimited (mmHg)
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Time frame: 2-year
Abnormal cardiac function without other heart disease by echocardiography
Ejection fraction: 0-unlimited%
Time frame: 2-year
Pericardial effusion wihtout other heart disease by echocardiography
Pericardial effusion: mild, moderated, severe with hemodynamic unstability
Time frame: 2-year
Arrhythmia on electrocardiogram
Number of participants with arrhythmia on electrocardiogram
Time frame: 2-year
University of California Los Angeles Scleroderma Clinical Trials Consortium Gastrointestinal Scale (UCLA SCTC GIT) 2.0
0-3 scale (0: no GI problems to 3: most severe)
Time frame: 2-year
Scleroderma-Specific Health Assessment Questionnaire (SHAQ)
0-3 (where 0 = without difficulty and 3 = unable to do)
Time frame: 2-year
Short Form(SF)-36 Health Survey
0-100 scale (a score of zero is equivalent to maximum disability and a score of 100 is equivalent to no disability)
Time frame: 2-year