Systemic Sclerosis
Conditions
Keywords
systemic sclerosis, cohort, Korean
Brief summary
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.
Detailed description
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.
Interventions
OTHERThis is an observational prospective study.
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.
Sponsors
Seoul National University Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 99 Years
Healthy volunteers
No
Inclusion criteria
* SSc diagnosis according to 1980 or 2013 ACR classification criteria or 2001 criteria for the classification of early systemic sclerosis
Exclusion criteria
* When patients refuse to take part in the study
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Internal organ involvement | 2-year | Number of participants with internal organ involvement (Interstitial lung disease, Pulmonary arterial hypertension, Cardiac involvement, Gastrointestinal involvement, Renal involvement) |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Interstitial lung disease-progression | 2-year | Number of participants with progression of ILD from baseline by computed tomography of chest |
| Interstitial lung disease-Changes of FVC% from baseline | 2-year | Changes of FVC% from baseline per year =FVC% at 1 year (0-unlimited%) - FVC% at baseline (0-unlimited%) |
| Interstitial lung disease-Changes of DLCO% from baseline | 2-year | 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 |
| Pulmonary arterial hypertension by echocardiography | 2-year | Number of participants with pulmonary arterial hypertension (PAH) by electrocardiography -pulmonary arterial systolic pressure: 0-unlimited (mmHg) |
| Pulmonary arterial hypertension by right heart catheterization (optional) | 2-year | Number of participants with PAH confirmed by right heart catheterization mean pulmonary arterial pressure: 0-unlimited (mmHg) |
| Interstitial lung disease | 2-year | Number of participants with ILD (at baseline and and during follow up) by computed tomography of chest |
| Pericardial effusion wihtout other heart disease by echocardiography | 2-year | Pericardial effusion: mild, moderated, severe with hemodynamic unstability |
| Arrhythmia on electrocardiogram | 2-year | Number of participants with arrhythmia on electrocardiogram |
| University of California Los Angeles Scleroderma Clinical Trials Consortium Gastrointestinal Scale (UCLA SCTC GIT) 2.0 | 2-year | 0-3 scale (0: no GI problems to 3: most severe) |
| Scleroderma-Specific Health Assessment Questionnaire (SHAQ) | 2-year | 0-3 (where 0 = without difficulty and 3 = unable to do) |
| Short Form(SF)-36 Health Survey | 2-year | 0-100 scale (a score of zero is equivalent to maximum disability and a score of 100 is equivalent to no disability) |
| Abnormal cardiac function without other heart disease by echocardiography | 2-year | Ejection fraction: 0-unlimited% |
Countries
South Korea
Outcome results
None listed