Seoul National University Hospital Systemic Sclerosis Cohort

Systemic Sclerosis Cohort in Seoul National University Hospital Single Center Prospective Cohort, of Patients With Systemic Sclerosis in Korea

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.

Study Overview

• Status: Unknown
• Conditions: Systemic Sclerosis
• Intervention / Treatment: Other: This is an observational prospective study.

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.

• Study Type: Observational
• Enrollment (Anticipated): 150

Contacts and Locations

Study Locations

• Korea, Republic of
  • Seoul, Korea, Republic of, 110-744
    • Seoul National University Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 99 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

SSc diagnosis according to 1980 or 2013 ACR classification criteria or 2001 criteria for the classification of early systemic sclerosis

Description

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

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Internal organ involvement	Number of participants with internal organ involvement (Interstitial lung disease, Pulmonary arterial hypertension, Cardiac involvement, Gastrointestinal involvement, Renal involvement)	2-year

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Interstitial lung disease	Number of participants with ILD (at baseline and and during follow up) by computed tomography of chest	2-year
Interstitial lung disease-progression	Number of participants with progression of ILD from baseline by computed tomography of chest	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%)	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	2-year
Pulmonary arterial hypertension by echocardiography	Number of participants with pulmonary arterial hypertension (PAH) by electrocardiography -pulmonary arterial systolic pressure: 0-unlimited (mmHg)	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)	2-year
Abnormal cardiac function without other heart disease by echocardiography	Ejection fraction: 0-unlimited%	2-year
Pericardial effusion wihtout other heart disease by echocardiography	Pericardial effusion: mild, moderated, severe with hemodynamic unstability	2-year
Arrhythmia on electrocardiogram	Number of participants with arrhythmia on electrocardiogram	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)	2-year
Scleroderma-Specific Health Assessment Questionnaire (SHAQ)	0-3 (where 0 = without difficulty and 3 = unable to do)	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)	2-year

Collaborators and Investigators

• Sponsor: Seoul National University Hospital
• Investigators: Principal Investigator: Eun Bong Lee, MD, PhD, Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea

Publications and helpful links

General Publications

• Gabrielli A, Avvedimento EV, Krieg T. Scleroderma. N Engl J Med. 2009 May 7;360(19):1989-2003. doi: 10.1056/NEJMra0806188. No abstract available.
• Galluccio F, Walker UA, Nihtyanova S, Moinzadeh P, Hunzelmann N, Krieg T, Steen V, Baron M, Sampaio-Barros P, Kayser C, Nash P, Denton CP, Tyndall A, Muller-Ladner U, Matucci-Cerinic M. Registries in systemic sclerosis: a worldwide experience. Rheumatology (Oxford). 2011 Jan;50(1):60-8. doi: 10.1093/rheumatology/keq355.

Study record dates

Study Major Dates

• Study Start: March 1, 2016
• Primary Completion (Anticipated): June 1, 2018
• Study Completion (Anticipated): June 1, 2018

Study Registration Dates

• First Submitted: May 13, 2016
• First Submitted That Met QC Criteria: June 28, 2016
• First Posted (Estimate): June 30, 2016

Study Record Updates

• Last Update Posted (Estimate): June 30, 2016
• Last Update Submitted That Met QC Criteria: June 28, 2016
• Last Verified: June 1, 2016

More Information

Terms related to this study

• Keywords: cohort; systemic sclerosis; Korean
• Additional Relevant MeSH Terms: Pathologic Processes; Skin Diseases; Connective Tissue Diseases; Sclerosis; Scleroderma, Systemic; Scleroderma, Diffuse
• Other Study ID Numbers: 2520140100

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO