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Title: Disease characteristics of the Singapore systemic sclerosis cohort
Authors: Low, A.H.L. 
Teng, G.G. 
Law, W.G.
Ng, S.C.
Santosa, A.
Chan, G.
Lim, A. 
Png, M.E.
Teo, H.Q.
Lee, P.T.
Thumboo, J. 
Keywords: Asian
Disease characteristics
Systemic sclerosis
Issue Date: 2013
Citation: Low, A.H.L.,Teng, G.G.,Law, W.G.,Ng, S.C.,Santosa, A.,Chan, G.,Lim, A.,Png, M.E.,Teo, H.Q.,Lee, P.T.,Thumboo, J. (2013). Disease characteristics of the Singapore systemic sclerosis cohort. Proceedings of Singapore Healthcare 22 (1) : 8-14. ScholarBank@NUS Repository.
Abstract: Introduction: Systemic sclerosis (SSc) has one of the worst prognoses among autoimmune diseases. Multi-ethnic Western cohorts have shown ethnic differences in disease characteristics. Due to a paucity of data in Asia, we aimed to describe the disease characteristics and autoantibody profile of Asian SSc patients. Methods: The Singapore SSc database consists of prevalent and incident cases from four institutions. Data were recorded annually according to a standardised protocol. Cumulative disease characteristics were analysed using descriptive statistics. Results: From 2008 to 2012, 200 patients (85.5% females; Chinese 78%, Malays 10.5%, Indians 6%) diagnosed at a mean age of 46 years, fulfilled the American College of Rheumatology (ACR) (88.0%) or the Very Early Diagnosis of SSc (VEDOSS) (12%) criteria. SSc subtypes include limited cutaneous SSc (LcSSc) (32%) or diffuse cutaneous SSc (DcSSc) (37.6%), SSc-overlap syndromes (27.4%) and sine scleroderma (3%). Predominant disease manifestations were Raynaud's phenomenon (n=161, 80.5%), gastrointestinal (GI) (n=125, 62.5%), joint pain (n=116, 58%) and interstitial lung disease (ILD) (n=99, 51.8%). Pulmonary arterial hypertension (PAH) by echocardiography was present in 23.2% (39/168) of patients (mean systolic pulmonary arterial pressure 55.0+19.7mmHg). Renal crisis was rare (n=4, 2%). Anti-nuclear antibodies (ANA) tested positive in 78.7% (155/197), with anti-Scl-70 (69/197, 35.0%), anti-ribonucleoprotein (RNP) (56/192, 29.2%) and anti-Ro60 (40/193, 20.7%) most frequently detected. Anti-centromere antibodies were infrequent (23/190, 12.1%). Treatment included corticosteroids (80.7%), for peripheral vasculopathy (61.4%), GI (68%), PAH (11.2%) and immunosuppression (62.9%). Conclusion: Compared to Caucasian patients, our predominantly Chinese Asian SSc patients were diagnosed at an earlier age, with more severe PAH, less frequent Raynaud's phenomenon, an autoantibody profile characterised by anti-Scl70, anti-RNP and anti-Ro60, infrequently anti-centromere and more patients had DcSSc.
Source Title: Proceedings of Singapore Healthcare
ISSN: 20101058
Appears in Collections:Staff Publications

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