Clinical Characteristics and Mortality in Systemic Sclerosis: A Comparison between Early- and Late-Referred Diseases

Chingching Foocharoen MD*, Ajanee Mahakkanukrauh MD*, Siraphop Suwannaroj MD*, Ratanavadee Nanagara MD*

Affiliation : * Department of Medicine, Khon Kaen University, Khon Kaen, Thailand

Objective : To determine if there is any significant difference in the clinical characteristics and mortality between early-and late-referred systemic sclerosis (SSc). Material and Method: An historical cohort study was performed among referred-SSc patients at Srinagarind Hospital between January 2006 and December 2010. ‘Early referrals’ occurred during the edematous phase while ‘late referrals’ occurred after that.
Results : Forty two percent of the SSc cases (229 of 543) were referred; 108 (47.2%) were early-referrals. Early referrals were for proper management (49.1%) and diagnosis (41.7%), whereas the majority of late referrals (79.3%) were for proper management, followed by additional investigations (10.7%). The respective median duration of disease at referral between early and late was 3.7 (IQR 2.6-5.6) and 20.7 months (IQR 12.2-37.4). Joint contracture, cardiac involvement and pulmonary fibrosis presented more frequently among late-referrals (p<0.001, p = 0.03 and p = 0.04, respectively). The respective mortality rate among early- vs. late-referrals was 15.1 (95% CI 10.0-21.8) vs. 23.0 (95% CI 15.8-32.3) per 100 person-year. Two-thirds of deaths were associated with the disease, pulmonary fibrosis being most common among both early- and late- referrals (50 and 42.7%, respectively).
Conclusion : The number of early vs. late referrals was comparable and cardiopulmonary involvement and joint contracture were common presentations in late-referrals. Late-referral was associated with high mortality commonly from pulmonary fibrosis.

Keywords : Systemic sclerosis (SSc), Scleroderma, Refer, Mortality, Autoimmune disease

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