Clinical characteristics and mortality in systemic sclerosis: a comparison between early- and late-referred diseases.

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.