TY  - JOUR
IS  - 4
AV  - public
N2  - OBJECTIVE: Although diffuse cutaneous systemic sclerosis (dcSSc) is associated with a reduction in life expectancy, there are no validated prognostic models for determining 5-year mortality in patients with dcSSc. The objective of this study was to derive and validate a rule for predicting 5-year mortality in patients with early dcSSc. METHODS: We studied an inception cohort of 388 US Caucasian patients with early dcSSc (<2 years from the appearance of the first symptom). Predefined baseline variables were analyzed in a stepwise logistic regression model in order to identify factors independently associated with 5-year all-cause mortality. We rounded the beta weights to the nearest integer and summed the points assigned to each variable in order to stratify patients into low-risk (<0 points), moderate-risk (1-2 points), and high-risk (?3 points) groups. We then applied this rule to an external validation cohort of 144 Caucasian patients with early dcSSc from the Royal Free Hospital cohort and compared stratum-specific 5-year mortality. RESULTS: Six independent predictors (rounded beta weight) comprised the model: age at first visit (points allotted: -1, 0, or 1), male sex (points allotted: 0 or 1), tendon friction rubs (points allotted: 0 or 1), gastrointestinal involvement (points allotted: 0 or 1), RNA polymerase III antibodies (points allotted: 0 or 1), and anemia (points allotted: 0 or 1). The 3-level risk stratification model performed well, with no significant differences between the US derivation cohort and the UK validation cohort. CONCLUSION: We derived and externally validated, in US and UK cohorts, an easy-to-use 6-variable prediction rule that assigns low-risk, moderate-risk, and high-risk categories for 5-year mortality in patients with early dcSSc. Only history, physical examination, and basic laboratory assessments are required.
SN  - 2326-5205
A1  - Domsic, RT
A1  - Nihtyanova, SI
A1  - Wisniewski, SR
A1  - Fine, MJ
A1  - Lucas, M
A1  - Kwoh, CK
A1  - Denton, CP
A1  - Medsger, TA
EP  - 1003
SP  - 993
VL  - 68
JF  - Arthritis & Rheumatology
Y1  - 2016/04//
UR  - http://dx.doi.org/10.1002/art.39490
ID  - discovery1478961
N1  - This is the peer reviewed version of the following article: Domsic, RT; Nihtyanova, SI; Wisniewski, SR; Fine, MJ; Lucas, M; Kwoh, CK; Denton, CP; (2016) Derivation and External Validation of a Prediction Rule for Five-Year Mortality in Patients With Early Diffuse Cutaneous Systemic Sclerosis. Arthritis & Rheumatology, 68 (4) pp. 993-1003, which has been published in final form at: http://dx.doi.org/10.1002/art.39490. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Self-Archiving
TI  - Derivation and External Validation of a Prediction Rule for Five-Year Mortality in Patients With Early Diffuse Cutaneous Systemic Sclerosis
ER  -