OBJECTIVE: Systemic sclerosis (SSc) is associated with a reduction in life expectancy, but there are no validated prognostic models for determining short-term mortality. The objective of this study was to derive and validate a prediction rule for 2-year mortality in patients with early diffuse cutaneous SSc (dcSSc). METHODS: We studied a prospectively enrolled cohort of 387 US Caucasian patients with early dcSSc (<2 years from the appearance of the first symptom), randomly divided into a derivation cohort (n = 260) and a validation cohort (n = 127). Predefined baseline predictor variables were analyzed in a stepwise multivariable logistic regression model in order to identify factors independently associated with 2-year all-cause mortality using a cutoff of P < 0.05. We rounded the beta values to the nearest integer and summed the points assigned to each variable in order to stratify patients into low-risk, moderate-risk, and high-risk groups. We then applied this rule to an external validation cohort of 110 Caucasian patients with early dcSSc from a single UK center and compared stratum-specific mortality using chi-square statistics. RESULTS: Four independent predictor variables (with assigned integer values) comprised the model: age at first visit (points allotted: -1, 0, or 1), skin thickness progression rate (points allotted: 0 or 1), gastrointestinal tract severity (points allotted: 0, 1, or 2), and anemia (points allotted: 0 or 2). The prediction model performed well, with no significant differences between the derivation cohort and the US or UK validation cohorts in the low-risk and moderate-risk groups. CONCLUSION: We derived a 4-variable prediction rule that can be used to stratify patients with early dcSSc into groups by risk of 2-year mortality, and we validated that prediction rule in US and UK cohorts.
OBJECTIVE:Systemic sclerosis (SSc) is associated with a reduction in life expectancy, but there are no validated prognostic models for determining short-term mortality. The objective of this study was to derive and validate a prediction rule for 2-year mortality in patients with early diffuse cutaneous SSc (dcSSc). METHODS: We studied a prospectively enrolled cohort of 387 US Caucasian patients with early dcSSc (<2 years from the appearance of the first symptom), randomly divided into a derivation cohort (n = 260) and a validation cohort (n = 127). Predefined baseline predictor variables were analyzed in a stepwise multivariable logistic regression model in order to identify factors independently associated with 2-year all-cause mortality using a cutoff of P < 0.05. We rounded the beta values to the nearest integer and summed the points assigned to each variable in order to stratify patients into low-risk, moderate-risk, and high-risk groups. We then applied this rule to an external validation cohort of 110 Caucasian patients with early dcSSc from a single UK center and compared stratum-specific mortality using chi-square statistics. RESULTS: Four independent predictor variables (with assigned integer values) comprised the model: age at first visit (points allotted: -1, 0, or 1), skin thickness progression rate (points allotted: 0 or 1), gastrointestinal tract severity (points allotted: 0, 1, or 2), and anemia (points allotted: 0 or 2). The prediction model performed well, with no significant differences between the derivation cohort and the US or UK validation cohorts in the low-risk and moderate-risk groups. CONCLUSION: We derived a 4-variable prediction rule that can be used to stratify patients with early dcSSc into groups by risk of 2-year mortality, and we validated that prediction rule in US and UK cohorts.
Authors: K Nishioka; I Katayama; H Kondo; H Shinkai; H Ueki; K Tamaki; K Takehara; S Tajima; M Maeda; S Hayashi; H Kodama; Y Miyachi; H Mizutani; A Fujisaku; T Sasaki; M Shimizu; J Kaburagi Journal: J Dermatol Date: 1996-10 Impact factor: 4.005
Authors: T J Laing; B W Gillespie; M B Toth; M D Mayes; R H Gallavan; C J Burns; J R Johanns; B C Cooper; B J Keroack; M C Wasko; J V Lacey; D Schottenfeld Journal: Arthritis Rheum Date: 1997-04
Authors: P J Clements; P A Lachenbruch; J R Seibold; B Zee; V D Steen; P Brennan; A J Silman; N Allegar; J Varga; M Massa Journal: J Rheumatol Date: 1993-11 Impact factor: 4.666
Authors: Dustin A Carlson; Jacqueline E Prescott; Emma Germond; Darren Brenner; Mary Carns; Chase S Correia; Marie-Pier Tetreault; Zsuzsanna H McMahan; Monique Hinchcliff; Wenjun Kou; Peter J Kahrilas; Harris R Perlman; John E Pandolfino Journal: Neurogastroenterol Motil Date: 2021-10-28 Impact factor: 3.960
Authors: Robyn T Domsic; Shiyao Gao; Maureen Laffoon; Steven Wisniewski; Yuqing Zhang; Virginia Steen; Robert Lafyatis; Thomas A Medsger Journal: Rheumatology (Oxford) Date: 2021-10-02 Impact factor: 7.580
Authors: Jessica Meijs; Anne A Schouffoer; Nina Ajmone Marsan; Theo Stijnen; Hein Putter; Maarten K Ninaber; Tom W J Huizinga; Jeska K de Vries-Bouwstra Journal: RMD Open Date: 2015-12-01