Table 1.
Definitions of study outcomes.
Fig 1.
Flow chart of patients included and excluded in the analysis.
Table 2.
Comparison of disease characteristics at the baseline visit between patients included in this analysis (visit within 1 year after onset of RP, study population) and those excluded (no visit within 1 year after onset of RP).
ACA, anti-centromere autoantibodies; ANA, anti-nuclear autoantibodies; anti-RNAP-III, anti-RNA-polymerase-III autoantibodies; anti-TOPO, anti-topoisomerase-I autoantibodies; DLCO, single breath diffusing capacity for monoxide; FVC, forced vital capacity; IQR, interquartile range; mRSS, modified Rodnan skin score; PAPsys, systolic pulmonary artery pressure as estimated by echocardiography; RP, Raynaud's phenomenon; SD, standard deviation.
Fig 2.
Kaplan-Meier curves with 95% CI of the manifestation of any first non-RP feature after RP onset in all SSc patients in the entire EUSTAR cohort (A) and stratified by sex (B), the median age at RP onset (C) and the autoantibody status (D). CI, confidence interval; RP, Raynaud’s phenomenon; Pts, patients; ACA, anti-centromere autoantibodies; anti-TOPO, anti-topoisomerase-I autoantibodies; anti-RNAP-III, anti-RNA-polymerase-III autoantibodies; hash marks represent censored observations.
Fig 3.
Kaplan-Meier curves of incident organ involvement in SSc patients of the study population after RP onset.
RP, Raynaud’s phenomenon; Pts, patients; DLCO, single breath diffusing capacity for monoxide; GI symptoms, gastrointestinal symptoms, defined as a history of either dysphagia, reflux, early satiety, vomiting, diarrhoea, bloating or constipation; Skin involvement, defined as a modified Rodnan skin score of ≥2 at any part of the body; Cardiac involvement, defined as either the presence of diastolic dysfunction, conduction blocks, a left ventricular ejection fraction (LVEF) < 50%, or a pericardial effusion; FVC, forced vital capacity; PAPsys, systolic pulmonary artery pressure as estimated by echocardiography.
Fig 4.
Kaplan-Meier curves with 95% CI of incident pulmonary restriction (FVC<80% of predicted; (A-D)), suspected pulmonary hypertension (PAPsys>40 mmHg; (E-H)), cardiac involvement (I-L) and renal crisis (M-P) after RP onset in SSc patients of the study population; stratified by sex (A/E/I/M), the median age at RP onset (B/F/J/N), autoantibody status (C/G/K/O) and extent of skin involvement within the first year after RP onset (D/H/L/P). CI, confidence interval; FVC, forced vital capacity; PAPsys, systolic pulmonary artery pressure as estimated by echocardiography; cardiac involvement, defined as either the presence of diastolic dysfunction, conduction blocks, a left ventricular ejection fraction (LVEF)<50%, or a pericardial effusion; RP, Raynaud’s phenomenon; yrs, years; ACA, anti-centromere autoantibodies; Anti-TOPO, Anti-topoisomerase-I autoantibodies; Anti-RNAP-III, anti-RNA-polymerase-III autoantibodies; hash marks represent censored observations.
Table 3.
Cox multivariable regression analysis of risk factors for the time to incident FVC<80% of predicted, PAPsys>40 mmHg, any cardiac dysfunction, diastolic dysfunction, conduction block, pericardial effusion and renal crisis.
ACA, anti-centromere autoantibodies; Anti-RNAP-III, anti-RNA-polymerase-III autoantibodies; Anti-TOPO, anti-topoisomerase-I autoantibodies; cardiac involvement, defined as either the presence of diastolic dysfunction, conduction blocks, left ventricular ejection fraction (LVEF)<50%, or pericardial effusion; CI, confidence interval; FVC, forced vital capacity; HR, hazard ratio; PAPsys, systolic pulmonary artery pressure as estimated by echocardiography; RP, Raynaud's phenomenon.
Fig 5.
Kaplan-Meier curves of incident cardiac manifestations after RP onset in SSc in the study population.
RP, Raynaud’s phenomenon; Pts, patients; LVEF, left ventricular ejection fraction; hash marks represent censored observations.