Session Information
Session Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s – Clinical Aspects and Therapeutics
Session Type: Abstract Submissions (ACR)
Background/Purpose: to identify risk factors for early mortality in a large group of recently diagnosed systemic sclerosis (SSc) patients
Methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS), on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres, in an annual basis. Patients with disease duration of less than 3 years at the first EUSTAR entry, and with at least one follow-up visit, were selected. Baseline data from the first visit were compared between SSc cases registered as dead up to December 2011, and living patients. Kaplan-Meier analysis was used to estimate survival, and Cox proportional hazards regression analysis, corrected by age at the end of follow-up, was used to identify factors associated with early mortality.
Results: From 1188 patients, 671 (19% men) had at least one follow-up visit. From those, 276 had diffuse and 348 had limited disease. Mean age at entry was 53±15 years, and at first non Raynaud symptom 50±15 years. Mean disease duration was 19±6 months and time between the onset of Raynaud and first non Raynaud symptom was 4±7 months. After 43±24 months of follow-up from the first visit and 57±26 months from the first non Raynaud symptom, 111 patients (17%) were dead. Death occurred after 43±27 months from the first non Raynaud symptom. Mean survival for the whole group was 116 (95%CI 110-122) months. By Cox proportional hazards regression multivariate analysis, main risk factors for mortality were: higher skin score (HR 1-03, 95%CI 1.003-1.05, p=0.02), acute phase reactants elevation (HR 1.8, 95%CI 1.1-2.8; p=0.02), joint contractures (HR 1.8; 95%CI 1.1-2.8; p=0.002), CK elevation (HR 1.9, 95%CI 1.1-3.3, p=0.02), cardiac blocks (HR 2.1, 95%CI 1.3-3.3, p=0.004), diastolic dysfunction (HR 2.1, 95%CI 1.3-3.3, p=0.002) and ischemic ulcers (HR 1.9, 95%CI 1.2-2.9, p=0.007). When only diffuse patients were analyzed, CK elevation, FVC below 80% and pulmonary hypertension were the risk factors for mortality. In the other hand, bad prognostic factors in limited patients were joint contractures, CK elevation, cardiac blocks, proteinuria and renal crisis.
Conclusion: In this large group of SSc patients, risk factors for early mortality are higher skin involvement with more severe vascular disease and cardiac involvement, especially if associated to elevated acute phase response. Muscular and cardiac involvement appear as a risk factor for mortality both in limited and diffuse disease, whereas lung and renal involvement have a mayor impact in diffuse and limited cases respectively.
Disclosure:
P. E. Carreira,
None;
L. Carmona,
None;
B. E. Joven,
None;
C. P. Denton,
None;
Y. Allanore,
None;
U. A. Walker,
None;
M. Matucci-Cerinic,
None;
U. Müller-Ladner,
None;
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