RESULTS: Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29% ABSTRACT ObjectivesTo determine the causes and predictors of mortality in systemic sclerosis (SSc). Methods Patients with SSc (n=5860) fulfi lling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. Results Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fi brosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the nonSSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%).Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modifi ed Rodnan skin score (HR 1.20 per 10 score points). Conclusion Disease-related causes, in particular pulmonary fi brosis, PAH and cardiac causes, accounted for the majority of deaths in SSc.Systemic sclerosis (SSc) is a multisystem disease with vascular, infl ammatory and fi brotic components.

Combining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients' survival.

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Republication or reproduction of this report or its storage and/or dissemination by electronic means is permitted without the need for formal IUPAC permission on condition that an acknowledgmentAbstract: Fullerenes are a new allotrope of carbon characterized by a closed-cage structure consisting of an even number of three-coordinate carbon atoms devoid of hydrogen atoms. This class was originally limited to closed-cage structures with 12 isolated five-membered rings, the rest being six-membered rings.Although it was recognized that existing organic ring nomenclature could be used for these structures, the resulting names would be extremely unwieldy and inconvient for use. At the same time it was also recognized that established organic nomenclature principles could be used, or adapted, to provide a consistent nomenclature for this unique class of compounds based on the class name fullerene. However, it was necessary to develop an entirely new method for uniquely numbering closed-cage systems.This paper describes IUPAC recommendations for naming and uniquely numbering the two most common fullerenes with isolated pentagons, the icosahedral C 60 fullerene and a D 5h -C 70 fullerene. It also describes recommendations for adapting organic nomenclature principles for naming fullerenes with nonclosedcage structures, heterofullerenes, derivatives formed by substitution of hydrofullerenes, and the fusion of organic rings or ring systems to the fullerene cage. Finally, this paper suggests methods for describing structures consisting of two or more fullerene units and for denoting configurations of chiral fullerenes and their derivatives. CONTENTS

OBJECTIVES: To measure the prevalence of, and factors associated with, left ventricular (LV) dysfunction in systemic sclerosis (SSc). METHODS: The EUSTAR database was first searched. A casecontrol study of a patient subset was then performed to further identify independent factors associated with LV dysfunction by simple and multiple regression. RESULTS: Of 7073 patients, 383 (5.4%) had an LV ejection fraction (EF) of <55%. By multiple regression analysis, age, sex, diffuse cutaneous disease, disease duration, digital ulcerations, renal and muscle involvement, disease activity score, pulmonary fibrosis and pulmonary arterial hypertension were associated with LV dysfunction. In the second phase, 129 patients with SSc with LVEF <55% were compared with 256 patients with SSc with normal LVEF. Male sex (OR 3.48; 95% CI 1.74 to 6.98), age (OR 1.03; 95% CI 1.01 to 1.06), digital ulcerations (OR 1.91; 95% CI 1.05 to 3.50), myositis (OR 2.88; 95% CI 1.15 to 7.19) and use of calcium channel blockers (OR 0.41; 95% CI 0.22 to 0.74) were independent factors associated with LV dysfunction. CONCLUSION: The prevalence of LV dysfunction in SSc is 5.4%. Age, male gender, digital ulcerations, myositis and lung involvement are independently associated with an increased prevalence of LV dysfunction. Conversely, the use of calcium channel blockers may be protective. Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of systemic sclerosis patientsAllanore Y (1), Meune C (2), Vonk MC (3), Airo P (4), Hachulla E (5), Caramaschi P (6), Riemekasten G (7), Cozzi F(8), Beretta L (9), Derk CT (10) AbstractStudy objectives and methods To measure the prevalence of, and factors associated with, left ventricular (LV) dysfunction in SSc, we first queried the EUSTAR database. In a second phase, we performed a case-control study of a patient subset, to further identify independent factors associated with LV dysfunction by simple and multiple regression. Results Among 7,073 patients, 383 (5.4%) had a LV ejection fraction (EF) <55%. By multiple regression analysis, age, sex, diffuse cutaneous disease, disease duration, digital ulcerations, renal and muscle involvement, disease activity score, pulmonary fibrosis and pulmonary arterial hypertension (PAH) were associated with LV dysfunction. In a second phase, 129 SSc patients with LVEF <55% were compared with 256 SSc patients with normal LVEF. Male sex (OR 3.48; 95% CI1.74-6.98), age (OR 1.03; 95% CI 1.01-1.06), digital ulcerations (OR 1.91; 95% CI 1.05-3.50), myositis (OR 2.88; 95% CI 1.15-7.19), and calcium channel blockers (CCB) use (OR 0.41; 95% CI 0.22-0.74) were independent factors associated with LV dysfunction.

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