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ObjectiveTo identify a core set of preliminary items considered as important for the very early diagnosis of systemic sclerosis (SSc).MethodsA list of items provided by European League Against Rheumatism (EULAR) Scleroderma Trial and Research(EUSTAR) centres were subjected to a Delphi exercise among 110 experts in the field of SSc. In round 1, experts were asked to choose the items they considered as the most important for the very early diagnosis of SSc. In round 2, experts were asked to reconsider the items accepted after the first stage. In round 3, the clinical relevance of selected items and their importance as measures that would lead to an early referral process were rated using appropriateness scores.ResultsPhysicians from 85 EUSTAR centres participated in the study and provided an initial list of 121 items. After three Delphi rounds, the steering committee, with input from external experts, collapsed the 121 items into three domains containing seven items, developed as follows: skin domain (puffy fingers/puffy swollen digits turning into sclerodactily); vascular domain (Raynaud's phenomenon, abnormal capillaroscopy with scleroderma pattern) and laboratory domain (antinuclear, anticentromere and antitopoisomerase-I antibodies). Finally, the whole assembly of EUSTAR centres ratified with a majority vote the results in a final face-to-face meeting.ConclusionThe three Delphi rounds allowed us to identify the items considered by experts as necessary for the very early diagnosis of SSc. The validation of these items to establish diagnostic criteria is currently ongoing in a prospective observational cohort.

In this study, we investigate the possibility of detecting, by dobutamine stress echocardiography (DSE), the presence of early or subclinical myocardial functional changes in patients with systemic sclerosis (SSc) without symptoms of ischaemic cardiac involvement.

Systemic sclerosis (SSc) is known to be characterised by a diffuse microvascular pathological process leading to cutaneous and visceral changes and to related clinical manifestations. Both necropsy studies1,2 and in vivo investigations3–5 have shown that in a number of patients with SSc there is evidence of a coronary microvascular disease, while coronary artery disease does not exceed that seen in a control group. In particular, myocardial perfusion defects on thallium-201 scintigraphy usually occur in the absence of angiographic evidence of coronary stenosis.3 Recently, we used a new and non-invasive method of contrast enhanced, transthoracic, second harmonic echo Doppler in patients with SSc to evaluate the coronary flow reserve (CFR), a functional variable measuring the ability of the coronary microvasculature to adapt its lumen to a vasodilating stimulus.6 We detected a significant reduction of the CFR …

Antiphospholipid antibodies (aPL) are detected in a variety of autoimmune disorders, most commonly systemic lupus erythematosus, but also in some infectious diseases, lymphoproliferative disorders, and even in apparently healthy people. Although a wide prevalence of aPL in systemic sclerosis has been reported (between 0 and 41%), most studies have focused on anticardiolipin antibodies (aCL) and very little is known about other aPL in this disease. We determined the prevalence and clinical significance of aCL, antibodies to β2-glycoprotein I (anti-β2GPI), and antibodies to phosphatidylserine-prothrombin complex (aPS-PT) in 25 patients with scleroderma (18 with limited and 7 with diffuse scleroderma, as defined by LeRoy et al 1) (table 1). Twenty four patients were female (median age 50 years (range 28–70), median disease duration 3 years (range 1–20)). …

Background Over the past decade, several advances in the management of Systemic Sclerosis (SSc) have occurred that may affect hospitalization patterns 1 . Major causes of hospitalization for SSc patients are periodic intravenous treatment, management of typical manifestations and complications or coexisting medical conditions. Objectives The aim of the study was to quantify the burden of SSc in Sardinia, an autonomous region of Italy with about 1.600.000 residents, with particular emphasis on the access to health services, through a population-based study analyzing hospitalization data in the decade 2001-2010. Methods The study was performed on data regarding the hospital discharge records (SDO) with a diagnosis of SSc, identified by the ICD-9-CM code (710.1), in the field of both principal (defined as the condition chiefly responsible for the hospitalization) and secondary diagnosis, The SDO data were obtained from the Department of Hygiene and Health of the “Regione Autonoma della Sardegna”. The abstracted data allowed to be aware of the number of patients hospitalized but not repeat hospitalizations of individuals to be known. All the hospitalizations of SSc patients have been analyzed according to sex, age (0-16 yrs; 17-24 yrs; 25-44 yrs; 45-64 yrs; >65 yrs), types of admission to hospital (in-patient and day-hospital), length of stay or number of accesses. Results The study included 4.144 hospitalizations in 624 patients. SSc was the principal diagnosis in 2.916 hospitalization (70.4%). The 49.7% of hospitalization occurred in patients aged 45-64 years and involved women in 84.7% of admissions. During the study period an increasing trend in the number of hospitalizations with SSc as the principal discharge diagnosis, has been observed (from 148 in 2001 to 427 in 2010), whileit remained stable for those with secondary diagnosis. The overall mean length of stay for in-patient hospitalizations were 8.3 days (standard deviation ± 5.7; median 7 days) and 8.5 days (standard deviation ± 9.4; median 6 days) for SSc as a principal and secondary discharge diagnosis, respectively. After stratification for the type of admission the 68% of hospitalizations resulted as in-patient care and the 32% as day-hospital (a programmed one day hospitalization). The in-patient/day-hospital ratio decreased from 3.5:1 in 2001 to 1.3:1 in 2010. Between 2001 and 2010 the hospitalizations with SSc as the principal discharge diagnosis were 0.8 per 1.000, considering all the admission with any diagnosis in the Regional area. The numberof patients admittedin thedecade was 624: 84% women and 16% men (F:M ratio 5.4:1). The largest proportion of patients (42.3%) were aged 45-64 years, followed by those aged >65 years (37.5%). Conclusions This study suggests that between 2001 and 2010 the hospitalization patterns has changed with greater need of day-hospital admission and an increase in the number of access. This may beattributableto an increase ofhospitalization for intravenoustreatmentwith prostanoids and a decrease of systemic complications. References Chung L, et al. Hospitalizations and mortality in systemic sclerosis: results from the Nationwide Inpatient Sample . Rheumatology (Oxford). 2007;46:1808-13. Disclosure of Interest None Declared