T Santiago

Coexisting primary Sjögren’s syndrome and sarcoidosis: coincidence, mutually exclusive conditions or syndrome?

Herein, we describe a 44-year-old female diagnosed with histologically proven coexistence of primary Sjögren’s syndrome and sarcoidosis with pulmonary and muscular involvement. The differential diagnosis may be difficult, but this is not an exceptional case, which highlights the need to critically revise the consideration of sarcoidosis as an exclusion for primary Sjögren’s syndrome, as established in current classification criteria.

Ultrasonography for the assessment of skin in systemic sclerosis: a systematic review

To identify and synthesize the best available evidence on the use of ultrasound to assess skin involvement in systemic sclerosis (SSc).

SAT0501 Early versus late-onset systemic sclerosis: are there clinical and immunological differences?

Background The clinical course of Systemic Sclerosis (SSc) depends on subtype, organ involvement and age. Peak age at onset of SSc is between 30 and 50 years, although SSc may also start in both young and elderly patients. Few data have been reported on patients suffering from late-onset SSc. Objectives To characterise clinical and immunological features of early and late-onset SSc in a tertiary referral hospital. Methods We analysed data from 178 patients followed at our SSc clinic. All the patients fulfilled the ACR/EULAR 2013 classification criteria for SSc or the LeRoy’s criteria for the classification of early SSc. Based on the mean of age of onset of the whole series (50±15 years), ages extremes were defined as younger than 35 versus older than 65 years of age at onset. Disease characteristics as well as clinical and immunological features were evaluated. Results The early and the late-onset groups included 35 and 31 patients, respectively. Patients’ current mean age was 42.8±14.1 vs. 75.8±6.2 with a mean disease duration of 14.5±14.7 vs. 4.3±4.6 years. The most common first manifestation of disease was Raynaud phenomena followed by arthritis/inflammatory arthralgia, in both groups. However, the time between clinical onset and SSc diagnosis was higher in the late-onset group (p=0.034). A higher number of diffuse and pre-SS was observed in the early group but this difference didn’t prove statistically significant. There was a higher prevalence of centromere antibodies in the late-onset group (p=0.001). Clinical manifestations and target-organ damage didn’t differ between groups, except for a higher prevalence of heart conduction abnormalities in the late-onset group (p=0.02). In multivariate analyses, age alone (OR=1.04; 95% CI 1.0, 1.1), but not disease duration (OR=0.99; 95% CI 0.9–1.0), was an independent predictor for the presence of heart conduction abnormalities.Abstract SAT0501 – Table 1 Demographic, clinical and immunological features of Early and Late-Onset SSc Patients. Abbreviations: yr = years; SSc = Systemic Sclerosis. * confirmed by esophageal manometry. ** Based on pulmonary function tests with diffusing capacity of lung for carbon monoxide. *** Diagnosed with echocardiography and confirmed by right heart catheterization wherever available. Conclusions In line with findings from other studies, late-onset SSc shows a distinct clinical and immunological presentation. The present study confirms that late-onset is associated with longer diagnostic delay, positive centromere and heart conduction abnormalities. These observations may be due to age and potential age-associated confounders, rather than the disease itself. Knowledge of these different characteristics can help to improve the management of the disease. References [1] Alba M, et al. Early-versus Late-Onset Systemic Sclerosis. Medicine2014;93(2):73–81. [2] Hugle T, et al. Late-onset systemic sclerosis – a systemic survey of the EULAR scleroderma trials and research group database. Rheumatology (Oxford)2010;50(1):161–5. [3] Manno R, et al. Late-Age Onset Scleroderma. J. Rheumatol2011;38(7):1317–1325. Disclosure of Interest None declared

AB0817 Vitamin D serum levels in a cohort of patients with systemic sclerosis: Prevalence, determinants and associations with clinical and biological aspects

Background Besides the genetic background, it is known that environmental factors play an important role in the pathogenesis and activity of autoimmune diseases and vitamin D deficiency has been associated with the risk and severity of autoimmune diseases. Apart from traditional calcium-related actions, vitamin D is now being recognized for its role in immune-homeostasis, affecting both innate and adaptative immune systems and contributing to immune-tolerance of self-structures. Objectives To compare the vitamin D serum levels in systemic sclerosis (SSc) patients with healthy controls and to explore the predictors of vitamin D serum levels in SSc patients and the association between its levels with clinical and biological aspects. Methods The levels of 25-hidroxyvitamin D (25OHD) were determined in a cohort of 45 SSc patients and in 27 healthy controls gender-, age- and season-matched, not taking vitamin D supplements. The SSc patients underwent a complete clinical and laboratorial evaluation, which included sun exposure time, 25OHD serum level and modified Rodnan skin score. Sun exposure time was estimated using a semi-quantitative scale. 25OHD levels were classified as deficient (<10ng/ml), insufficient (≥10ng/ml and <30ng/ml) and normal (≥30ng/ml). Data was analyzed using the SPSS® version 19.0 for windows. Categorical data is presented as proportions/percentages and continuous variable as median and inter-quartile range. Comparisons across groups were performed using non-parametric tests. P<0,05 is considered statistically significant. Results The mean age was 59.4+11.5 years and 53.7+11.5 years for SSc patients and controls respectively. Females represented 87.7% of the SSc group and 85.2% of the control group. We registered a statistically significant difference in 25OHD levels between SSc group and control group (Z=-4,392, p<0,001, median 16,3ng/ml versus 27ng/ml). Estimated sun exposure time was smaller in SSc patients than in controls. The difference in 25OHD levels between groups remained statistically significant even after adjusting 25OHD levels for sun exposure time. In logistic regression analysis both the disease (β=0,329, p=0,001) and sun exposure time (β=0,483, p<0,001) were considered predictors of 25OHD levels. The mean level of 25OHD among SSc patients was 16,6±8,3 ng/ml and suboptimal levels of 25OHD were observed in 40 patients (88,9%). 25OHD serum levels were lower in patients with shorter sun exposure time (χ2=2,32, p<0,001). A significant negative correlation was observed between 25OHD serum levels and Rodnan skin score (rs=-0,458, p=0,002). Significant correlation was not found between 25OHD serum concentrations and other clinical or biological aspects. Conclusions Patients with SSc have significantly lower median serum vitamin D concentrations than healthy control subjects, and a higher prevalence of vitamin D deficiency. Both disease and sun exposure time are considered good predictor of vitamin D values. Lower levels of vitamin D are observed in patients with shorter sun exposure time and a negative correlation was observed between vitamin D levels and skin involvement. References Caramaschi P, Dalla Gassa A, Ruzzenente O, Volpe A, Ravagnani V, Tinazzi I, Barausse G, Bambara LM, Biasi D. Very low levels of vitamin D in systemic sclerosis patients. Clin Rheumatol. 2010 Dec;29(12):1419-25. Disclosure of Interest None Declared