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Objectives: Diffuse cutaneous systemic sclerosis (dcSSc) is associated with anti-topoisomerase (ATA) whereas limited cutaneous (lcSSc) and sine scleroderma (ssSSc) are mainly associated with anti-centromere antibody (ACA). Serodiscordant patients were defined as lcSSc subjects with ATA, dcSSc with ACA, and ssSSc with ATA. The aim of the present study was to compare the clinical manifestations and prognosis between serodiscordant patients and their counterparts (those with lcSSc with ACA, dcSSc with ATA and ssSSc with ACA, respectively)., Methods: From the Spanish Scleroderma Registry we selected those patients for whom skin involvement (dcSSc, lcSSc or ssSSc) was detailed at baseline and last visit and ACA and ATA had been determined. Demographic, clinical characteristics, and survival data were compared according to the antibody status., Results: The whole cohort comprised 901 patients and six mutually exclusive groups were defined: lcSScACA in 511 (57%) patients, lcSScATA group in 87 (10%), dcSScATA group in 172 (19%), dcSScACA group in 21 (2%), ssSScACA group in 92 (10%), and ssSScATA group in 18 (2%) patients, respectively. Interstitial lung disease (ILD) and severe ILD were more frequent in patients with dcSScATA than in those with dcSScACA. Conversely, the prevalence of isolated pulmonary hypertension (without ILD) was higher in those with dcSScACA (15% vs. 2%; p=0.018). No differences were found regarding survival when comparing serodiscordant patients with the seroconcordants patients., Conclusions: In our cohort, the prevalence of serodiscordant SSc patients was low. They differed from their counterparts in some clinical manifestations. The management of patients with SSc should be guided by both serology and cutaneous subtype.

Introduction: Our objective was to evaluate the pulmonary hypertension (PH) data for Spanish patients with systemic sclerosis (SSc), define the PH types and determine the associated factors., Method: Descriptive study of PH-related data from the multicentre RESCLE registry. Estimated systolic pulmonary artery pressure (esPAP), measured via echocardiogram was considered elevated if ≥ 35 mmHg. Left heart disease (LHD) and interstitial lung disease (ILD) were identified. When performed, data from right heart catheterisation (RHC) were collected., Results: esPAP was elevated in 350 of 808 patients (43.3%). One hundred and forty-four patients (17.8%) were considered to have PH (88 via RHC and the rest due to elevated esPAP along with evidence of significant LHD or ILD): PAH 3.7%, secondary to ILD 8.3%, secondary to LHD 2.8% and unclassified 3%. Prevalence of elevated esPAP was greater in diffuse SSc (dSSc) than in limited scleroderma (lSSc) (50.5 vs. 42.2%, p 0.046). In the group with elevated esPAP, a lower prevalence of anti-centromere antibodies (41.9% vs. 52.3%, p 0.006) and a greater prevalence of anti-topoisomerase-1 antibodies (ATA) (25.1% vs. 18.6%, p 0.04) were observed compared to the group with normal esPAP. Patients with elevated esPAP had a lower rate of digital ulcers (50.6% vs. 60.2%, p 0.007) and esophageal involvement (83.6% vs. 88.7%, p 0.07) and higher rate of renal crisis (4.6% vs. 1.8%, p 0.066)., Conclusions: Prevalence of PAH was lower than expected (3.7%). Probability of having elevated esPAP was higher among patients with dSSc and among those with ATA.

Objectives: To assess the clinical manifestations and prognosis of Spanish patients with systemic sclerosis (SSc) according to their immunological profile., Methods: From the Spanish Scleroderma Study Group or RESCLE (Registro de ESCLErodermia as Spanish nomenclature) Registry we selected those patients in which anti-centromere (ACA), anti-topoisomerase I (ATA), and anti-RNA polymerase III (ARA) antibodies had been determined, and a single positivity for each SSc specific antibody was detected. Demographic, clinical, laboratory, and survival data were compared according to the serologic status of these antibodies., Results: Overall, 209 SSc patients were included. In 128 (61%) patients ACA was the only positive antibody, 46 (22%) were only positive for ATA, and 35 (17%) for ARA. Of note, the three groups were mutually exclusive. In univariate analysis, patients with ACA presented more frequently limited cutaneous SSc (lcSSc) (p<0.001), whereas diffuse cutaneous SSc (dcSSc) was the most frequent subtype in patients with ATA (54%) and ARA (62%) (both p<0.001). Positive patients for ARA showed the highest prevalence of joint involvement (p<0.001) and those from ATA group had a higher prevalence of interstitial lung disease (ILD) (p<0.001). Scleroderma renal crisis was more frequent in the ARA group (p<0.001). In multivariate analysis, ACA were associated with female gender and were protective for dcSSc and ILD. ATA were found to be protective for lcSSc and they were independently associated with interstitial reticular pattern. ARA positivity was independently associated with dcSSc. We did not find differences in mortality between the three groups., Conclusions: In Spanish SSc patients, the presence of SSc specific antibodies conferred a distinctive clinical profile.

Objectives: The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in Ssc patients, and contradictory results have often been show among those studies that have been performed., Methods: A prospective study was conducted with the Spanish RESCLE register to analyse the influence of gender on survival of SSc patients., Results: In total, 1506 SSc patients (1341 women, 165 men) were recruited from 21 centres. Older age at onset (OR 1.02), shorter time from onset to diagnosis (OR 0.96), smoking (OR 2.57), interstitial lung disease (ILD) (OR 1.58), less predisposition to sicca syndrome and to antinuclear antibody positivity (OR 0.29 and 0.43, respectively), and higher compliance with the ACR 1980 criteria (OR 1.79) were independently associated with the male sex. During follow-up, 30.4% of men versus 14.6% of women died (p<0.001). Survival at 10 years from the onset of symptoms was 75.3% for men and 92.9% for women (p<0.001), and the difference remained after selecting only SSc-related deaths (85.6% vs. 96.1%, p<0.001). The mortality predictive factors were diffuse SSc (OR 2.26), ILD (OR 1.82), digital ulcers (OR 1.38), tendon friction rubs (OR 1.74), male sex (OR 1.53), increased age at onset (OR 1.13) and isolated PH (considering only deaths from diagnosis), both in the overall (OR 3.63) and female cohorts (OR 3.97). The same risk factors were observed in the male cohort, except for isolated PH and ILD., Conclusions: The present study confirms the existence of epidemiological, clinical, laboratory and prognostic gender differences in systemic sclerosis patients.

Objectives: To determine the changes in the pattern of death of patients with systemic sclerosis (SSc) throughout 20 years., Methods: Data were collected from the Spanish Scleroderma Registry (RESCLE), retrospective multicentre database from 1990 to 2009. SSc-related and SSc-non related causes of death were assessed., Results: 987 patients were recruited. Overall standardised mortality ratio (SMR) was 2.34 (2.24-2.44). SSc-related causes of death were responsible of 72% of all deaths of those patients diagnosed within 1990-99 vs. 48% within 2000-09 (p=0.006). Relative pulmonary death rate was stable over time (68.1% within 1990-99 vs. 63.9% within 2000-09, p=0.815). Relative renal death rate was decreasing over time (17% within 1990-99 vs. 5.5% within 2000-09, p=0.175). Heart distribution tripled its ratio (12.8% within 1990-99 vs. 30.6% within 2000-09, p=0.058)., Conclusions: SSc-related causes of death were decreasing over time and, among them, pulmonary involvement was the leading cause of death in both decades. The ratio of renal causes decreased since 1990 at the time that the ratio of cardiac causes increased.

Objectives: To evaluate a new ultrasound sign, pleural irregularity (PI), for the study of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc) and antisynthetase syndrome (ASS)., Methods: The study included patients from our SSc and ASS cohorts with varying degrees of ILD, enrolled from 2011 to 2014. Chest high-resolution computed tomography (HRCT), pulmonary function tests (FVC and DLCO) and chest sonography were performed in each patient. Ultrasound PI and B-lines were quantified using a 72-sonographic point score and HRCT lung abnormalities were quantified using Warrick and Wells scores and categorised through Goh's algorithm. PI was correlated with HRCT and pulmonary function test parameters and its diagnostic performance to detect and classify the extent of ILD was evaluated and compared with B-lines., Results: Thirty-seven patients were studied, 21 with ASS and 16 with SSc (8 without ILD). PI correlated with the Warrick score both in SSc (r=0.6, p=0.01) and ASS patients (r=0.6, p=0.005), showing a higher performance to detect ILD than using B-lines (p=0.01). In SSc patients PI also correlated with Wells score (r=0.7, p<0.001) and with DLCO (r=-0.5, p=0.05), showing a high diagnostic value for detecting ILD (AUC=0.85, 95% CI 0.64-1) and classifying it into limited or extensive (AUC=0.81, 95% CI 0.57-1). A modification of the Goh algorithm including PI was developed as a screening tool to avoid the use of HRCT in SSc patients without ultrasound evidence of extensive ILD., Conclusions: PI is useful for evaluation of ILD in SSc and ASS patients, and can be incorporated into a diagnostic algorithm in SSc patients to reducing the need for exposure to ionising radiation.

Objectives: To study the bone mass in patients with scleroderma (SSc) from two different Spanish regions and to evaluate the prevalence of vitamin D deficiency and insufficiency in this population and its possible relation to bone mineral density (BMD)., Methods: Disease, bone mineral density related variables and vitamin D were collected from all patients. Statistical analysis was carried out using the SPSS 17 statistics software for Windows. A p<0.05 was considered significant., Results: A Z-score <-1 was found in 21.9% of the control population and 43% of SSc patients. The prevalence of osteopenia/osteoporosis was 50% in the control population and 77% in SSc (p<0.0001). We did not find differences between the prevalence of low BMD in the south (79%) and in the north of Spain (76.3%); but patients from the north had lower levels of vitamin D (27.4±16.2 ng/dL vs. 20.7±11.0 ng/dl; p<0.031). Low levels of vitamin D (<30ng/ml) were found in 69 patients out of 90, ten of them with insufficiency (<10ng/ml). Eighty-four point six percent of the patients with low levels of vitamin D (<30ng/ml) had LBMD compared whith 66.7% of those with normal levels (p=0.073)., Conclusions: The prevalence of osteoporosis/osteopenia in Spanish patient with SSc is very high. Although there are a high prevelence of vitamin D deficiency, we could not demonstrate a relationship of vitamin D deficiency with low mineral density.