BackgroundStudies evaluating the difference between men and women diagnosed of scleroderma (SSc) are scarce (1-5) because of the shortage of male patients.ObjectivesAssessing the existence of differences in clinical presentation and mortality causes among men and women in a large cohort of Spanish patients diagnosed with SSc.MethodsA registry of patients with SSc has been performed by the Spanish network for systemic sclerosis (RESCLE) since 2008, in which 90 clinical, immunological and capillaroscopic variables were collected prospectively. Data were collected until May 2014 for this study.Results1506 patients (1341 women), were included. Overall ratio female/male was 8/1. By subtypes lcSSc and preSSc were more frequent in females [84 (51%) vs 808 (60%), p0.023; 6 (3.6%) vs100 (7.5%), p0.076, respectively] and dcSSc in males [62 (38%) vs 293 (22%), p<0.001]. Diagnostic delay was longer in women (2.92 yrs vs 1.30yrs, p<0.001) and smoking was more prevalent in men. Myositis, tendon rubs, interstitial lung disease (ILD), cardiac conduction disturbances and renal crisis were more frequent in males, while isolated pulmonary arterial hypertension (PAH) and sicca syndrome in women. Positivity for ANA, ACA and antiRo was more frequent in women and antiScl-70 and antiRNA pol III in men. There were no sex differences in capillaroscopic patterns. In the multivariate study remained significant in the male predominance in smoking (OR 2.33, CI 1.49 to 4.14, p=0.002) and ILD (OR 1.70, CI 1.11 to 2 60, p=0.015) and in women sicca syndrome (OR 3, 1.63 to 5.52) and ANA positivity (OR 2.67, 1.29 to 5.51).Follow-up time (years) since the onset of symptoms was in males 8.69 yrs vs women 11.79, p=0.001. Altogether, 261 patients died (17%). Although there was no difference in the age at death, this was more common in men (31% men vs 15% women), and time from the onset of symptoms to death was longer in women [14.06 yrs (5.95 to 24.29) vs 8.33 (3.87 to 12.66), p<0.001]. Death was related to SSc in 112 (46%) (22 males vs 90 in women, p ns) while 129 (54%) (31 males vs. 103 for females, p ns) were due to other causes. ILD was the most common cause of death in men (27,5%) and PH in women (28,6%). PAH was more frequently a cause of death in women 39 (19,9%) than in men 0 (0,0%), p<0,001. The cumulative survival at 20 years from onset was 90.9% in women versus 76.3% in men, p<0.001.Conclusions1. In the RESCLE cohort, smoking and ILD were more frequent in males, and sicca syndrome and ANA positivity in women. 2. ILD was the most common cause of death in men and PAH in women. 3. PAH was more frequently a cause of death in women than in men. 4. Men had less survival since the onset of symptoms.ReferencesSimeόn CP et al, Br J Rheumatol 1996.Gaultier JB et al, Rev Med Interne 2008.Panopoulos ST et al, J Rheumatol 2013.Hussein H et al, J Rheumatol 2014.Elhai M et al, Ann Rheum Dis 2014.Disclosure of InterestNone declared

BackgroundSystemic sclerosis (SSc) can virtually affect any organ system (such as lungs, kidneys, gastrointestinal tract, and heart). However, it is the pulmonary manifestations that account for the majority of deaths, especially interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH).ObjectivesOur aim was to assess the differences between severe and mild-to-moderate ILD in SSc.MethodsA descriptive study was performed, using the available data from the Spanish Scleroderma Study Group (RESCLE). ILD was deemed as serious when forced vital capacity (FVC) was <50%. Patients were classified attending the modified classification criteria proposed by LeRoy and Megdsger.ResultsFourteen referral centers for SSc participated in the registry. By April 2014, 1374 patients with SSc had been enrolled, 541 of whom (39.4%) had ILD, which was severe in 72 of them (13.2%). There were no significant differences as far as sex and age at onset is concerned. Patients with diffuse SSc presented with severe ILD more frequently than those with limited SSc (57% vs. 35%, p=0.002), as well as those who had tested positive for ATA (51% vs. 33%, p=0.005). Aditionally, prevalence of FVC<50% was higher in patients with myopathy (32% vs. 15%, p=0.002). Mean FVC was 40.2±6.4 in the severe ILD group, whilst it was 80.3±18.9 in the mild-to-moderate one (p<0.001), and mean DLco was 36.7±15.2 and 62.9±34.5, respectively (p<0.001). Likewise, DLco<70% was also more frequent among patients with severe ILD (100% vs. 69%, p<0.001), as well as mean DLco/VA (56.2±24.2 vs. 74.2±42.0, p=0.002). PAPs was equally higher when FVC<50% (42.2±18.2 vs. 35.1±13.4, p=0.034), and so was the frequency of PAPs>40mmHg (66% vs. 29%, p<0.001) and PAH by right heart catheterism (19% vs. 11%, p=0.050). Finally, by means of a multiple logistic regression, both ATA positivity [OR 0.17 (0.05–0.58), p=0.005] and low DLco [0.93 (0.91–0.95), p=0.000] were found to be related with FVC<50%ConclusionsPatients with ACA positivity and with a limited variant of SSc seem to be at lower risk of severe interstitial lung involvement. Furthermore, the presence of myopathy may contribute to explain the decrease of FVC in SSc patients.References Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 2007; 66: 940–4.LeRoy EC, Medsger TA, Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol 2001; 28: 1573–6. Disclosure of InterestNone declared

BackgroundSome patients with LV-GCA does not have cranial symptoms of giant cell arteritis (C-GCA) and the temporal artery biopsy (TAB) is negative for arteritis. This subset of patients usually has diagnostic delay and inappropriate treatment [1]. Furthermore, patients with biopsy-proven C-GCA and isolated ESR increase could be erroneously considered as a relapse and put on steroid treatment. In these 2 groups of patients, where clinical decisions are difficult the 18F-FDG PET/CT scan may be usefulObjectivesTo study if the realization of a PET/CT with 18F-FDG can be helpful for the diagnosis of difficult cases of LV-GCA and to evaluate relapses in patients with diagnosed C-GCA and elevated markers of inflammationMethodsSince January 2012 two groups of patients, who agree to participate, were prospectively studied at our Hospital: Group 1: Patients with clinical suspicion of C-GCA but with a negative TAB and/or patients with inflammatory general syndrome of unknown origin who did not meet criteria for C-GCA [1]. Group 2: patients with biopsy-proven C-GCA and ESR persistently elevated where relapse was considered. In the 2 groups, once the diagnostic work-up has ended, an 18F-FDG PET/CT was performedResultsA total of 22 patients were studied, 17 were women (77%) with a mean age of 72 years (range: 50-85 years). Group 1 (n=18): The main complaint was: constitutional symptoms in 13 (72%), fever of unknown origin 7 (39%), optic neuritis 1 and relapsing polymyalgia rheumatica (PMR) in another. Symptoms of upper or lower extremity claudication were present in 8 (44%) and 1 (6%) respectivelysevere, abdominal pain in 4 (22%), no patient had stroke. 4 patients (all with normal TAB) had temporal artery abnormalities. Anemia was documented in 10 patients (55%) and ESR>50 in 18 (mean 96; range: 52-140 mm/h). TAB was performed in 9 patients and was negative in all cases. PET data showed vascular uptake suggestive of LV-GCA without temporal enhancement in 16/18 (89%). No patients with a negative PET have LV-GCA: the optic neuritis was caused by B. henselae and the other improved without treatment. The patient previously diagnosed with PMR showed vascular enhancement so this information changed the treatment. In the 4 patients with abdominal pain intense uptake of the abdominal aorta was seen. Group 2 (n=4): all patients had ESR>100 mm/h. 1 patient had symptoms of PMR and the other 3 were asymptomatic. However, 2 patients were on intermittent treatment with steroids. The PET/TC was negative for arteritis in 3 patients which were followed without treatment during a mean of 16.5 months. In the other case, the PET/TC images were typical of PMR (without signs of LV-GCA) and had a good response to low doses of steroidsConclusionsThe judicious use of PET/CT was useful in the diagnosis and management of selected cases of LV-GCA and to avoid unnecessary steroid treatments in patients without vasculitis or relapseReferencesWeyand CM, Gorozny JJ. Gian-Cell Arteritis and Polymyalgia Rheumatica. N Engl J Med 2014; 371:50-7.Disclosure of Int...

Background18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography (18F-FDG PET/CT) is a non-invasive imaging technique commonly used in clinical oncology. 18 F-FDG accumulation is recognised as useful for diagnosing and monitoring the response to therapy in patients with some inflammatory disorders, but the role of the PET/TC in the management of these diseases is debated.ObjectivesThe aim of this study was to investigate the role of 18F-FDG PET/CT in the diagnosis of the disease and assessing disease activity in an autoimmune diseases unit, and to evaluate if the results of this image technique imply a change in clinical management.MethodsWe retrospectively reviewed all 18F-FDG PET/CT requested since August 2015 to August 2017 by our unit. Data collected were: patient demographics, reason for PET request, PET results and change in therapy.ResultsPET/CT were performed in 88 patients and were positive in 68 (77,3%) cases. Patients (49 women/39 men) had a mean age of 58,1±15,7 years (range, 27–92 years). The clinical diagnosis at the moment of ordering the PET/CT were: sarcoidosis (n=45), large-vessel vasculitis (LVV) (n=16), immunoglobulin G4-related disease (IgG4-RD) (n=9), collagen-vacular diseases (CVD) (n=7), mesenteric panniculitis (n=4), myopathy (n=4), polymyalgia rheumatica (PMR) (n=2) and ANCA-associated vasculitis (n=1). (See table 1).Abstract AB1211 – Table 1SarcoidosisLVVIgG4-RDCVDMesenteric panniculitismyopathyPMR n451697442women27(60%)13 (81,2%)1(11,1%)4(57,1%)1(25%)2(50%)1(50%)age50,9(27–8372,5 (29–92)63,7(48–8053(30–8060,566,272,5PET to the diagnosis9(20%)2 (12,5%)1(11,1%)3(42,9%)3(75%)2(50%)2(100%)PET to disease activity36(80%)14(87,5%)8(88,9%)4(57,1%)1(25%)2(50%)0Positive PET/TC36(80%)11(68,7%)6(66,7%)6(85,7%)3(75%)3(75%)2(100%)Change of therapy31(68,9%)11(68,7%)6(66,7%)7(100%)2(50%)3(75%)2(100%)PET results supported a change in therapeutic management in 71.6% of the cases. In the group of sarcoidosis there was a change in treatment in 68.9% of cases. PET/TC revealed extrapulmonary manifestations in 57,8%. All patients with extracranial manifestations of giant cell arteritis (GCA) showed uptake in PET/TC. That result supported the clinical diagnosis, although negative temporal artery biopsy. PET was useful in the diagnosis and treatment of IgG4-RD in 66,7% of cases. PET scan did not find malignancy in inflammatory myopathies nor mesenteric panniculitis.ConclusionsPET/TC is an increasingly utilised in our patients with inflammatory disorders as a support for the diagnosis and management. PET/TC detection extrapulmonary sarcoidosis may have therapeutic and prognostic clinical implications. PET may be useful in patients with GCA supicion with negative biopsy and extracranial symptoms. More studies will be necessary to establish the real role of PET/TC in autoimmune and inflammatory diseases.Disclosure of InterestNone declared

Background Progressive systemic sclerosis (PSS) is a complex multisystem connective tissue disease whith unknown aetiology, but environmental and genetic factors may play a role. Since the first association of silica exposure and PSS by Erasmus in 1957 [1], several case reports documented this co-existence [2], but with conflicting results depending on the population studied [3]. Objectives To stablish the incidence and to describe the clinical and analytical characteristics of patients with Erasmus syndrome in our health area. Methods Medical records of patients admitted to the Complejo Hospitalario Universitario of Vigo from 1994 to 2011 with the diagnosis of PSS were reviewed, selecting those with documented exposure to silica. Results During this time, the diagnosis of PSS was confirmed in 86 inpatients. 10 patients (7 of the 13 total males, 3 of the 73 total females) had also documented environmental exposure to silica dust and were selected for the study. 6 patients had worked in granite quarries, one digging tunnels and the other 3 in ceramic factories. The mean age at diagnosis of scleroderma was 51 years (range 26-76). According the classification proposed by LeRoy and Medsger [4] six were diffuse and three limited variants. Other two were diagnosed of mixed connective tissue disease. The initial symptom was Raynaud’s phenomenon (RP) in 50% and cutaneous sclerosis in 20%, having appeared a median of 1 year prior to diagnosis. Clinical manifestations at the moment of diagnosis were: dyspnoea 90%, RP 80%, esophageal dysmotility 70%, skin ulcers 40%, telangiectasia 30%, arthritis 30%, tendinopathy 20%, calcinosis and renal crisis 10%. Autoantibody positivity was observed as follows: 10 ANA (1 anticentromere pattern), 2 anti-dsDNA, 1 anti-SM, 1 anti-RNP, 6 anti-Scl70, 2 ACLA, 3 RF. In 9 patients we observed interstitial lung disease data on imaging tests. Impairment in the diffusion test was observed in 7 of the 8 patients in whom lung function studies were performed, 5 of them with also a typical restrictive ventilatory pattern and decreased forced vital capacity. Bronchoscopy and transbronchial biopsy was done in 4 patients, and fibrosis and deposition of silica was described in all samples. Transthoracic echocardiography was performed in 7 patients and estimated PASP was above 25 mmHg in 5. Right cardiac catheterization was performed only in 2 patients, with mPAP measure confirmatory of PAH. 7 patients died during follow-up, with a mean of 4.2 years from diagnosis. Cause of death was respiratory failure in 3 cases, refractory heart failure in 2, liver cirrhosis in one and unknown in another one. Conclusions Erasmus syndrome is a common condition among men with PSS who require admission in our health area, diffuse variant being predominant. Clinical course of our patients is rapidly progressive, with frequent development of cardiopulmonary mixed complications and a median survival of 4.2 years from diagnosis. References Erasmus LD. Scleroderma in goldminers of the Witwatersrand with particular referen...