Search

Objective.Major salivary gland ultrasonography (SGUS) is a widely used imaging technique to evaluate salivary gland involvement in primary Sjögren syndrome (pSS). The aim of this study was to evaluate the relationship between SGUS, salivary flow rate (SFR) as an objective measure of the gland function, and oral health-related quality of life (OHRQOL) as a patient-reported outcome measure (PROM) in a pSS cohort.Methods.Sixty-six patients with pSS were examined by SGUS according to Hocevar and Milic scoring systems. Patients with inhomogeneity/hypoechoic areas with scores ≥ 2 in parotid and submandibular glands were classified separately as “severe glandular involvement.” Further, oral health, SFR, and Oral Health Impact Profile-14 (OHIP-14) for OHRQOL were assessed.ResultsBoth total Hocevar and Milic scores were higher in 21 pSS patients with low unstimulated whole salivary flow rate (U-WSFR) than 45 pSS patients without low U-WSFR (P = 0.001 and P < 0.0001, respectively). Increased scores of homogeneity, hypoechoic areas and glandular border visibility were observed in patients with low U-WSFR (P < 0.05). Among these variables, homogeneity score was found to be an independent risk factor for low U-WSFR in pSS according to logistic regression analysis (OR 1.586, P = 0.001). Moreover, a higher OHIP-14 score was observed in severe parotid involvement compared to nonsevere cases (23.26 ± 21.19 vs 8.32 ± 13.82, P = 0.004).Conclusion.High Milic and Hocevar SGUS scores are associated with reduced SFR and poor OHRQOL as a PROM. The inhomogeneity component of the SGUS score is associated with low U-WSFR and is an indicator of severely affected gland function.

To compare the clinical features, laboratory findings, and prognosis of Behçet's disease (BD) patients with and without Budd-Chiari syndrome (BCS).This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017.Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14-47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%).To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points • Mortality rate is higher in BD-associated BCS patients with IVC involvement. • Chronic and silent form of BD-associated BCS has a better prognosis. • The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial.

Objectives: To determine the prevalence of vitamin D deficiency in patients with small and medium vessel systemic vasculitis.& nbsp;Methods: In this cross-sectional study, 25-hydroxy (OH) vitamin D3 levels were measured in adult patients with systemic small and medium vessel vasculitis including antineutrophil cytoplasmic antibody-associated vasculitis (AAV), cryoglobulinaemic vasculitis (CryV), IgA vasculitis (IgAV) and pol-yarteritis nodosa (PAN), and age- and sex-matched healthy subjects (HS) and patients with rheumatoid arthritis (RA) as control groups. 25OH vitamin D3 levels < 30 ng/ml and < 20 ng/ml were regarded as insufficiency and deficiency, respectively.& nbsp;Results: Fifty-seven patients (42 AAV, 2 CryV, 8 IgA vasculitis, 5 PAN) with systemic vasculitis, 101 HS, and 111 RA patients were included. The mean 25OH vitamin D3 level was 21.8 +/- 14.2 ng/mL in patients with vasculitis, 42.7 & nbsp;+/- 27.6 ng/mL in HS (p < .001) and 20.1 +/- 18.47 ng/mL in patients with RA (p = .54). Vitamin D insufficiency and deficiency were significantly higher in patients with systemic vasculitis compared to HS (75.4% vs 33.7%, p < .001; %50 vs 21.8%, p < .001, respectively). Vitamin D status was not different in patients with systemic vasculitis compared to RA. There was a negative correlation between vitamin D status and CRP levels (=-.364, p = .007). The multivariate logistic regression analysis showed that renal involvement was significantly associated with vitamin D deficiency/insufficiency in patients with vasculitis (OR 22.5 [95% CI 1.6-128.9].& nbsp;Conclusion: Vitamin D deficiency and insufficiency are more frequent in patients with systemic small and medium vessel vasculitis and RA than HS. Renal involvement is one of the factors associated with vitamin D deficiency/insufficiency in patients with vasculitis. Objetivos: Determinar la prevalencia de la deficiencia de vitamina D en los pacientes con vasculitis sistémica de peque ̃nos y medianos vasos. Métodos: En este estudio transversal se midieron los niveles de 25-hidroxivitamina D3 en pacientes adul- tos con vasculitis sistémica de peque ̃nos y medianos vasos, incluyendo vasculitis asociada a anticuerpos anticitoplasma de neutrófilos (AAV), vasculitis crioglobulinémica (CryV), vasculitis IgA (IgAV) y poliar- teritis nodosa (PAN), y sujetos sanos pareados por edad y sexo (SS) y pacientes con artritis reumatoide (AR) como grupos control. Se consideraron insuficientes y deficientes los niveles de 25-hidroxivitamina D3 < 30 ng/ml y < 20 ng/ml, respectivamente. Resultados: Se incluyeron 57 pacientes (42 de AAV, 2 de CryV, 8 de vasculitis IgA y 5 de PAN) con vasculitis sistémica, 101 SS y 111 pacientes de AR. El nivel medio de 25-hidroxivitamina D3 fue de 21,8 ± 14,2 ng/ml en pacientes con vasculitis, 42,7 ± 27,6 ng/ml en SS (p < 0,001) y 20,1 ± 18,47 ng/ml en pacientes con AR (p = 0,54). La insuficiencia y deficiencia de vitamina D fueron significativamente más altas en los pacientes con vasculitis sistémica en comparación con los SS (75,4 vs. 33,7%; p < 0,001; 50 vs. 21,8%; p < 0,001, respectivamente). El estatus de vitamina D no fue diferente en los pacientes con vasculitis sistémica en comparación con AR. Existió una correlación negativa entre el estatus de vitamina D y los niveles de PCR = −0,364; p = 0,007. El análisis de regresión logística multivariante reflejó que el compromiso renal estuvo significativamente asociado a la deficiencia/insuficiencia de vitamina D en los pacientes con vasculitis (OR: 22,5; IC 95%: 1,6-128,9). Conclusión: La insuficiencia y deficiencia de vitamina D son más frecuentes en los pacientes con vasculitis sistémica de peque ̃nos y medianos vasos y AR que en los SS. El compromiso renal es uno de los factores asociados a la deficiencia/insuficiencia de vitamina D en los pacientes con vasculitis.

Tezcan, Mehmet Engin/0000-0002-1753-4936; TUFAN, Abdurrahman/0000-0001-6244-9362; SARI, Ismail/0000-0001-7737-4180; YILMAZ, Sedat/0000-0002-4691-3417 WOS: 000436403100017 PubMed: 28992387 ObjectiveApproximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients. MethodsIn all, 21 centers from different geographical regions of Turkey were included in the current study. The medical records of all FMF patients who had used anti-IL-1 treatment for at least 6 months were reviewed. ResultsIn total, 172 FMF patients (83 [48%] female, mean age 36.2 years [range 18-68]) were included in the analysis; mean age at symptom onset was 12.6 years (range 1-48), and the mean colchicine dose was 1.7 mg/day (range 0.5-4.0). Of these patients, 151 were treated with anakinra and 21 with canakinumab. Anti-IL-1 treatment was used because of colchicine-resistant disease in 84% and amyloidosis in 12% of subjects. During the mean 19.6 months of treatment (range 6-98), the yearly attack frequency was significantly reduced (from 16.8 to 2.4; P < 0.001), and 42.1% of colchicine-resistant FMF patients were attack free. Serum levels of C-reactive protein, erythrocyte sedimentation rate, and 24-hour urinary protein excretion (5,458.7 mg/24 hours before and 3,557.3 mg/24 hours after) were significantly reduced. ConclusionAnti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients.

Background: Radiographic severity of ankylosing spondylitis (AS) shows such great variance that some patients never develop syndesmophytes throughout the entire disease span, whereas some develop bamboo spine relatively early. Objective: To study the association between ERAP1, IL23R and PTGER4 single nucleotide polymorphisms (SNPs) and radiographic severity in AS patients. Methods: rs27044 and rs30187 (ERAP1), rs11209032 (IL23R) and rs10440635 (PTGER4) SNPs were genotyped in 235 AS patients fulfilling the modified New York criteria. Patients were classified as mild- and severe-AS according to modified Stoke AS spinal score (mSASSS). Mild-AS is defined as having mSASSS of “0” following at least 10 years of disease duration. Severe-AS is defined as having mSASSS of >20 (patients with mild vertebral changes (i.e. squaring or erosions) were omitted for clear stratification) regardless of disease duration. Results: The genotype distributions and allele frequencies of ERAP1 rs27044 and rs30187, IL23R rs11209032 and PTGER4 rs10440635 SNPs were similar in mild- (n=171, mSASSS=0, 55.6% HLA-B27 positive) and severe-AS patients (n=64, mSASSS=48.5±17.8, 73.4% HLA-B27 positive). After adjustment for clinical differences between groups (gender, disease duration, HLA-B27 and smoking status) by logistic regression analysis, none of the alleles in the investigated SNPs were found to be associated with radiographic severity of AS. Conclusion: In radiographically well-categorized AS patients, ERAP1 rs27044 and rs30187, IL23R rs11209032 and PTGER4 rs10440635 SNPs are not found to be associated with radiographic severity of AS.

Objective To determine the frequency of subclinical atherosclerosis in patients with systemic sclerosis (SSc; scleroderma) compared to healthy subjects (HS) and rheumatoid arthritis (RA) patients and to determine the ability of cardiovascular (CV) risk indices in detecting SSc patients with subclinical atherosclerosis. Methods A total of 110 SSc patients (102 females and 8 males, mean ± SD age 50.5 ± 11.9 years), 110 age- and sex-matched RA patients, and 51 HS without CV disease were examined with ultrasonography (US). Carotid intima-media thickness (cIMT) >0.90 mm and/or carotid plaques were used as the gold standard for subclinical atherosclerosis (US+). Systematic Coronary Risk Evaluation (SCORE), QRisk II, and 2013 American College of Cardiology (ACC)/American Heart Association (AHA) CV risk indices were calculated. Results Twenty-one (19.1%) SSc patients, 24 (21.8%) RA patients, and 3 (5.9%) HS had subclinical atherosclerosis (SSc versus RA: P = 0.62, SSc versus HS: P = 0.029). cIMT in SSc was higher compared to HS (0.68 ± 0.15 mm versus 0.61 ± 0.10 mm; P = 0.008) but similar to RA patients (0.66 ± 0.14 mm; P = 0.82). Subclinical atherosclerosis in SSc was associated with age (odds ratio [OR] 1.07, P = 0.013), elevated erythrocyte sedimentation rate (OR 3.4, P = 0.045), and pulmonary arterial hypertension (OR 4.27, P = 0.012). Concerning CV risk indices, of the 21 US+ SSc patients only 0, 3 (14.2%), and 6 (28.6%) were classified as high CV risk according to SCORE, QRisk II, and ACC/AHA risk indices, respectively. Conclusion Subclinical atherosclerosis in SSc patients is more frequent than in HS, but is as frequent as in RA patients in which accelerated atherosclerosis is clearly defined. CV risk indices for the general population are considerably insufficient to detect SSc patients with atherosclerosis.

Behçet's disease (BD) is characterized by recurrent oro-genital ulcers, mucocutaneous lesions, and serious organ involvement. We investigated the salivary microbiome in BD using high-throughput sequencing of the 16S rRNA V4 region. Stimulated saliva samples were collected from 31 BD patients and 15 healthy controls, and in 9 BD patients, a second saliva sample was collected following dental and periodontal treatment. Sequence analysis identified a total of 908 operational taxonomic units (OTUs) present across all samples. Patients had a microbial community structure that is significantly less diverse than healthy controls. The most overabundant species in BD was Haemophilus parainfluenzae, while the most depleted included Alloprevotella rava and species in the genus Leptotrichia. Periodontal treatment improved oral health indices in BD but had no short-term effect on bacterial community structure. Neither the BD-associated genetic risk locus within the HLA-B/MICA region nor being on immunosuppressive medications explained the differences between patients and controls.

Background Salivary flow rates (SFR) and oral health were known to be frequently impaired in Sjogren’s syndrome (SjS) due to chronic inflammation and destruction of the salivary glands. Ultrasonography (USG) of major salivary glands (SG-USG) is a non-invasive widely used tool to evaluate salivary glands in SjS. Objectives The aim of the study was to assess the relationships between SFRs, oral health and USG changes of major salivary glands in patients with primary SjS. Methods Fifty-nine SjS patients (F/M:57/2) with the mean age of 52.2±11.5 years. The duration of follow-up period of 9.±7.1 years fulfilling ACR-EULAR classification criteria (2002) were included. Major salivary glands (bilateral parotis and submandibular glands) were scored according to two different scoring systems which are Hocevar A.(0–48) and Milic VD. (0–12). Oral health was assessed by indices. Measurements of whole unstimulated and stimulated SFRs were carried out in patients between 9 a.m. and 10 a.m in the morning. Oral health related quality of life (OHRQoL) as a patient reported outcome measure was evaluated by using Oral health impact profile (OHIP-14). High scores indicated poor OHRQoL status. Pathologic findings in oral health (GM) and USG images (NI) were evaluated by two authors as double-blind. Results Unstimulated SFR (0,2±0,2 ml/min) was correlated with stimulated SFR (1,1±0,7 ml/min) in the whole group (r=0,8 p=0.000). Moderate correlations were seen between unstimulated and stimulated whole SFRs and scores of hypoechogenic areas in bilateral parotid and submandibular glands (p Conclusions Unstimulated and stimulated SFRs were found correlated with the structural changes of major salivary glands. Tooth loss and poor OHRQoL were shown in patients due to reduced salivary outputs. USG images of salivary glands could give insight to physicians about oral health and OHRQoL as outcome measure in patients with SjS. Disclosure of Interest None declared

Objectives Corticosteroids (CS) are accepted as the standard first-line treatment for giant cell arteritis (GCA). However, controlled trials of tocilizumab and abatacept demonstrated relapse rates of up to 70%–80% in patients on CS-only protocols in 12–24 months. Though level of evidence is low and not suggested by guidelines (except for methotrexate), conventional immunosuppressives (ISs) are also commonly used. We aimed to assess the relapse rates in patients with GCA in routine practice, retrospectively. Methods We assembled a retrospective cohort of patients with GCA from Turkey. All data was abstracted from records. Relapse was defined as any new manifestation or increased acute-phase response leading to the change of the CS dose or use of a new therapeutic agent by the treating physician. Results The study included 156 (F/M: 95/61) patients with GCA (table 1). The mean age at disease onset was 67.8±9.1 years. Polimyalgia Rheumatica was also present in 48 (30,8%) patients. Diagnosis was proven histopathologically in 99 patients.All patients received 1 mg/kg/day CS for remission induction, additional CS pulses were given to 36 (23.1%) patients. Conventional ISs including methotrexate and azathioprine were used in 89 (56.1%) and 26 (16.6%) patients respectively, while 10 (6.4%) patients received biologic treatments (8 tocilizumab,2 etanercept). Fourty-four (28.2%) patients used only CS during follow-up. Follow-up of at least 6 months was available for 132 patients, and median follow-up duration was 35 (6–268) months. Relapses occurred in 27 (20.5%) patients during follow-up. Mortality rate was 7.5% (n=10) during follow-up. VDI score was 2.4±1.7. Main causes of damage were related to CS treatments such as cataract, osteoporosis and diabetes mellitus. Conclusions In this first multi-centre series of GCA from Turkey, we observed that only one fifth of patients had relapses during a mean follow-up of 35 months. This lower relapse frequency suggests a different clinical spectrum in routine practice compared to patients included in controlled trials. Our results also suggest that there is a clear need for a steroid sparing agent in patients with GCA, that is a older aged population prone to CS side effects. Disclosure of Interest None declared

Background Ultrasonography (USG) of major salivary glands (SG-USG)is a non-invasive tool that has been used to evaluate salivary glands in primary and secondary Sjogren’s syndrome (SjS). Objectives We aimed to investigate relation between the ultrasonographic scoring of major salivary glands and systemic disease activity or salivary secretion in patients with primary SjS. Methods Seventy-five SjS patients (F/M:73/2) with the mean age of 52±12 and duration of follow-up period of 58±54 months fulfilling ACR-EULAR classification criteria (2002) were included. Disease activity indexes (Sjogren’s Syndrome Patients Reported Index (ESSPRI), Visual Analogue Scale (VAS), EULAR Sjogren’s Syndrome Disease Activity Index (ESSDAI)] were recorded. Simultaneously, sialometric evaluation of the salivary glands was performed. Major salivary glands (bilateral parotis and submandibular glands) were scored according to two different scoring systems [Hocevar A.(0–48) and Milic VD. (0–12)] and elastography was recorded as well. Results Demographics, clinical characteristics, disease activity indexes and SG-USG scores were summarised in table 1 and table 2. Forty-one (55%) and 45 (60%) patients had the cut-off values of ≥17 (Hocevar) and ≥6 (Milic USG). The patients with the scores of ≥17 (Hocevar) were found to have higher scores of ESSPRI-total (16±6 vs 13±7, p=0,045) and lower sialometry (4,6±4,7 vs 8,4±4,6 ml, p=0,002). Scores of Hocevar and Milic-USG were negatively correlated with sialometry (r=-,430, p=0001 and r=-,430, p=0,001). Hocevar, Milic and elastography-USG scores were shown to be higher in patients with sialometry of ≤1,5 ml (n=7)(28±3 vs 17±10, p=0,010, 8±1 vs 5±3, p=0006 and 9±1 vs 5±2, p=0,028) and anti-Ro positivity(n=24) (24±10 vs 13±8 7±3 vs 4±2, p Conclusions Hocevar scoring system of major salivary glands was found to be related to patient reported activity in SjS. USG scores were associated with reduced saliva secretion and anti-Ro positivity. Severe parotid involvement was shown to be related to anti-RO and La positivity. Evaluation of SG-USG including different scoring systems and elastography might reflect function of the salivary glands. Disclosure of Interest None declared

Purpose: To describe posterior segment findings of antiphospholipid syndrome (APS) and compare them with systemic lupus erythematosus (SLE). Methods: A total of 11 patients with primary APS, 29 secondary APS patients, and 29 SLE patients without APS were included. All patients were referred from rheumatology clinic for detailed ophthalmologic examination. When patients had suspicious lesions, fundus fluorescein angiography was performed (n = 56). Results: The most common retinal examination finding was peripheral venous tortuosity (17.5%) in APS, which was not observed in SLE group. Common FFA findings were pigment epithelial window defects (10%) and vascular filling delays (7.5%) in APS, which were observed in 27.5% and 3.5% of patients with SLE consecutively. Conclusion: Venous tortuosity was significantly more in patients with APS. There was no significant difference for other ocular findings between the groups. Ocular complication rate was lower compared to earlier reports, probably due to better management of disease activity with current treatment protocols. © 2018, © 2018 Taylor & Francis Group, LLC. 2-s2.0-85058681751 PubMed: 30556792

Objectives The prevalence of atherosclerotic risk factors and disease in Takayasu's arteritis (TAK) has not been well defined. We aimed to assess the frequency of cardiovascular (CV) risk factors and the incidence of CV events (CVEs) in patients with TAK from two ethnically different populations. Methods Patients with TAK followed at Mayo Clinic, Rochester, MN, USA and Marmara University, Istanbul, Turkey were included in this retrospective study. Patients with TAK were compared with age-, sex- and calendar year-matched controls from the same geographical region without TAK. The 2008 Framingham 10-year general CV risk score (FRS) was used for the evaluation of CV risk at the time of TAK incidence/index date. Results In total, 191 patients with TAK and 191 non-TAK controls were included. Hypertension and the prevalence of lipid-lowering treatments were significantly more frequent in TAK. Prior to the incidence/index date, occurrence of CVE was significantly higher in TAK. The FRS was significantly higher in TAK compared with non-TAK at incidence/index date. The cumulative incidence of CVE was 15.4% at 10 years in TAK vs 5.8% in non-TAK; the risk of CVE was increased among patients with TAK (hazard ratio = 4.36; 95% CI: 1.25, 15.13). Conclusion CV risk factors are more common in patients with TAK, particularly hypertension. The FRS is higher in patients with TAK at the time of diagnosis. The cumulative incidence of CVE was also significantly higher during follow-up in TAK. Our results suggest that patients with TAK should undergo careful assessment of CV risk factors, and an aggressive risk modification approach is warranted.

Background Severity of peripheral vascular involvement (PVI)is known as an important determinig cause of morbidity in sytemic sclerosis (SSc). Different vasodilating agents have been found to reduce severity and contribute to healing of digital ulcers (DU)in SSc. Objectives We aimed to evaluate the characteristics and capillaroscopic patterns of the patients with severe PVI under different vasodilator therapeutic regimens. Methods The patients were grouped as “severe PVI” if score of PVI is ≥2 (PVT=2,digital pitting scars; PVT=3, digital tip ulcerations and PVT=4, digital gangrene; Medsger) and “non-severe PVI” if score of PVI is ≤1 (PVI=0,no Raynaud9s; PVI=1, Raynaud9s requiring vasodilators). We included patients with severe PVI who received cyclic iloprost and bosentan and/or sildenafil and compared to non-severe PVI. Nail fold video-capillaroscopy (NVC)was assessed qualitatively (Cutalo et al., early, active and late patterns). Results Severe PVI group more frequently had diffuse cutaneous form, contractures, lung disease, anti-Scl70 positivity and high acute phase response and exposed to immunosuppressives (table-1). Scores of telangiectasia, skin, activity and severity were lower in non-severe group. NVC late pattern was frequent and early pattern was rare in severe PVI groups. Forty-one% (9/22) of patients received second oral agent for PVI. Monotherapy and combination groups had similar scores (table-2). Conclusions NVC late pattern was frequent in severe PVI group with long disease duration, exposed to intensive immunosuppresives and received specific vasodilators for PVI. Disease activity and severity were higher in severe PVI group. One third of the severe group required oral combination therapy for PVI. Monotherapy and combination groups were similar in terms of severity. NVC is a useful method to monitor digital ischemia and severe organ involvement in SSc. Disclosure of Interest None declared

Background Gastrointestinal system (GI) is commonly involved in systemic sclerosis (SSc) beginning after early stages of disease. Objectives We aimed to investigate capillaroscopic findings among the SSc patients with GI involvement and malnutrition. Methods GI involvement was evaluated by UCLA SCTC GIT 2.0 questionnaire (Khanna D.) (7 multi-item; reflux, distension, soilage, diarrhea, social functioning, emotional wellbeing and constipation) in SSc patients ACR/EULAR classification criteria (2013). To report the nutrition status of the patients “malnutrition universal screening tool (MUST)” (sum of the scores of body mass index, weight loss in last 3–6 months and acute disease effect) scores were calculated. Simultaneously, nail fold video-capillaroscopy (NVC) was performed in all paatients to deterimine ealy, active late scleroderma patterns (Cutalo et al.). Results In 58 SSc patients (51 female); the mean age, duration of Raynaud9s, non-Raynaud symptom (year) and follow-up (month) were 46±13, 54±50, 10±9 and 7±7, respectively. Limited cutaneous form, positive ANA and anti-Scl70, telangiectases, dysphagia, digital ulcers, lung disease were found in 40 (71%), 48 (83%) and 21 (36%), 45 (78%), 39 (67%), 33 (57%), 23 (40%) in patients, respectively. Of the NVC patterns, early was found in 16 (28%), active was in 11 (19%), late was in 28 (48%) and normal in 3 (5%). The scores of disease activity, severity and GI were shown to be higher in patients with late NVC pattern (table-1). Of the patients with MUST score of >0 (n=10), 9 had late and 1 had early NVC pattern. Conclusions This SSc cohort predominantly had limited cutanous disease and vascular manifestations. In patients with late scleroderma pattern, severity of GI was higher similar to disease activity and severity. Undernutrition was frequently seen in patients with late pattern. NVC might be useful to predict the severity of GI and malnutrition and allow to direct the patients to required procedures earlier. Disclosure of Interest None declared

Background MR Enterography (MRE), a part of the diagnosis in patients with inflammatory bowel disease (IBD), is increasingly used to exclude Crohn9s Disease (CD) in SpA patients with diarrhea. Two important retrospective studies1,2, on IBD suggest that acute and structural findings of sacroiliitis can be evaluated on MRE. But, it needs to be verified whether it really correlates with sacroiliac MRI. Objectives We aimed to determine whether assessment of sacroiliitis on MRE correlates with magnetic resonance imaging (MRI) of sacroiliac (SI) joint. Methods MREs used for screening of IBD in Axial SpA patients with chronic diarrhea and routine semi-coronal SI joint MR images were screened for the presence of acute inflammatory lesions and structural changes of the SI joint by the same radiologist in a blinded fashion to time and diagnosis. Firstly, MRE images and then MR images were evaluated on two separate occasions. Only patients with two imaging modalities with a maximum time distance of a month were evaluated. Results Forty-four patients with MRE imaging were included. Two MRE studies were excluded because of low resolution. Of those 11 patients (26%) had active inflammatory lesions involving mostly both SI joints and 3 had accompanying chronic structural changes. Ten patients (%24) in the MRE group had chronic structural changes, only. In the remaining 20 (47%) MRE evaluated patients SI joint were not affected. Twenty-five axSpA patients had both MRE and SI joint MRIs performed within a month. In 19 cases, out of 25 with both modalities the finding “no sacroiliitis” overlapped. An additional four patients had acute inflammatory lesions on both investigations. In only two patients either MRE or SI joint MRI had acute inflammatory lesions. In general, chronic structural changes overlapped in both modalities as well; Fourteen out of 25 patients with no changes and eight with chronic changes overlapped in both examinations. Both modalities differ in only three patients; Chronic changes was present in two patients in SI joint MRI and one patient in MRE only. Conclusions In SpA patients with chronic diarrhea a present MRE may substitute a conventional semi-coronal MRI of the SI joints and may hence decrease diagnostic expenses. Evaluation of MRE for the acute inflammatory and chronic structural changes of the SI joints may also have a place in the diagnostic flow in IBD patients referred by the gastroenterology clinics, as well. References Leclerc-Jacob, S., et al. The prevalence of inflammatory sacroiliitis assessed on magnetic resonance imaging of inflammatory bowel disease: a retrospective study performed on 186 patients. Alimentary pharmacology & therapeutics, 2014, 39.9: 957–962. Gotler, Jakob, et al. Utilizing MR Enterography for detection of sacroiliitis in patients with inflammatory bowel disease. Journal of Magnetic Resonance Imaging, 2015, 42.1: 121–127. Disclosure of Interest None declared

Background/aim: The aim of this study was to assess the relationships between the course of Behcet's disease (BD), disease-specific fears, and work productivity and activity impairment. Materials and methods: In this cross-sectional study, 110 consecutive BD patients were included. The Work Productivity and Activity Impairment questionnaire was used. Results: In the group of employed patients, 30.41% had missed work during the previous week. The mean percentages of daily activity impairment were higher in patients with musculoskeletal involvement (39.81 +/- 33.61%) compared to those without (23.48 +/- 32.45%) (P = 0.008). A greater decrease in working hours was observed in patients with eye involvement (45.52 +/- 15.29 h) compared to those without (54.15 +/- 15.29 h) (P = 0.007). More of the male patients (67.8%) were afraid of losing their jobs compared to females (30%) (P = 0.000). Conclusion: The highest levels of lost productivity and the most severe effects on daily life are consequences of eye and musculoskeletal involvement in the study population. More effective therapeutic approaches are required to improve the working lives of patients with BD. Moreover, male patients had a higher fear of losing their jobs, suggesting a match between the expected clinical course and the predictions of BD patients.

OBJECTIVE: The aim of this study is to evaluate the demographic characteristics of critically ill patients and to determine intensive care unit (ICU) mortality and its predictors. METHODS: This study was undertaken in the Istanbul Medeniyet University Goztepe Training and Research Hospital Medical ICU. Between May 2012 and January 2013, 111 patients (53 female, 58 male; mean age, 73.79±14.73, mean length of ICU length stay, 9.1±10.7; prevalence of geriatric patients, 77.5%) were admitted to the ICU. The common indications for ICU admission, prevalence of mechanical ventilation support, hematological and biochemical parameters and their effects on mortality were assessed. RESULTS: The common indications for ICU admission were hemodynamic instability (48.6%), respiratory failure (27.9%) and sepsis (15.3%). Hypertension (46.8%) was the most common comorbidity. Prevalance rates of heart failure and diabetes mellitus were 32.4% and 25.2% respectively. Mortality rate was 52.3% in all patients. Approximately 80% of all deaths was observed within the first fifteen-day. In additon, mortality rate (85.7%) was promi nent within patients in need of the mechanical ventilation support. Mechanical ventilation requirement, increased ferritin and vitamin B12 levels were independent risk factors for mortality in critically ill patients (p

WOS: 000380854200011, PubMed ID: 26834222, Objective. To assess the performance of the new 2012 provisional European League Against Rheumatism (EULAR)/ American College of Rheumatology (ACR) polymyalgia rheumatica (PMR) clinical classification criteria in discriminating PMR from other mimicking conditions compared with the previous 5 diagnostic criteria in a multicenter prospective study. Methods. Patients older than 50 years, presenting with new-onset bilateral shoulder pain with elevated acute-phase reactants (APR), were assessed for the fulfillment of the new and old classification/diagnostic criteria sets for PMR. At the end of the 1-year followup, 133 patients were diagnosed with PMR (expert opinion) and 142 with non-PMR conditions [69 rheumatoid arthritis (RA)]. Discriminating capacity, sensitivity, and specificity of the criteria sets were estimated. Results. Discriminating capacity of the new clinical criteria for PMR from non-PMR conditions and RA as estimated by area under the curve (AUC) were good with AUC of 0.736 and 0.781, respectively. The new criteria had a sensitivity of 89.5% and a specificity of 57.7% when tested against all non-PMR cases. When tested against all RA, seropositive RA, seronegative RA, and non-RA control patients, specificity changed to 66.7%, 100%, 20.7%, and 49.3%, respectively. Except for the Bird criteria, the 4 previous criteria had lower sensitivity and higher specificity (ranging from 83%-93%) compared with the new clinical criteria in discriminating PMR from all other controls. Conclusion. The new 2012 EULAR/ ACR clinical classification criteria for PMR is highly sensitive; however, its ability to discriminate PMR from other inflammatory/noninflammatory shoulder conditions, especially from seronegative RA, is not adequate. Imaging and other modifications such as cutoff values for APR might increase the specificity of the criteria.

Objective Deep venous thrombosis (DVT) is the most common form of vascular involvement in Behcet's disease (BD). Chronic post-thrombotic syndrome (PTS) develops in up to one-half of patients with DVT and is associated with impaired quality of life (QoL). There are no data on the severity of DVT-related PTS and its effect on QoL in patients with vascular BD (VBD). We aimed to evaluate PTS and venous disease-specific QoL in patients with VBD. Methods This study included 50 patients (41 men, nine women) with VBD and 31 individuals matched for age and gender (22 men, nine women) with DVT associated with non-BD causes. The Villalta scale was used to assess PTS. The Venous Disability Score and the Venous Clinical Severity Score were used for the assessment of venous disease. Venous disease-specific QoL was measured through Venous Insufficiency Epidemiological and Economic Study Quality of Life/Symptom (VEINES-QoL/Sym) questionnaire. The Behcet Syndrome Activity Score (BSAS) questionnaire was used to assess disease activity. Results We observed a significantly lower risk of PTS together with significantly better VEINES-QoL/Sym, and Venous Disability Score in the VBD group compared with the non-BD group. When VBD patients with PTS were compared with patients without PTS, VEINES-QoL/Sym, and Venous Clinical Severity Score were significantly worse. BSAS was also significantly higher in patients with PTS. An inverse correlation was found between the VEINES-QoL and the BSAS in multivariate analysis. There were no differences between anticoagulant users and nonusers regarding the presence of PTS and scores of all of the venous assessment tools. Conclusions We found lower PTS risk and better venous disease-specific QoL, symptom severity, and venous disability scores in VBD patients compared with the non-BD group. Venous disease-specific QoL negatively correlated with disease activity. Effective control of vascular inflammation through treatment in VBD might explain lower rates of PTS and better venous QoL outcomes in VBD patients. Our results suggest that successful control of BD activity might decrease the development of PTS, improve venous disease-specific QoL, and prevent relapses in VBD.

Background Behcet9s disease (BD) is a systemic disease characterized by oral aphthosis, genital ulcers, ocular lesions and systemic involvement including major vessels. Vascular Behcet9s disease (VBD) is observed in up to 40% of BD. It is one of the major causes ofmorbidity and mortality in BD. Deep venous thrombosis (DVT) is the most common form of vascular involvement in Behcet9s disease (BD). Post-thrombotic syndrome (PTS) develops in up to one-half of patients with DVT and is associated with impaired quality of life (QoL). There is no data on severity of DVT related PTS and its impact on quality of life in patients with Vascular Behcet9s Disease (VBD). Objectives We aimed to evaluate frequency of PTS and venous disease spesific QoL in VBD and to compare it with patients having DVT associated with non-Behcet9s disease (NBD) causes. Methods This study included 50 patients (Male/Female: 41/9) with VBD and 31 age and gender-matched individuals (Male/Female: 22/9), with DVT associated with non-BD causes. Villalta scale was used to assess of PTS. Venous Disability Score (VDS) and Venous Clinical Severity Score (VCSS) were used for the assessment of venous disease. Venous disease-specific QoL was measured through Venous Insufficiency Epidemiological and Economic Study Quality of Life/Symptom questionnaire (VEINES-QoL/Sym). Behcet Syndrom Activity Score (BSAS) questionnaire was used to assess disease activity. Results We observed significantly lower risk of PTS together with significantly better VEINES-QoL, VEINES-Sym and VDS in VBD group compared to NBD group. When VBD patients with PTS were compared to patients without PTS; VEINES-QoL, VEINES-Sym VCSS were significantly worse. BSAS was also significantly higher in patients with PTS. An inverse correlation was found between VEINES-QoL and BSAS in multivariate analysis. There were no differences between anticoagulant users and non-users regarding the presence of PTS and scores of all venous assessment tools. Conclusions We found lower PTS risk and better venous disease spesific QoL, symptom severity and venous disability scores in VBD compared to NBD group. Venous disease spesific QoL negatively correlated with disease activity. Effective control of vascular inflammation through treatment in VBD might explain lower PTS and better venous QoL outcomes in VBD patients. Our results suggest that successful control of disease activity might decrease development of PTS, improve venouse disease spesific QoL as well as preventing the relapses in VBD. Disclosure of Interest None declared

Background Patient global assessment (PtGA) is included in all the current remission definitions for RA despite the fact that PtGA is not exclusively related to disease activity. Objectives In this study in order to objectively identify whether patient9s assessment is better than physician9s (Ph) or not, we compared disease activities assessed with ultrasound (US) of Boolean remission patients with PtGA and with PhGA. Methods RA patients in clinical remission (DAS28 ESR Results A total of 88 out of 389 RA patients (22.6%) in DAS28 remission were enrolled (F/M=55/33, mean age 53.4±12.0, disease duration 10.5±6.3 years, bDMARDs 44.3%, RF/Anti-CCP positivity 72%). Of 88 patients, 33 (37.5%) fulfilled the Boolean remission criteria. When PtGA was substituted with PhGA, Boolean remission rate increased to 61.4% (n=54) ( P grade 1 were taken into account, % of patients without PD, GS or both signals were also similar Table 1). US disease activity parameters of Boolean PtGA and Boolean PhGA remission and their counterpart nonremission patients were compared as well, Boolean remission either with PtGA or PhGA had significantly lower PD scores compared to Boolean nonremission groups with PtGA or PhGA (1 [0–4] vs 3 [1–7], P =0.016 and 1.5 [0–4] vs 4 [0.75–7], P =0.027, respectively). Conclusions RA patients in Boolean remission with PhGA do not have higher disease activity that is verified by US, compared to patients in Boolean remission with PtGA. Furthermore Boolean remission with PhGA differentiates patients with US evinced inflammation, especially PD scores, as good as PtGA. These data suggest that PtGA might be substituted with PhGA in Boolean criteria with higher remission achievement rates. Prospective follow up and recruitment of more patients will clarify the consistency of these results over time and impact of this substitution on functionality. Disclosure of Interest None declared

Background At the present time remission is the target for treatment of rheumatoid arthritis (RA). Various composite indices are available that can be used to define remission. Although the new ACR/EULAR Boolean criteria is more stringent than others, it is difficult to achieve and even more difficult to sustain this Boolean remission. Objectives In this study we aimed to determine the differences of the ultrasonographically (US) assessed actual disease activities of the patients in remission according to different remission criteria. Methods RA patients in clinical remission (DAS28-ESR Results A total of 88 out of 389 RA patients (22.6%) in DAS28 remission were enrolled (F/M=55/33, mean age 53.4±12.0, disease duration 10.5±6.3 years, bDMARDs 44.3%, RF/Anti-CCP positivity 72%). Of the 88 patients, 82 (93.2%), 72 (81.8%), 49 (55.7%), 40 (45.5%) and 33 (37.5%) fulfilled the DAS28-CRP, DAS28-ESR-3, SDAI, CDAI and Boolean remission criteria, respectively. PD synovitis scores which have shown to be associated with erosion development and worse functional outcome were lowest in CDAI, Boolean and SDAI remission. However despite that, those differences in US disease activity parameters (both PD and GS synovitis sum scores and percentages of patients without PD and GS signals [with both omitting and including grade 1 signals]) in remission patients according to different criteria were not statistically significant ( Table 1 ). In comparison of US disease activity parameters of remission vs nonremission groups, only patients with Boolean and CDAI remission had significantly lower PD scores compared to their counterpart nonremission group (for both, 1 [0–4] vs 3 [1–7], P =0.015). Conclusions US verified joint inflammation is the lowest in RA patients who are in remission according to Boolean and CDAI criteria. However, the clinical significance of this statistically nonsignificant inflammatory activity compared to other clinical remission criteria should be assessed in prospective studies. Till then, in clinical practice if Boolean remission could not be achieved, CDAI or SDAI could also be preferred as the remission target. Disclosure of Interest None declared

Background Growing evidence suggests that vitamin D has important immunomodulatory effects and plays key roles in endothelial dysfunction, atherosclerosis and pathogenesis/progression of some of the inflammatory rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus. Although recent data indicated that vitamin D deficiency is quite prevalant and affects endothelial dysfunction in Behcet9s Disease and Takayasu9s arteritis, vitamin D levels in small/ medium vessel vasculitis and its effects on disease course have not been evaluated. Objectives To determine the prevalance of 25-hidroxyvitamin D3 [25(OH)D3] deficiency and to investigate its effects on disease activity in patients with small and/or medium vessel vasculitis. Methods In this cross-sectional study, 25(OH)D3 levels were measured in adult patients with systemic small-medium vessel vasculitis including ANCA-associated vasculitis (AAV), cryoglobulinemic vasculitis (CryV), IgA vasculitis (IgAV) and polyarteritis nodosa (PAN), and sex-matched healthy subjects (HS). All patients9 baseline demographics, disease characteristics, acute phase reactants, medications and disease extents (DEI), disease activities (BVAS) and damage scores (VDI) were determined. 25(OH)D3 level Results Forty five patients (29% new diagnosis) with systemic vasculitis and 63 HS were included ( Table 1 ). The mean 25(OH)D3 level was 21.8±14.3 ng/mL in patients with vasculitis and 56.7±23.9 ng/mL in HS (p Table 1 ). Thirty eight patients (84.4%) were active (BVAS≥1) at the time of recruitment. 25(OH)D3 levels were not different in active (21.6±14.8) and inactive (23.1±12.1) patients (p=0.79). Of the 24 vitamin D deficient patients 23 (95.8%) had renal involvement. Vitamin D deficient patients had slightly higher BVAS (6.5±6.7 vs 3.7±3.7), VDI (3.8±1.4 vs 3.2±1.3) and DEI (3.0±2.8 vs 1.8±2.4) scores at recruitment but the differences were not statistically significant (p>0.05). Likewise there was no difference in acute phase reactants according to vitamin D status. Conclusions Vitamin D insufficiency and deficiency are quite common in patients with small/medium vessel systemic vasculitis. In this preliminary study we could not demonstrate any association with vitamin D deficiency and disease activity in patients with vasculitis. However, prospective followup of a larger sample would better clarify the role of vitamin D on disease activity. Considering also the use of high dose glucocorticoid treatment, these patients should be adequately evalauted and treated for vitamin D deficiency. Disclosure of Interest None declared

Background Accelerated atherosclerosis associated with chronic inflammation is one of the major complications of systemic inflammatory diseases. Takayasu arteritis (TAK) is a rare, systemic large-vessel vasculitis predominantly affecting the aorta and its major branches. The prevalence of atherosclerotic risk factors and cardiovascular (CV) disease in patients with TAK has not been well defined. Objectives The purpose of this study was to assess the frequency of CV risk factors and the incidence of CV events (CVE) in patients with TAK. Methods Patients fullfilling the American College of Rheumatology criteria for TAK from Mayo Clinic, Rochester, USA and Marmara University, Istanbul, Turkey were included in this retrospective cohort study. Data on cardiovascular risk factors at the time of TAK diagnosis and incident CVE during follow-up were abstracted from the medical records. Patients with TAK were compared to age, sex and calendar year–matched controls from the same geographic region without TAK. The 2008 Framingham 10-year general cardiovascular risk score (FRS) was used for the evaluation of CV risk at the time of TAK incidence/index date. For patients without lipid profiles, the office-based FRS, which does not require lipid values, was computed according to previously published algorithms. Results A total of 175 patients with TAK and 175 non-TAK controls were included in the study (115 from Mayo Clinic, 60 from Marmara University for each group). Among patients age ≥30 years at index date (107 TAK,109 non-TAK), complete data to calculate FRS were available in 93 (87%) TAK and 91 (83%) non-TAK subjects. Hypertension diagnosis and lipid lowering treatment were significantly more frequent in the TAK group compared to non-TAK). Prior to the incidence/index date, the occurrence of CVE was significantly higher in the TAK group The overall Framingham 10-year CV risk score was significantly higher in the TAK group compared to non-TAK at incidence/index date (Table 1).)After excluding patients with prevalent CVE, 18 TAK and 3 non-TAK patients developed CVE during a mean follow-up of 7.4 years in TAK and 7.9 years in non-TAK groups. The cumulative incidence of CVE was 16.4% at 10 years in TAK group vs. 6.0% in non-TAK group and the risk of CVE was increased among patients with TAK (hazard ratio: 4.52; 95% CI: 1.29, 15.78 adjusted for age, sex and country). Conclusions CV risk factors are more common in patients with TAK, and the Framingham 10-year cardiovascular risk score is higher in patients with TAK at the time diagnosis compared to non-TAK subjects of similar age and sex. The cumulative incidence of CVE was also significantly higher during follow-up in the TAK group. Patients with TAK should undergo careful assessment of CV risk factors, and an aggressive risk modification approach is warranted. Disclosure of Interest None declared

Background Behcet9s Disease (BD) is a systemic inflammatory disease characterized by involvement of vessels of any size and type. In the course of systemic vasculitis, it is important to distinguish disease activity from disease damage to prevent unnecessary immunosuppression. Additionally damage is an important surrogate for long-term outcome in vasculitis. Although a well-established validated damage index, “vasculitis damage index” (VDI) is available for vasculitis, its performance in determining the damage in BD has never been assessed. Objectives To assess the damage in BD by using VDI, to determine whether VDI covers all BD-associated damage and lastly to develop a preliminary BD-specific damage index with the obtained data. Methods A total of 139 consecutive patients with BD (F/M: 58/81, mean age 38.9±10.9 yrs, disease duration 9.5±7.4 yrs) from a single center were evaluated for damage in a cross-sectional study using the VDI. Considering the patients data and items of VDI with further literature search for BD-specific damages, new items (detailed cardiovascular, ocular, and vascular involvement-, particularly venous disease-related damages) were added to VDI. This modified VDI (Behcet9s Disease-VDI [B-VDI]) was also scored in all patients. Results Seventy seven patients (55.4%) had major organ involvement and 105 (75.5%) patients were on immunosupressive (IS) treatment (Table 1). The mean VDI score was 0.3±0.7 and the majority of the patients had score “0” (“0” in 107 [77%], “1” in 22 [15.8%], “2” in 7 [5%], “3” in 1 [0.7%], and “4” in 2 [1.4%] patients). The mean B-VDI score was 0.6±1.1 (“0” in 92 [66.2%], “1” in 25 [18%], “2” in 13 [9.4%], “3” in 4 [2.9%] and “4” in 3 [2.2%] patients, “5” in 1 [0.7%], and “6” in 1 [0.7%] patient) and was significantly higher than VDI (p Conclusions Both VDI and B-VDI capture reliable data on damage among patients with BD. However, VDI does not cover all vascular involvement-related damages of BD. Therefore addition of new items, especially about vascular damage, to VDI will provide more comprehensive assessment of damage in BD. Further prospective follow-up is required to determine the prognostic value of these damage indices in BD. Disclosure of Interest None declared

Background Antiphospholipid Syndrome (APS) can affect all vessels in the body. Ocular complications are seen most commonly in posterior segment of the eye in these patients. The purpose of this study is to evaluate the posterior segment signs in APS patients and to compare these signs between Primary APS, Secondary APS and Systemic Lupus Erythematosus (SLE) patients without APS. Methods We have included 11 Primary APS, 2 Sjogren9s Syndrome/ 27 SLE patients diagnosed as Secondary APS and 29 SLE patients without APS followed at Marmara University, Division of Rheumatology Outpatient Clinics in this study. All of the patients had a detailed ophthalmological examination, including dilated fundus examination and color fundus imaging. 10 patients with Primary Antiphospholipid Syndrome, 23 patients with Secondary APS and 23 patients with SLE had fundus fluorescein angiography imaging. Results Mean age of the patients was 34.8±12.5 years in Primary APS, 38.3±11.1years in Secondary APS and 38.3±10.9 years in SLE group. In Primary APS 45.5% of patients had venous thrombosis, 45.5% had arterial thrombosis and 72.7% had obstetric complications. In Secondary APS, 44.8% patients had venous thrombosis, 27.6% had arterial thrombosis and 72.4% had obstetric complications. Venous tortuosity was observed in 6 eyes of 3 patients in Primary APS group and 8 eyes of 4 patients in Secondary APS group. One patient with Secondary APS had bilateral branch retinal arterial occlusion. The most common ocular finding of the FFA was pigment epithelial window defect and was observed totally in 19 eyes of 12 patients. There was no statistically significant difference of ocular findings between the Primary APS, Secondary APS and SLE patients. Conclusions In this study, ocular posterior segment findings of Primary APS had been observed less commonly than previous studies. The lower prevalence of ocular complications in this study can be the result of early diagnosis and better control of relapses in these patients. Disclosure of Interest None declared

Objectives To evaluate the diagnostic and discriminative ability of the new 2012 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) polymyalgia rheumatica (PMR) classification criteria compared to previous five diagnostic/classification criteria for PMR in a multi-centre prospective study. Methods Patients older than 50 years of age (n=163), presenting with new onset (symptom duration ≤24 weeks) bilateral shoulder pain with elevated acute phase reactants were enrolled from 15 rheumatology clinics in Turkey. Patients were prospectively followed and the diagnosis of PMR was established when the diagnosis was maintained without an alternative diagnosis at 6 months of follow-up. Those who were diagnosed as other than PMR at the 6th month were designated as control group. All patients were classified by each of the six different criteria for PMR and 2010 ACR/EULAR Rheumatoid arthritis (RA) classification criteria. Results Of the 163 patients with new-onset (mean symptom duration 10.9±7.5 weeks) bilateral shoulder pain 92 (56.4%) patients were diagnosed as PMR and 71 (43.6%) were diagnosed as nonPMR (RA: n=26). The discriminative ability as estimated by the area under the receiver operating characteristic (ROC) curve, was better for the Chuang criteria (0.83) than 2012 EULAR/ACR clinical criteria for PMR (0.69), Jones (0.68), Bird (0.68) and Nobunaga (0.77) criteria (Table 1). The 2012 EULAR/ACR clinical criteria for PMR had a sensitivity of 90.2% and a specificity of 49.3%. Jones and Chuang criteria had the highest specificity (91.5 and 87.3%, respectively). The specificity of the new 2012 EULAR/ACR clinical criteria for PMR slightly increased to 53.8% in RA patients. Although the new 2010 ACR/EULAR RA classification criteria classified only 9 out of 92 PMR patients as RA, the new 2012 EULAR/ACR clinical criteria for PMR classified 12 out of 26 RA patients as PMR. Conclusions The new 2012 EULAR/ACR clinical classification criteria for PMR can classify PMR patients with high sensitivity, however, its ability to discriminate PMR from other inflammatory conditions with shoulder pain, especially RA is poor. Other criteria sets, Jones or Nabunaga criteria, despite involvement of similar clinical parameters, perform better in discriminating PMR from RA and other inflammatory/noninflammatory articular diseases. Our results, therefore, suggest that in seronegative patients with bilateral shoulder pain and acute-phase response, differential diagnosis of PMR and RA may require imaging (as proposed also in the other version of the new criteria) or biomarker studies. Disclosure of Interest None declared

Background Vascular involvement especially in young males and is one of the major causes of mortality and morbidity. Lower extremity deep vein thrombosis (DVT) is the most frequent form of vascular involvement. Chronic post-thrombotic syndrome develops in up to one-half of patients with deep venous thrombosis. Patients with post-thrombotic syndrome have substantially impaired quality of life. Objectives There is no data about post-thrombotic syndrome in deep venous thrombosis associated with Behcet9s Disease. In this study,we aimed to evaluate venous disease spesific quality of life in patients with Vascular Behcet9s Disease (VBD). Methods In this study, 62 patients (Male/Female: 47/15, mean age: 38.6±9.2 years) with VBD, who were regularly followed in Marmara University Behcet clinic, 29 patients (Male/Female: 20/9, mean age: 41.6±11.8 years) with DVT associated with non-BD reasons, who were followed in Hematology and Vascular Surgery Department, were assessed. Venous Disability Score (VDS) and Venous Clinical Severity Score (VCSS) were used to evaluate the severity of venous insufficiency. Venous disease-specific quality of life (the primary outcome) was measured using the Turkish validated form of Venous Insufficiency Epidemiological and Economic Study Quality of Life/Symptom questionnaire (VEINES-QoL/Sym). After calculation, VEINES-QoL and VEINES-Sym sub-score were identified. Higher score shows better QoL. Short Form (SF-36) and Behcet Syndrom Activity Score (BSAS) questionnaire were also fullfilled to patients. Results Physical and mental component of SF-36 were similar between VBD and non-BD group. VCSS and VDS were significantly worse in non-BD group compared to VBD. VEINES-QoL and VEINES-Sym were significantly better in VBD group (Table 1). Mean duration after DVT was 6.7±5 years in VBD, 1.6±1 years in non-BD group. There was no correlation between VEINES-QoL, VEINES-Sym score and duration after DVT, daily sitting and standing time. But VEINES-QoL and VEINES-Sym negatively correlated with VCSS (respectively; r: -0.367, p Conclusions In our study, we found that venous spesific QoL and symptoms worsen together with increasing VDS and VCSS score. These results suggest that controlling of disease activity increase the OoL as well as preventing the relapses. Disclosure of Interest None declared

Background Colchicine is the mainstay of Familial Mediterranean Fever (FMF) treatment that reduces the frequency of attacks and prevents amyloidosis in the majority of patients. Colchicine, despite its narrow therapeutic window, is a well-tolerated oral drug that is rapidly absorbed by the gastrointestinal tract. However not all patients respond to colchicine and the response is not universal at the same doses. Therefore some patients require higher doses of colchicine to suppress their attacks. Objectives To investigate the relationship between colchicine plasma level and clinical response to colchicine in FMF patients. Methods Forty FMF patients with normal renal and hepatic functions, receiving colchicine regularly (steady state dose for at least 3 months with good -compliance) were enrolled to study in a 6 month-period. Blood samples were collected 30 minutes before the next colchicine dose. Colchicine plasma levels have been measured by using the device consecutive high performance liquid chromatography-mass spectrometry (LCMS-MS). Internal standard was added to plasma and the solution was extracted with ether:dichlormethane. Organic phase was evaporated to dryness under nitrogen. The residue dissolved in methanol was injected into the system. Quantitation was based on monitoring precursor ion and product ion for colchicine m/z 400.2 >310.3, and for internal standart colchicine- d3 m/ z 403.3>359.2. The assay was linear for colchicine over the range of 0.25-8 ng/ ml, r 2 is 0.997. Results Of the 40 patients, 24 were receiving 1.5 mg/day and 16 were receiving 2 mg/day. The mean plasma colchicine concentration of the entire cohort was 1.097±0.42 ng/mL and was within the therapeutic range (0.5-3 ng/mL). The mean plasma colchicine concentrations of patients who were on 1.5 mg/day (1.05 ng/mL) and 2 mg/day (1.17 ng/mL) colchicine treatment were not significantly different ( P =0.40). Plasma colchicine concentrations were positively correlated with daily oral colchicine dose. Before colchicine treatment, the mean attack number was 24.9±3.2/year and t after colchicine treatment was 1.6±0.4/ year ( P P =0.43). Optimal clinical response (decrease in the frequency of attacks) was found at oral doses of 1.5-2 mg/day, corresponding to plasma levels of approximately 1.097±0.42 ng/ml. Conclusions These data indicate that colchicine plasma levels correlate with daily oral doses of colchicine. FMF patients receiving 1.5 mg/day and 2 mg/day colchicine doses have similar plasma colchicine concentrations and also similar decrease in attack frequency. To evaluate the role of colchicine metabolism and plasma concentrations on the different dose requirements for optimal suppresion of attacks and on the colchicine-refractory FMF, further data is currently being studied by our group. Disclosure of Interest None declared

Background Interleukin-17 (IL-17) has been associated with the pathogenesis of numerous autoimmune/inflammatory diseases. The aim of our study was to investigate the expression of Th17-related immunity in two phenotypically different disorders: Behcet9s disease (BD) with innate and Takayasu9s arteritis (TAK) with adaptive immune responses. Methods Peripheral blood mononuclear cells (PBMC) from 32 patients (age: 39.2±10.1 years) with BD, 13 patients (age: 50.9±15.5 years) with TAK and 18 HC (age: 40.9±6.9 years) were cultured in Th17 inducing condition (IL-6, PHA, IL-1beta and IL-23) for 6 days. Cultured cells were stained with CD4, CD8, CD3, TCRgamma/delta, CD19, IFN-g and IL-17 antibodies to determine the intracellular cytokine secretion by flow cytometry. Results In BD patients, IL-17 expression by CD4 T cells was observed to be higher than both TAK patients and HC (p=0.03 and p=0.02, respectively). IL-17 expression by gamma delta and CD8 T cells was also higher in BD compared to HC (p=0.03, p Conclusions Our preliminary results suggest that under Th17 stimulating condition, T cells express higher IL-17 levels in BD. More prominent IL-17 and IFN-g production by all lymphocyte subsets in BD might be associated with the increased innate responses and early tissue neutrophil infiltrations in BD, which is not observed in TAK. Disclosure of Interest None declared