A. Omma

Metabolic syndrome is not only a risk factor for cardiovascular diseases in systemic lupus erythematosus but is also associated with cumulative organ damage: a cross-sectional analysis of 311 patients.

Background/Purpose Patients with systemic lupus erythematosus (SLE) have increased rates of cardiovascular disease (CVD) that are one of the major causes of mortality. The aim of this study was to determine the frequencies of metabolic syndrome (MetS) and CVD in SLE patients and investigate the link between these and clinical features of SLE. Methods A total of 311 SLE patients were consecutively assessed for cumulative organ damage (SDI/SLICC scores), history of CVD and MetS as defined by the National Cholesterol Educational Program Adult Treatment Panel III (NCEP ATP III). Clinical data of SLE patients were collected from the records. Results The mean age of the patients was 40.2u2009±u200913.4 years and 89% were female. The frequencies of CVD and MetS were 15.2% and 19%, respectively. In this SLE cohort increased age, cumulative damage, disease duration and CVD were associated with MetS. CVD was associated with disease duration, cumulative damage, pericarditis, hematologic involvement, lymphopenia, thrombocytopenia, neurological involvement and antiphospholipid antibody (aPL) positivity. Hydroxychloroquine (HCQ) use was found as a protective factor for CVD. Conclusion In SLE patients, MetS was associated with CVD and both increased with disease duration. Patients who developed MetS and/or CVD had increased cumulative organ damage. Certain clinical features of SLE and the presence of aPL were also associated with CVD. There was a significant protective effect of HCQ from CVD. The prevention of MetS and long-term use of HCQ may be beneficial in improving the prognosis of SLE.

A Nationwide Experience With The Off‐label Use of Interleukin‐1 Targeting Treatment in Familial Mediterranean Fever Patients

Approximately 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.

Can the Thiol/Disulfide Imbalance Be a Predictor of Colchicine Resistance in Familial Mediterranean Fever?

Familial Mediterranean fever (FMF) is a chronic autoinflammatory condition characterized by fever attacks and recurrent polyserositis. Subclinical inflammation that persists during attack-free periods can result in oxidative stress (OS) damage. Thiol groups bind to reactive oxygen radicals and protect cells and tissues from OS damage. The aim of this study was to investigate the relationship between thiol-disulfide balance and colchicine resistance in FMF patients during an attack or attack-free period. A newly developed spectrophotometric method was used to measure native thiol (NT) and disulfide (DS) levels in FMF patients and an age-sex matched group of healthy controls. NT and DS levels were compared in FMF patients 1) with vs. without colchicine resistance; and 2) during an attack (FMF-AP) vs. attack-free period (FMF-AFP). A total of 118 FMF patients and 60 healthy controls were studied. NT (P < 0.001) and total thiol (TT) (P < 0.001) levels in FMF patients were significantly lower compared to healthy controls. NT (P = 0.030) and TT (P = 0.010) levels of FMF-AP patients were significantly lower than that of FMF-AFP patients. FMF-AP patients had significantly higher DS levels than FMF-AFP patients (P = 0.039). Compared to FMF patients without colchicine resistance, elevated levels of DS (P = 0.019) but not NT (P = 0.620) and TT (P = 0.718) were found in those with colchicine resistance. Thiol-disulfide homeostasis is altered in FMF patients during an attack period and this imbalance may be associated with colchicine resistance.

THU0203 Cross-sectional assessment of damage in takayasu arteritis with a validated tool

Background Takayasu arteritis (TA) is a large vessel vasculitis with a chronic course, usually causing damage by the time of diagnosis. To our knowledge, there is no reported study assessing the damage related to TA itself or treatment. Objectives We aimed to evaluate the damage cross-sectionally in TA patients with Vasculitis Damage Index (VDI), a generic tool developed for systemic vasculitides. Methods A collection of 103 TA patients (92 female) fulfilling ACR criteria and followed-up more than 6 mo from 4 centers in Turkey, were enrolled into the study. All patients underwent detailed examination including eye, vascular imaging, echocardiography and bone dansitometry. Clinical, angiographical and treatment characteristics and damage items of VDI were recorded using a standardized protocol. Disease activity and quality of life (QoL) were evaluated by Kerr criteria and SF-36, respectively. TA patients with persistant disease activity ≥6 mo were considered as resistant. The correlation between VDI scores and disease duration, cumulative glucocorticoid (GC), cyclophosphamide (CYC) duration and doses, and mental (MCS) and physical (PCS) component summary scores of SF-36 were analysed by Pearson correlation test. VDI scores according to the disease resistance and poor QoL (MCS and PCS scores <50) were compared by Mann Whitney U test. Results The mean age, follow-up time and disease duration were 40±12 years, 71±68 mo and 98±92 mo, respectively. Type I (45%) was the most common type of vascular involvement. Cumulative doses/duration of GC and CYC were 12±11g/70±65 mo and 2,4±6,5g/2,6±7,1 mo, respectively; 40% of them had resistant course. Major vessel stenosis, absent pulses, claudication and hypertension were demonstrated in ≥50% of TA patients, as damage items. Osteoporosis (26%) and cataract (15%) were the main treatment related damages. Cumulative VDI scores in TA cohort were found to be 4.7±2.2, mainly (4.1±1.8) due to disease itself. MCS and PCS scores were calculated as 44±10 and 39±12, respectively. Poor SF-36 MCS scores were demonstrated in 66% and PCS scores in 79% of the patients. VDI scores were found to be correlated with disease duration (p<0.01, r=0.44), cumulative doses of GC (p<0.01, r=0.26) and CYC (p=0.02, r=0.21) and duration of GC (p<0,01 r=0.35). A negative correlation was observed between the VDI and both MCS (p=0,002, r=-0,29) and PCS scores (p<0.001, r=-0,37). The higher VDI scores were detected in the subgroup of patients PCS <50 (5,1±2,1 vs 3±1,5, p<0,01) and resistant disease activity (5,4±2,3 vs 4,2±2, p=0,02). Conclusions Cross-sectional analysis of this TA cohort with a long disease duration revealed vascular damage scores comparable to the scores of severe systemic necrotizing vasculitides. Majority of TA patients had disease related damage, characterized by peripheral vascular involvement. The longer disease duration and higher GC and CYC exposure were significantly associated with the damage. The severe damage scores (≥5) were observed in TA patients with resistant disease and poor health quality. Disclosure of Interest None Declared

Decreased Chitotriosidase Activity and Levels in Familial Mediterranean Fever

Different studies have demonstrated changes in chitotriosidase (ChT) activity and concentrations in multiple diseases. However, changes in ChT activity and concentrations have not been concurrently evaluated in patients with Familial Mediterranean Fever (FMF). In this study, we analyzed the changes in serum ChT activity and concentrations in patients with FMF. The study included a total of 80 patients with FMF and 80 healthy controls. ChT enzyme activity and concentrations were measured and then compared between the groups. ChT activity was measured by using fluorometric ELISA and ChT concentrations were measured by using colorimetric ELISA methods. The median ChT activity was 10.00 (6.00–15.00) nmol/mL/hr in the patients and 14.00 (6.25–20.75) nmol/mL/hr in the controls. There was a statistically significant difference in the ChT activity between the controls and patients (P = 0.027). The median ChT concentrations were 65.40 (46.20–84.92) pg/mL and 125.00 (75.72–143.95) pg/mL in the patients and controls, respectively (P < 0.001), which were expressed as median percentiles (25th–75th). Additionally, we found no correlation between C-reactive protein and ChT activity (P = 0.978, r = 0.003) and concentrations (P = 0.446, r = −0.87). Serum ChT enzyme activity and concentrations may not be considered as a biomarker in FMF patients taking colchicine. New studies are needed to evaluate the changes of enzyme activity and concentration in colchicine-negative patients.

Compliance to colchicine treatment and disease activity in Familial Mediterranean Fever (FMF) patients in Middle/Black Sea Region of Turkey (in Corum region).

Background and question Colchicine is the gold standard treatment for prevention of inflammatory attacks and prevention of reactive amyloidosis in FMF. However, noncompliance to colchicine treatment is common among FMF patients. On the other hand, the disease may not be controlled in some patients despite use of full dose colchicine. In this study, we aimed to investigate the rates of disease control, compliance to colchicine treatment, and need for an additional treatment despite use of full dose colchicine in patients with FMF in Corum region where FMF is common in Turkey.

SAT0353 Geographical differences in psoriatic arthritis: a transatlantic comparison

Background The environmental and genetic factors play a crucial role in the pathogenesis of psoriatic arthritis (PsA) which may cause a difference in disease characteristics for patients from different geographical regions. Objectives The aim of the study was to explore the disease characteristics, treatment choices and comorbidities in patients with PsA in different countries to see the impact of geographic factors. Methods PsArt-ID (Psoriatic Arthritis- International Database) is a prospective, multicentre registry in PsA, which was initially developed in Turkey in 2014, with participation of Canada since 2015 and Italy since 2017. Patients with PsA are consecutively registered to this registry with the aim of investigating the real-life data. Patient characteristics across Turkey (n=1283) and Canada (n=119) are compared for this analysis.Abstract SAT0353 – Table 1 The demographics and clinical characteristics in two countries TURKEY CANADA p value Female* 827/1283 (64.5) 60/119 (50.4) 0.002 Age (years) 47 (36–56.7) 49 (34–61) <0.001 BMI (kg/m2) 27.47 (24.5–31.2) 29 (23.7–33.5) 0.013 At onset age for PsA 36 (29–49.7) 39 (30–48) 0.058 Smoking (package/years) 10 (3–19.7) 14.5 (5–26.25) 0.007 Education years 8 (5–12) 15 (13–16) <0.001 SJC 2 (1–5) 2 (1–7) 0.461 TJC 4 (2–8) 6.5 (2–17) 0.340 TEP 2 (1–2) 1 (1–2) 0.021 BSA 5 (1–13.75) 1 (0–5) <0.001 BASDAI 37 (20–54) 38 (22–58) 0.027 Pt GA 45 (20–60) 31 (12–70) <0.001 PGA 30 (20–50) 34 (18–66) <0.001 Pain VAS 40 (20–60) 33 (18–78) <0.001 TJC: tender joint counts; TEP: tender entheseal points; BSA: body surface area; PtGA: patient global activity; PGA: physician global activity. All data were given n/total n (percentage (%))* or median (first-third percentiles).Abstract SAT0353 – Figure 1 The distribution of the treatment choices in Turkey and Canada, excluding patients with new diagnosis at the time of recruitment. DMARD: Disease-modifying anti-rheumatic drug; anti-TNF: anti-tumour necrosis factor. All data were given n/total n (percentage (%). Results Canadian patients were older at the time of recruitment (Table). They also were more frequently smokers, had higher duration of education and higher BMI than patients in Turkey. Patients in Canada had more frequent polyarthritis (66.7% vs 39.6%, p<0.001), DIP joint disease (34.2% vs 16%, p<0.001), dactylitis (38.1% vs 29%, p=0.037) nail involvement (55.9% vs 45.7%, p=0.008) and higher number deformed joints (29.3% vs 20.7%, p=0.035) whereas Turkish patients had oligoarthritis more often (37.6% vs 24.8%, p=0.016). For disease activity, tender and swollen joint counts were similar for whereas the skin activity was higher in Turkish patients. There were no major differences between countries regarding treatment choices with similar frequencies of patients on biologic therapies (34.5% vs 30.2%, p=0.339) (figure 1). Although the numbers were very low, there was more frequent cancer in Canada than Turkey (4.3% vs 1.4%, p=0.022) whereas all the other comorbidities were similar. Conclusions Geographical differences have impacts on the disease features in PsA, which may be due to genetic, environmental and cultural differences. The treatments are comparable suggesting a similar approach by the physicians. Disclosure of Interest None declared

FRI0513 The impact of depression, anxiety and fatigue in patients with behÇet’s disease

Background Behçet’s disease (BD) is a type of systemic vasculitis and inflammatory disease with unknown etiology which is associated with fatigue and lower quality of life (QoL).1 Objectives In this study we aimed to assess the relationship between BDCAF and BDQoL, depression, anxiety and fatigue in Behçet’s disease. Methods This is a cross-sectional study of 155 Behcet’s syndrome (BS) patients and 107 healthy controls in Turkey. All subjects completed the Multidimensional Assessment of Fatigue (MAF) questionnaire, Hospital Anxiety and Depression (HADS) scale. Disease activity among BS patients was assessed using the Behçet’s Disease Current Activity Form (BDCAF), and the physician’s global assessment (PGA). And BD patients completed the Behçet’s Disease Quality of Life (BDQoL) questionnaire. Results There was no significant difference with age and gender between the groups. BS patients had significantly higher HADS-anxiety (HADS-A), HADS-depression (HADS-D) and MAF scores than the healthy controls (p<0.05) (table 1). BS patients with active disease had significantly higher MAF and HADS-A scores compared to inactive BS patients (p<0.05). MAF scores showed positive correlations with HADS-A, HADS-D, BDCAF and BDQoL (table 2).Abstract FRI0513 – Table 1 Comparison of BS patients and healhy controls Patients Healty controls p BDCAF 2.7±1.6 - BDQoL 9.4±9.2 - HADS-Anxiety 67 (%43.2) 11 (%10.3) 0.001 HADS-AnxietyScore 8 (0–21) 6 (0–13) 0.004 HADS-Depression 63 (%40.6) 23 (%21.5) 0.001 HADS-Depression Score 5 (0–20) 4 (0–10) 0.340 MAF 25.0 (6.5–48) 19.2 (7.3–44.2) 0.000Abstract FRI0513 – Table 2 Corelations between MAF score and BDCAF, BDQoL, HADS-A, HADS-D in BS patients Parametre MAF score R p HADSD 0459 <0001 HADSA 0548 <0001 BDQoL 0572 <0001 BDCAF 0359 <0001 DrVAS 0285 <0001 Patient VAS 0434 <0001 Age 0119 0141 Disease duration 0070 0384 Conclusions Fatigue and anxiety is common in clinically active BS patients compared with healthy controls and inactive BD patients. Reference [1] Tascilar NF, Tekin NS, Ankarali H, et al. Sleep disorders in Behcet’s disease, and their relationship with fatigue and quality of life. J Sleep Res2012;21(3):281–288. Disclosure of Interest None declared

SAT0670 Dynamic thiol/disulfide homeostasis as a novel oxidative marker in behÇet’s disease

Background Behçet’s disease (BD) is a relapsing systemic inflammatory disorder of unknown etiology.1 Objectives In this study, we aimed to evaluate the relationship between the thiol-disulfide balance and disease activity and organ involvement in BD. Methods One hundred fifty (150) patients with BD and 100 age-gender matched healty controls were included in the study. Disease activity was assessed with the BD Current Activity Form (BDCAF) score. Serum levels of native thiol (NT), total thiol (TT), and disulfide were measured and the disulfide/native thiol, disulfide/total thiol and native thiol/total thiol levels were calculated for the patient and control groups. Results NT, TT, NT/TT values of the BD patients were significantly lower than those of the control group. The disulfide/NT, disulfide/TT values of BD patients were higher compared to the control group and the disulfide value of the BD group was slightly higher compared to the control group (table 1). No correlation was determined between thiol levels and disease activity and organ involvement in BD.Abstract SAT0670 – Table 1 Laboratory results of the BD and the control group BD HC p Native Thiol (SH)(µmol/l) 357.93±76.65 453.01±60.8 0.000* Total Thiol (µmol/l) 398.57±78.33 490.29±58.48 0.000* Disulfide (µmol/l) (SS) 20.32±8.19 18.64±7.87 0.109 Disulfide/native thiol x100 5.93±2.76 4.25±1.99 0.000* Disulfide/total thiol x100 5.2±2.13 3.86±1.69 0.000* Native thiol/total thiol x100 89.6±4.26 92.28±3.38 0.000* *p<0,05 Conclusions In patients with Behcet’s disease, the dynamic thiol-disulfide homeostasis balance shifted towards disulfide formation due to thiol oxidation. It may be used as a novel marker in BD because it is easy, practical, fully automated and relatively inexpensive. Acknowledgements The authors thank staff of Ankara Numune Training and Research Hospital, Department of Rheumatology for their generous friendly assistance in every step of this study. Disclosure of Interest None declared

Vitamin D status, serum lipid concentrations, and vitamin D receptor (VDR) gene polymorphisms in Familial Mediterranean fever

Vitamin D (VitD) is critical for the regulation of inflammatory processes, and VitD deficiency has been linked to several chronic inflammatory disorders. We aimed to investigate the concentrations of serum 25(OH)D3, lipid parameters, and three known VDR polymorphisms (BsmI, FokI, and TaqI) in patients with Familial Mediterranean fever (FMF), an autosomal recessive autoinflammatory disease. The study included 123 FMF patients and 105 controls. Seventy patients had no attack (group 1), 30 had 1-2 attacks (group 2), and 23 had 3 or more attacks (group 3) within last three months. Serum 25(OH)D3 concentrations were determined using liquid chromatography-tandem mass spectrometry. BsmI, FokI, and TaqI polymorphisms were analyzed by a competitive allele specific polymerase chain reaction assay (KASPar). Serum lipid parameters were measured with enzymatic colorimetric methods. 25(OH)D3 concentrations were lower in FMF patients compared to controls (p < 0.001). No difference was observed in 25(OH)D3 concentration between groups 1, 2, and 3. The distributions of FokI and TaqI genotypes were not significantly different between FMF patients and controls. There was a significant difference in the distribution of AA BsmI genotype between male FMF patients and male controls. Increased concentrations of triglycerides (p = 0.012) and decreased concentrations of high-density lipoprotein cholesterol [HDL-C] (p = 0.006) were found in FMF patients compared to controls. Although lower 25(OH)D3 concentrations were observed in FMF patients versus controls, no association was determined between FMF attack frequency and 25(OH)D3 concentrations. We showed that the AA genotype of BsmI polymorphism is associated with FMF in males but not in females. The effects of decreased HDL-C and increased triglyceride concentrations on cardiovascular events in FMF patients should be further investigated.