Ö. Bayındır

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.

The Psoriatic Arthritis Registry of Turkey: results of a multicentre registry on 1081 patients.

Objective. The aim was to assess the characteristics of PsA, find out how well the disease is controlled in real life, demonstrate the treatments and identify the unmet needs. Methods. The PsA registry of Turkey is a multicentre Web-based registry established in 2014 and including 32 rheumatology centres. Detailed data regarding demographics for skin and joint disease, disease activity assessments and treatment choices were collected. Results. One thousand and eighty-one patients (64.7% women) with a mean (S.D.) PsA duration of 5.8 (6.7) years were enrolled. The most frequent type of PsA was polyarticular [437 (40.5%)], followed by oligoarticular [407 (37.7%)] and axial disease [372 (34.4%)]. The mean (S.D.) swollen and tender joint counts were 1.7 (3) and 3.6 (4.8), respectively. Of these patients, 38.6% were on conventional synthetic DMARD monotherapy, 7.1% were on anti-TNF monotherapy, and 22.5% were using anti-TNF plus conventional synthetic DMARD combinations. According to DAS28, 86 (12.4%) patients had high and 105 (15.2%) had moderate disease activity. Low disease activity was achieved in 317 (45.7%) patients, and 185 (26.7%) were in remission. Minimal disease activity data could be calculated in 247 patients, 105 of whom (42.5%) had minimal disease activity. The major differences among sexes were that women were older and had less frequent axial disease, more fatigue, higher HAQ scores and less remission. Conclusion. The PsA registry of Turkey had similarities with previously published registries, supporting its external validity. The finding that women had more fatigue and worse functioning as well as the high percentage of active disease state highlight the unmet need in treatment of PsA.

Ulnar motor study to first dorsal interosseous: Best reference electrode position and normative data

Introduction: Reference electrode position affects nerve conduction study results. This study was undertaken to determine the optimal reference electrode position for ulnar motor recording from the first dorsal interosseous (FDI) muscle and to develop normative data. Methods: Fifty‐one subjects were tested using reference electrode positions on the thumb, index, and little fingers. Latencies were compared with a needle recording from the FDI. Analysis was performed to determine the surface placement that most closely matched the needle recording latency. A normative database was then derived on 100 healthy subjects. Results: Placing the reference electrode on the thumb yielded results closest to the “gold standard” needle recording latency. The 97th percentile (upper limit of normal) for latency was 4.0 ms. The 3rd percentile values (lower limit of normal) for amplitude were 9.0 mV for men and 9.3 mV for women. Conclusions: The reference position on the thumb yields latencies that most closely approximate needle recording. Normative data are presented. Muscle Nerve 52: 231–233, 2015

Axial psoriatic arthritis: the impact of underdiagnosed disease on outcomes in real life

Psoriatic arthritis (PsA) may affect different joints, including the spine. The prevalence of spinal involvement is variable depending on the definition and a subset of patients have been identified in cohorts that do not have clinical features of axial disease and yet have imaging findings. Still, there is not a consensus on how and when to screen axial disease. In this study, we aimed to investigate factors associated with being underdiagnosed for axial psoriatic arthritis (axPsA) and its impacts on outcomes. Disease features and outcomes of axPsA according to the physician (n = 415) were compared with patients with imaging findings only (sacroiliitis fulfilling the modified New York criteria, n = 112), using data from a real-life PsA registry. Patients with imaging findings only were more frequently women (83/220 (37.7%) vs 29/122 (23.8%); p = 0.008). This group also had higher peripheral disease activity (imaging only vs clinical AxPsA: mean (SD) tender joint count 5.3 (6.1) vs 3.3 (4.7), swollen joint count 1.9 (2.9) vs 1.2 (2.4); p < 0.001 for both comparisons) and was less often treated using TNF inhibitors (16.1 vs 38.2%; p < 0.001) than patients who were classified as axPsA. Patient-reported outcomes were similar in both groups. PsA patients, especially women with more severe peripheral disease, have a higher risk of being underdiagnosed for axPsA. The severity of peripheral symptoms may be a risk factor to mask the spinal features of PsA.

SAT0526 Is relapse rate of giant cell arteritis in real-life experience lower than in the controlled trials? results of a retrospective, multi-centre cohort study

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

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

THU0329 Budd-chiari syndrome in behÇet's disease: a retrospective multicenter study

Background The aim of this study was to determine the demographic, clinical, laboratory and management characteristics along with the clinical course of Budd-Chiari syndrome (BCS) associated with Behçets disease (BD). Methods Sixty patients with BD with BCS (40 male, 20 female) were identified in 23 rheumatology centers (Group I). A total of 169 consecutive patients (100 male, 69 female) with BD who did not have clinically apparent BCS during the follow-up were evaluated as the control group (Group II). Results Comparison of the demographic and clinical findings between the Group I and the Group II were as follows: The mean age of disease onset was 23.1 +/- 6.7 years vs. 26.8±0.6 years (p=0.013), mean age at diagnosis was 27.2±0.9 vs. 30.4±0.6 years (p=0.008), arthritis was 10% vs. 28.4% (p=0.002), papulopustular skin lesion was 48.3% vs 69.2% (p=0.003), central nervous system (CNS) involvement 10% vs. 3% (p=0.03), cardiac involvement was 16.7% vs. 2.4% (p<0.001), superficial thrombophlebitis was 23.3% vs. 4.7% (p<0.001), and deep vein thrombosis was 58.3% vs. 15.4% (p<0.01). On diagnosis 50% of BD patients with BCS were classified as Child-Pugh A. Inferior vena cava obstruction was observed in 38.3% and portal vein thrombosis was seen in 3.3% of the patients with BCS. Mortality in BCS patients with BD was 18.3%. BCS related treatment after diagnosis in patients with BD were as follows: 71.7% of patients were treated with monthly cyclophosphamide intravenous pulses, 53.3% received intravenous pulse corticosteroids, 55.9% used azathioprine, 54.2% had warfarine treatment, and 50.8% were treated with low molecular weight heparin. Conclusions This study shows a higher frequency of cardiac and CNS involvement, superficial thrombophlebitis, papulopustular skin lesion, deep vein thrombosis in BD patients with BCS. Arthritis was observed less common in BD patients with BCS. The mean age onset was lower in patients with BCS. Medical treatment with immunosuppressive agents and anticoagulation appears to be the treatment of choice in BD patients with BCS. The majority of the patients with BCS were Child–Pugh class A on diagnosis. The inferior vena cava is frequently involved and, often associated with deep vein thrombosis and cardiac involvement. Disclosure of Interest None declared

FRI0265 Validation of New 2012 EULAR/ACR Clinical Classification Criteria for Polymyalgia Rheumatica: Comparison with the Previous Criteria in a Prospective Multi-Center Study

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

SAT0582 Psoriasis Symptom Inventory is a Valid Patient-Reported Instrument for the Assessment of Skin Severityin Psoriatic Arthritis

Background Skin involvement in psoriatic arthritis (PsA) has significant impacts on health-related quality of lives in addition to the joint involvement. Due to this impact, its important to assess the severity of psoriasis to fully capture disease activity in PsA. In this study we aimed to test the validity of “Psoriasis symptom inventory” (PSI) in a cohort of patients with PsA. Methods PsART (Psoriatic Arthritis Registry of Turkey) is a prospective, multicentre, nationwide study in Turkey on patients with PsA. Patients are consecutively recruited to this registry, if they are diagnosed as PsA, regardless of any other disease characteristics. In this registry, PSI data was available in 237 patients. For the PSI the following items were scored on a scale between 0-4, by the patient: itching, redness, scaling, burning, stinging, cracking,flaking and pain. The PSI score was calculated as a sum of these items and ranged between 0-32. The correlations between PSI and other outcome measures were investigated. Results The mean (SD) age and BMI were 44.7 (12.3) and 28.7 (5.6), respectively. Sixty-six percent of the patients were female. The duration of psoriasis in this group was 167.3 (124.2) months. Mean (SD) PSI scores were 6.7 (6.7) with a range of 0-32. PSI scores were found to be significantly correlated to patient (R=0.338) and physician global assessments (R:0.342), fatique (R=0.226), pain (R=0.334) (p<0.001 for all comparisons) and HAQ (R=0.198; p=0.007). PSI was also correlated to body surface area involved, measured by the physician (R=0.256, p=0.07). Conclusions PSI has a good construct validity in comparison to other clinical assessment tools. Due to its high feasibility, PSI can be a useful tool to investigate and record the severity of psoriasis in PsA in clinical practice. Disclosure of Interest None declared

AB0829 Demographics of Patients with New-Onset Psoriatic Arthritis: Real Life Data from an Inception Cohort: Table 1.

Background Psoriatic arthritis is a complex disease with a wide range of manifestations. In this inception cohort of PsA patients we aimed to identify the initial manifestations as well as disease activity characteristics and compare with an unselected group of patients with longstanding disease. Methods PsART (Psoriatic Arthritis Registry of Turkey) is a prospective, multicentre study in Turkey on patients with PsA. Patients are consecutively recruited to this registry. Patients who have been newly diagnosed with PsA have been identified, and their characteristics were compared with longstanding disease. Results Within 746 patients recruited, 111 (14.9%) had a new diagnosis for PsA. Patients in the inception cohort were significantly younger than the rest of the cohort (p=0.03) (table). Females were 53.2% of the inception and 66.8% of the non-inception cohort (p=0.007). For types of joint patterns, 14.4% of the inception cohort had only axial involvement whereas this was seen in 8.2% for the other group (p=0.05). The other joint patterns were comparable among groups. Both groups had similar tender and swollen joint counts, BASDAI and BASFI. Patient (p=0.002) and physician global assessments (p<0.001), fatigue (p=0.02), duration of morning stiffness (p=0.02) and CRP (p=0.05) were higher in the inception cohort. Female patients had higher patient global assessment and fatigue scores despite similar physician global assessment, swollen and tender joint counts, BASDAI, BASFI and CRP with males in non-inception cohort. For the inception cohort, there were no differences between the 2 genders.Table 1. Disease characteristics of the groups Inception cohort Non-inception cohort Whole group Females Males Whole group Females Males n 111 59 52 635 424 211 age 42.5 (12.3) 44.4 (12.5) 40.4 (11.7) 45.5 (12.9) 46.7 (13) 43.1 (12.4) SJC 2.7 (3.4) 2.5 (3.2) 3 (3.6) 1.6 (2.8) 1.5 (2.8) 1.6 (2.7) TJC 4.1 (4) 3.8 (3.7) 4.5 (4.2) 3.4 (4.7) 3.5 (4.8) 3.4 (4.2) BASDAI* 4.5 (2.4) 4.3 (2.2) 4.8 (2.5) 3.7 (3.8) 3.8 (4.3) 3.5 (2.6) BASFI* 3.2 (2.4) 3 (2.3) 3.4 (2.4) 2.8 (2.7) 2.7 (2.7) 2.8 (2.7) Patient global* 4.9 (2.5) 4.7 (2.3) 5.1 (2.6) 3.9 (2.3) 4 (2.3) 3.5 (2.4) Physician global* 4.2 (1.9) 4 (1.6) 4.4 (2.1) 3.1 (2.1) 3.1 (2) 3 (2.2) fatigue* 4.9 (2.5) 5 (2.3) 4.9 (2.7) 4.2 (2.6) 4.5 (2.5) 3.7 (2.8) CRP (mg/lt) 13. 4 (23.4) 9.5 (16.5) 18 (29.1) 8.7 (16.2) 8.6 (16.1) 9 (16.5)* Data given on a scale between 0–10. There were missing data. The number of cases with available information are: BASDAI: 434, BASFI: 413; patient global assessment: 493; physician global assessment 440, fatigue: 506, CRP: 717, tender joint count: 677, swollen joint count: 672. Conclusions More patients present with sole axial involvement at the beginning, which may explain the higher disease activity agreed by the patient and the physician despite similar tender and swollen joint counts. Females may have a different perception of the disease with worse patient global assessments and fatigue in longstanding disease. Disclosure of Interest None declared