E. Gonullu

Amyloidosis and its related factors in Turkish patients with familial Mediterranean fever: a multicentre study

OBJECTIVE The primary aim of this study was to investigate the prevalence of amyloidosis and its related factors in a large number of FMF patients. METHODS Fifteen centres from the different geographical regions of Turkey were included in the study. Detailed demographic and medical data based on a structured questionnaire and medical records were collected. The diagnosis of amyloidosis was based on histological proof of congophilic fibrillar deposits in tissue biopsy specimens. RESULTS There were 2246 FMF patients. The male/female ratio was 0.87 (1049/1197). The mean age of the patients was 34.5 years (S.D. 11.9). Peritonitis was the most frequent clinical finding and it was present in 94.6% of patients. Genetic testing was available in 1719 patients (76.5%). The most frequently observed genotype was homozygous M694V mutation, which was present in 413 (24%) patients. Amyloidosis was present in 193 patients (8.6%). Male sex, arthritis, delay in diagnosis, M694V genotype, patients with end-stage renal disease (ESRD) and family history of amyloidosis and ESRD were significantly more prevalent in patients with amyloidosis compared with the amyloidosis-negative subjects. Patients with homozygous M694V mutations had a 6-fold higher risk of amyloidosis compared with the other genotypes (95% CI 4.29, 8.7, P < 0.001). CONCLUSION In this nationwide study we found that 8.6% of our FMF patients had amyloidosis and homozygosity for M694V was the most common mutation in these patients. The latter finding confirms the association of homozygous M694V mutation with amyloidosis in Turkish FMF patients.

Response rate of initial conventional treatments, disease course, and related factors of patients with adult-onset Still's disease: Data from a large multicenter cohort.

BACKGROUND Adult-onset Stills disease (AOSD) is a rare condition, and treatment choices are frequently dependent on expert opinions. The objectives of the present study were to assess treatment modalities, disease course, and the factors influencing the outcome of patients with AOSD. METHODS A multicenter study was used to reach sufficient patient numbers. The diagnosis of AOSD was based on the Yamaguchi criteria. The data collected included patient age, gender, age at the time of diagnosis, delay time for the diagnosis, typical AOSD rash, arthralgia, arthritis, myalgia, sore throat, lymphadenopathy, hepatomegaly, splenomegaly, pleuritis, pericarditis, and other rare findings. The laboratory findings of the patients were also recorded. The drugs initiated after the establishment of a diagnosis and the induction of remission with the first treatment was recorded. Disease patterns and related factors were also investigated. A multivariate analysis was performed to assess the factors related to remission. RESULTS The initial data of 356 patients (210 females; 59%) from 19 centers were evaluated. The median age at onset was 32 (16-88) years, and the median follow-up time was 22 months (0-180). Fever (95.8%), arthralgia (94.9%), typical AOSD rash (66.9%), arthritis (64.6%), sore throat (63.5%), and myalgia (52.8%) were the most frequent clinical features. It was found that 254 of the 306 patients (83.0%) displayed remission with the initial treatment, including corticosteroids plus methotrexate with or without other disease-modifying antirheumatic drugs. The multivariate analysis revealed that the male sex, delayed diagnosis of more than 6 months, failure to achieve remission with initial treatment, and arthritis involving wrist/elbow joints were related to the chronic disease course. CONCLUSION Induction of remission with initial treatment was achieved in the majority of AOSD patients. Failure to achieve remission with initial treatment as well as a delayed diagnosis implicated a chronic disease course in AOSD.

The role of methotrexate and low‐dose prednisolone on adiponectine levels and insulin resistance in patients with rheumatoid arthritis naïve to disease‐modifying antirheumatic drugs

Insulin resistance (IR) plays an important role in the development of cardiovascular events in rheumatoid arthritis (RA) patients. Adiponectin influences insulin sensitivity but its impact on IR in RA patients remains unclear. The present study aims to investigate the role of methotrexate (MTX) and low doses of prednisolone (LDP) on IR and adiponectin levels in RA patients who are naïve to disease‐modifying antirheumatic drugs (DMARDs), as well as determining the relationship between disease activity, acute phase response, IR and adiponectin levels in patients with RA.

Werner’s syndrome may be lost in the shadow of the scleroderma

We describe three patients with Werner’s syndrome (WS), two of whom had been mistakenly diagnosed as having scleroderma. We would like to discuss briefly the importance of differentiation of these two disorders from each other.

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

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

AB0827 Hydroxychloroquine Does not Increase Psoriasis in Psoriatic Arthritis: Time on Drug Analysis Based on Real Life Data

Background Antimalarials have been reported to worsen preexisting psoriasis, therefore is commonly avoided by the clinicians in the treatment of psoriatic arthritis (PsA). With the lack of any solid evidence, this precludes the potential use of hydroxychloroquine (HQ) as a part of the treatment. Objectives We aimed to analyze PsA patients who are treated with HQ, identify the time on drug and reasons of discontinuation of HQ. 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 disease characteristics. From the registry, patients who have ever used HQ were identified including the time on HQ, whether they still continue to treatment, and the reasons of discontinuation in case of a withdrawal. Results Until December 2014, 746 patients were recruited. 114 patients (15.3%) were either currently using or have been used HQ previously. 51.8% of these patients were currently using HQ (fig 1). The distribution of joint patterns were 50.9% polyarthritis, 30.7% oligoarthritis, 2.6% monoarthritis, 13.2% distal interphalangeal joint involvement, 26.3% axial disease, including patients who had combinations of different patterns. Joint patterns were not different among patients who had ever used HQ or not. The duration of HQ treatment was 41.4 (SD:38.4) months among current users vs 45.6 (SD:51.2) months in withdrawers. The reason of discontinuity could be identified in 44/55 among non-users. Within these 44 patients the most frequent reason was inefficacy (n=25) and incompliance of the patient (n=7). Retinopathy was observed in 5 patients. Liver function test abnormalities, pregnancy, hyperpigmentation of the skin, step down for being in remission and nausea were observed in 1 case each and were reported to be the reason of stopping the treatment. There were only 2 cases who had to discontinue treatment because of an increase in psoriatic lesions. One of these patients used HQ for one month and the other patient for 21 months. Conclusions These data show that only 2/114 patients had an increase in psoriatic lesions in PsA in a mean duration of 3.5 years due to the HQ treatment suggesting that HQ is a safe treatment choice in PsA. The efficacy and added benefits of HQ in terms of reducing cardiovascular risk factors cannot be enclosed with this registry. Disclosure of Interest None declared

P01-007 – Evaluation of potential risk factors of Amyloidosis

Familial Mediterranean Fever (FMF) is a genetic disease which is frequently seen in Middle East Region. The most common reason of morbidity and mortality is the end stage renal failure due to amyloidosis.