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Background/aim: to evaluate treatment adherence and predictors of drug discontinuation among patients with inflammatory arthritis receiving bDMARDs within the first 100 days after the announcement of the COVID-19 pandemic. Materials and methods: a total of 1871 patients recorded in TReasure registry for whom advanced therapy was prescribed for rheumatoid arthritis (RA) or spondyloarthritis (SpA) within the 3 months (6-9 months for rituximab) before the declaration of COVID-19 pandemic were evaluated, and 1394 (74.5%) responded to the phone survey. Patients' data regarding demographic, clinical characteristics and disease activity before the pandemic were recorded. The patients were inquired about the diagnosis of COVID-19, the rate of continuation on bDMARDs, the reasons for treatment discontinuation, if any, and the current general disease activity (visual analog scale, [VAS]). Results: a total of 1394 patients (493 RA [47.3% on anti-TNF] patients and 901 SpA [90.0% on anti-TNF] patients) were included in the study. Overall, 2.8% of the patients had symptoms suggesting COVID-19, and 2 (0.15%) patients had PCR-confirmed COVID-19. Overall, 18.1% of all patients (13.8% of the RA and 20.5% of the SpA; p = 0.003) discontinued their bDMARDs. In the SpA group, the patients who discontinued bDMARDs were younger (40 [21-73] vs. 44 years [20-79]; p = 0.005) and had higher general disease activity; however, no difference was relevant for RA patients. Conclusion: although the COVID-19 was quite uncommon in the first 100 days of the pandemic, nearly one-fifth of the patients discontinued bDMARDs within this period. The long-term effects of the pandemic should be monitored., Hacettepe Rheumatology Society

This study aimed to compare Tuberculin Skin Test (TST) and QuantiFERON (R)-TB Gold In-Tube (QFT-GIT) test in rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients scheduled for biological and targeted synthetic disease modifying anti-rheumatic drugs (DMARDs) in a Bacillus Calmette-Guerin-vaccinated population. Adult RA (n = 206) and SpA (n = 392) patients from the TReasure database who had both TST and QFT-GIT prior to initiation of biological and targeted synthetic DMARDs were included in the study. Demographic and disease characteristics along with pre-biologic DMARD and steroid use were recorded. The distribution of TST and performance with respect to QFT-GIT were compared between RA and SpA groups. Pre-biologic conventional DMARD and steroid use was higher in the RA group. TST positivity rates were 44.2% in RA and 69.1% in SpA for a 5 mm cutoff (p < 0.001). Only 8.9% and 15% of the patients with RA and SpA, respectively, tested positive by QFT-GIT. The two tests poorly agreed in both groups at a TST cutoff of 5 mm and increasing the TST cutoff only slightly increased the agreement. Among age, sex, education and smoking status, pre-biologic steroid and conventional DMARD use, disease group, and QFT-GIT positivity, which were associated with a 5 mm or higher TST, only disease group (SpA) and QFT-GIT positivity remained significant in multiple logistic regression. TST positivity was more pronounced in SpA compared to that in RA and this was not explainable by pre-biologic DMARD and steroid use. The agreement of TST with QFT-GIT was poor in both groups. Using a 5 mm TST cutoff for both diseases could result in overestimating LTBI in SpA.

OBJECTIVES: To analyse the clinical and laboratory factors associated with bamboo spine. METHODS: Data of patients fulfilling the 2009 ASAS classification criteria for axial spondyloarthritis, registered in the national, multicentre, longitudinal, and observational database of TReasure was analysed. Radiographs were assessed using the Bath Ankylosing Spondylitis Radiologic Index (BASRI). Data of patients with a bamboo spine (Group 1) was compared to data derived from patients with a longstanding disease of at least 15 years but no syndesmophytes (Group 2). RESULTS: Out of the 5060 patients, 1246 had eligible radiographs. There were 111 patients (8.9%) with a bamboo spine. Male sex was more common among patients with bamboo spine. The median BMI of 27.7 (25.8-31.1) in Group1 was higher than the BMI of 25.9 (22.9-29.2) in Group 2 (p

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.

Dear Editor, After the coronavirus disease 2019 (COVID-19) pandemic affected the whole world, rheumatologists began to think about how COVID-19 will progress in patients with inflammatory conditions. High cytokine levels play a role in the pathophysiology of COVID-19 infection. Tumor necrosis factor alpha (TNF-α) is a proinflammatory cytokine known to have a key role in the pathogenesis of chronic immune-mediated diseases. AntiTNF therapy may cause an increase in active tuberculosis, other granulomatous diseases, and serious infections [1]. According to many studies, rheumatological diseases have not been identified as a risk factor for severe COVID-19 infection [2]. Should significantly increased cytokine levels during COVID-19 infection make us consider anticytokine therapies that may be used in the treatment of patients with COVID-19 a risk? We aimed to explore whether the frequency of COVID-19 infection increased, the effect of comorbidities on the frequency of infection, and whether the severity of the disease and need for intensive care support increased in patients who used anti-TNF agents. We performed a retrospective case-control study between March and December 2020 in Sakarya University Training and Research Hospital. Retrospectively, we evaluated whether there was a difference in the frequency and severity of COVID-19 in our patients diagnosed with ankylosing spondylitis (AS), 77 of whom were using anti-TNF and 49 of whom didn't use anti-TNF. Hospitalization and intensive care unit (ICU) requirements were evaluated as endpoints. In the anti-TNF group, patients used adalimumab, etanercept, certolizumab, infliximab, and golimumab. Patients were questioned at an outpatient clinic in person or by phone. Seventy-seven patients with AS using anti-TNF agents (58 males, 19 females) and 49 patients with AS (38 males, 11 females) not using anti-TNF agents were included in the study (p = 0.943). Mean age of patients using antiTNF agents was 41.53 ± 10.38, and mean age of patients not using anti-TNF agents was 42.94 ± 10.86 (p = 0.468). Thirty-three (42.9%) patients were smokers in the antiTNF group, while 23 (46.9%) patients were smokers in the group not using TNFi (p = 0.791). There was 12 pack-year smoking in the anti-TNF group, and 14 pack-year smoking in not using TNFi (p = 0.623). The frequency of diabetes mellitus (DM), hypertension (HT), amiloidosis, familial mediterranean fever (FMF), coronary artery disease (CAD), chronic obstructive pulmonary disease (COPD) was similar in both groups (p = 0.403, p = 0.999, p = 0.521, p = 0.999, p = 0.999, respectively). Six patients using TNFi and 3 patients not using TNFi recovered from COVID-19 infection. However, this result was not statistically significant (p = 0.999). One patient using anti-TNF was hospitalized but with no need for admission to the ICU (p = 0.999). All 9 patients recovering from COVID-19 were male (p = 0.113). There were 2 (22.2%) smokers in the SARS-CoV-2 positive group and 54 (46.2%) smokers in SARS-CoV-2 negative group (p = 0.297). There was 37.5 pack-year smoking in SARS-CoV-2 positive group, and 12 pack-year smoking in SARS-CoV-2 negative group (p = 0.151). Nobody has comorbidities (DM, HT, amiloidosis, FMF, CAD, COPD) in SARS-CoV-2 positive group. There were patients with DM (5.1%), HT (15.4%), amiloidosis (1.7%), FMF (1.7%), CAD (0.9%) and COPD (0.9%) in SARS-CoV-2 negative group (p = 0.999, p = 0.356, p = 0.999, p = 0.999, p = 0.999, p = 0.999, respectively). Having comorbidities was not detected to be associated with frequency of COVID-19. 31 (40.3%) patients were using adalimumab, 25 (32.5%) patients were using etanercept, 13 patients were using (16.9%) certolizumab, 6 (7.8%) patients were using golimumab, and 2 patients (2.6%) were using infliximab in TNF group. Six patients using anti-TNF (2 adalimumab, 1 etanercept, 1 golimumab,2 infliximab) and 3 nonuser patients recovered from COVID-19 (p = 0.999). No statistically significant difference was found between SARS-CoV-2 positive and negative patients in terms of the types of anti TNF they used. Patients were called in March 2020, and they were advised to terminate their anti-TNF therapy, when the COVID-19 pandemic began. Among those who used antiTNF, 2 (33.3%) people who had COVID-19 and 38 (53.5%) people who did not have COVID-19 interrupted treatment (p = 0.419). Anti-TNF users who did not have COVID-19 stopped taking the treatment for an average of 3 months (min 2-max 4 months) starting from March 2020, and the patients who had COVID-19 (p = 0.102) stopped taking the treatment for 1.5 months (min 1-max 2 months). Duration of interrupting TNFi was not significant for the risk of COVID-19. Comorbidities, older age, and the presence of active disease have been associated with worse outcomes in previous studies [3]. In our study, the anti-TNF using and the nonuser groups were similar according to age, sex, and comorbidities. Although comorbidities in COVID-19 are associated with severe disease in the literature, we did not find a significant difference in our study. This result is probably related to our insufficient number of patients. As a result, we found that the use of anti-TNF did not increase the frequency and severity of COVID-19. In a recently published multicenter study, it was stated that the use of biological DMARDs in patients with inflammatory rheumatic diseases was not significantly associated with a worse outcome of COVID-19. But unlike our study, having no comorbidities was associated with a decreased risk of a worse outcome [4]. There are currently studies investigating the therapeutic utility of infliximab and adalimumab in hospitalized COVID-19 patients [5]. The results of these studies are very important. The usability of TNFi in treatment and at which stage of the disease anti-TNF agents can be used are wondered. We will see the course of the disease all over the world after the administration of the COVID-19 vaccines, but we still need more information about effective and safe treatment.The authors declare that there is no conflict of interest.The authors did not receive support from any organization for this work.

WOS: 000463722100007, PubMed ID: 30489254, Objective: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. Most of the identified disease-causing mutations are located on exon 10. As the number of studies about the effect of the exonal location of the mutation and its phenotypic expression is limited, we aimed to investigate whether the exonic location of the Mediterranean fever (MEFV) mutation has an effect on the clinical manifestation in patients with FMF. Methods: Study population was derived from the main FMF registry that included 2246 patients from 15 different rheumatology clinics. We categorized the mutations according to their exon locations and retrieved the clinical and demographic information from the database. Results: Patients having the MEFV mutations on exon 2 or 10 (n: 1526) were divided into three subgroups according to the location of the MEFV mutations: Group 1 (exon 2 mutations), Group 2 (exon 10 mutations), and Group 3 (both exon 2 and exon 10 mutations). Group 2 patients were of a significantly younger age at onset, and erysipel-like erythema, arthritis, amyloidosis, and a family history of FMF were more common in this group. Conclusion: Patients with FMF and exon 10 mutations show more severe clinical symptoms and outcome. Exon 2 mutations tend to have a better outcome.

Eritema Nodozum (EN), palpasyonda ağrılı ve çoğunlukla bacak ve ayak bileklerinin ekstansör yüzlerinde lokalize olan ani gelişen eritematöz, nodül veya plaklarla karakterize pannikülitin en yaygın şeklidir. Nodüller tipik olarak 1-3 cm çapındadır. EN'nin %55 kadarı idiyopatik olarak kabul edilirken, en yaygın nedenler enfeksiyonlar, ilaçlar, sarkoidoz ve inflamatuvar barsak hastalığı gibi sistemik hastalıklar, hamilelik ve malignitedir. Bu çalışmada, COVID-19'un klinik bulgusu olarak bir eritema nodozum olgusunu sunuyoruz ve 77 yaşındaki bir kadında hastalığın seyrini tanımlıyoruz.

Background: Several societies published recommendations for latent tuberculosis infection (LTBI) screening before biological and targeted synthetic disease-modifying anti-rheumatic drugs (DMARDs) in rheumatoid arthritis (RA) but not for other inflammatory arthritides such as spondyloarthritis (SpA). Using RA guidelines could result in overestimating Tuberculin Skin Test (TST) positivity in SpA. This study aimed to compare the distribution of TST results in SpA and RA patients along with comparison in terms of QuantiFERON®-TB Gold In-Tube (QFT-GIT) test in a Bacillus Calmette-Guérin-vaccinated population.Methods: Adult RA (n=206) and SpA (n=392) patients from the TReasure database who had both TST and QFT-GIT prior to initiation of biological and targeted synthetic DMARDs were included in the study. Demographic and disease characteristics along with pre-biologic DMARD and steroid use were recorded. The distribution of TST and performance with respect to QFT-GIT were compared between RA and SpA groups.Results: Pre-biologic conventional DMARD and steroid use was higher in the RA group. TST positivity rates were 44.2% in RA and 69.1% in SpA for a 5 mm cut-off (pConclusions: TST positivity was more pronounced in SpA compared to that in RA and this was not explainable by pre-biologic DMARD and steroid use. The agreement of TST with QFT-GIT was poor in both groups. Using a 5 mm TST cut-off for both diseases could result in overestimating LTBI in SpA.

Moxifloxacin is an antibiotic of quinolone group and its usage frequency for inpatients increases day by day. Reported side effects for this antibiotic include nausea, vomiting, stomachache, headache, hypoglycemia or hyperglycemia, anemia, arthralgia, myalgia and tendon rupture. Solely, side effects regarding electrolyte imbalance are rarely reported. Since an infiltration in the right basal region of the lung was found on chest X-ray, prophylactic moxifloxacin was started. The plasma sodium (Na) level of the patient decreased to 129 (135-145) mEq/L on the fifth day of the treatment while the potassium (K) level increased to 5,8 mmol/L on the fifth day of the treatment. When blood and urine culture test results came out as negative, moxifloxacin treatment was discontinued on its 5th day. Three days after the discontinuation, Na and K levels reached to normal levels. This situation made us think that hyperkalemia and hyponatremia occurred as a side effect of moxifloxacin treatment. If hyponatremia and/or hyperkalemia occurs during treatment of moxifloxacin, adverse effects must be thought and the treatment must be discontinued immediately.

Objectives: This study aims to determine femoral cartilage thickness using ultrasonography in familial Mediterranean fever (FMF). Patients and methods: A total of 45 patients (16 males, 29 females; mean age: 38.5±9.1 years; range, 24 to 49 years) with the diagnosis of FMF and 31 healthy individuals (6 males, 25 females; mean age: 37.0±8.7 years; range, 25 to 47 years) between January 2016 and July 2016 were included in this study. Clinical data and demographic characteristics of the patients were recorded. All FMF cases in the study were in remission with colchicine treatment. The thickness of femoral cartilage in both knees were evaluated using ultrasonography. Three measurements (mid-point) were taken from both knees (at the medial/lateral femoral condyles and intercondylar area). Results: Ultrasonographical measurements revealed that cartilage measurements of FMF patients were significantly thinner at both the medial/lateral femoral condyles and intercondylar area on the right knee and at the medial/lateral femoral condyles on the left knee (p Conclusion: Our study showed decreased femoral cartilage thickness in FMF patients. These findings indicate that even if these patients do not have an attack, they may have subacute and chronic arthritis in their joints, and their femoral cartilage thickness can be affected.

WOS: 000505281300007, PubMed: 30680951, Objective Psoriatic arthritis (PsA) has a genetic background. Approximately 40% of patients with psoriasis or PsA have a family history of psoriasis or PsA, which may affect disease features. The aim of this study was to assess the effects of family history of psoriasis and PsA on disease phenotypes. Methods Data from 1,393 patients recruited in the longitudinal, multicenter Psoriatic Arthritis International Database were analyzed. The effects of family history of psoriasis and/or PsA on characteristics of psoriasis and PsA were investigated using logistic regression. Results A total of 444 patients (31.9%) had a family history of psoriasis and/or PsA. These patients were more frequently women, had earlier onset of psoriasis, more frequent nail disease, enthesitis, and deformities, and less frequently achieved minimal disease activity. Among 444 patients, 335 only had psoriasis in their family, 74 had PsA, and 35 patients were not certain about having PsA and psoriasis in their family, so they were excluded from further analysis. In the multivariate analysis, family history of psoriasis was associated with younger age at onset of psoriasis (odds ratio [OR] 0.976) and presence of enthesitis (OR 1.931), whereas family history of PsA was associated with lower risk of plaque psoriasis (OR 0.417) and higher risk of deformities (OR 2.557). Family history of PsA versus psoriasis showed increased risk of deformities (OR 2.143) and lower risk of plaque psoriasis (OR 0.324). Conclusion Family history of psoriasis and PsA impacts skin phenotypes, musculoskeletal features, and disease severity. The link between family history of psoriasis/PsA and pustular/plaque phenotypes may point to a different genetic background and pathogenic mechanisms in these subsets., Turkish Society for Rheumatology; Scientific and Technological Research Council of TurkeyTurkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK), Supported by the Turkish Society for Rheumatology. Dr. Bakirci's work was supported by the Scientific and Technological Research Council of Turkey and the Turkish Society for Rheumatology.

Background:Axial spondyloarthritis (axSpA) is a chronic inflammatory rheumatic disease mainly affecting sacroiliac joints and spine. Peripheral arthritis, dactylitis and enthesitis may also occur. Extra musculoskeletal manifestations (EMMs; uveitis [AAU], inflammatory bowel disease [IBD] and psoriasis [Pso] are among the most common ones) are important features and might have an impact on the disease burden in patients with axSpA. The presence of EMM, in particular IBD and AAU could influence the choice of TNFi however little is known regarding the role of peripheral manifestations together with the EMM on the prescribing patterns in axSpA patients.Objectives:To examine the frequency of peripheral and EMMs in a real-world axSpA cohort and their effect on the choice of first advanced treatment.Methods:In total 1687 axSpA patients (58% male and the mean age (±SD) was 38.5 ± 10.9) who initiated his/her first biologic were included in the present analysis. The data for the current study was obtained from the TReasure web-based registry; in which RA and SpA patients treated with bDMARDs from different regions of Turkey. Baseline demographic, disease related characteristics, peripheral and EMMs were extracted. Characteristics of patients with and without peripheral/extra-musculoskelatal involvement were compared as well as factors/covariates associated with the choice of first TNFi and secukinumab was analysed.Results:Enthesis (28.2%) was found the most common peripheral manifestations and peripheral arthritis (26.4%) and hip arthritis (24.4%) followed it. Symptom duration to the first advanced treatment initiation was significantly shorter in axSpA patients with peripheral arthritis, enthesitis, dactylitis and psoriasis and longer in hip arthritis and AAU. HLA-B27 positivity was significantly lower in patients with arthritis, psoriasis and IBD and higher with hip arthritis and AAU. In multivariate analysis the presence of IBD is significantly associated with the preference of monoclonal TNFi (mab) over etanercept (ETA) (OR 5,770; 95%CI 1.788-18.616). However ETA was preferred in patients with hip arthritis (p=0.003), longer symptom duration (p=0.049), and using sulfasalazine (p=0.043). In comparison with mabs, secukinumab (SEC) prescription was found to be significantly associated with higher age (p=0.001), sulfasalazin (p=0.001) and methotrexate usage (p=0.053) among axSpA patients need their first advanced treatment.Conclusion:The results of the current study confirm the pathophsyologic associations of peripheral involvement and EMM in axSpA patients. Apart from hip arthritis the presence of IBD has an impact on the prescription of advanced treatment in real-life.Table 1.Clinical characteristics of patients in cohortAll patients(n=1678)Peripheral arthritis(n=445)Dactilitis(n=81)Enthesis(n=476)Uveitis(n=193)Psoriazis(n=152)IBD(n=78)Hip involvemet(n=412)Age, mean±SD38,5±10,938,3±11,637,4±11,137,9±10,741,3±11,439,9±11,341,6±12,239,2±11,2Male sex,n (%)974 (57,7)184 (41,3)34 (42)238 (50)96 (49,7)54 (35,5)43 (55,1)272 (66)Symptom duration, mean month±SD108,5±98,996,9±92,979,1±76,5100,4±92,7144,7±110,287,7±9494,5±98133,3±108,2HLA B27 positivity, n (%)621 (53,7)142 (46,3)27 (51,9)174 (49,4)104 (77)34 (36,2)16 (27,1)186 (59,8)Concomitant cDMARD usage (yes), n (%)420 (24,9)170 (38,2)39 (48,1)133 (27,9)53 (27,5)58 (38,2)24 (30,8)99 (24)BASDAİ,mean±SD5,1±2,55,1±35,3±3,15,3±2,94,7±2,55,6±2,44,8±2,35,3±2,1ASDAS-CRP, mean±SD3,1±1,52,6±1,92,5±1,82,8±1,72,9±1,73,4±1,33,1±1,53,7±1,4Disclosure of Interests:None declared

Background Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease. Disease-specific symptoms are fever and polyserositis attacks (1). The most important complication in the long term is amyloidosis (2). Many cytokines and acute phase reactants have been studied in patients with familial Mediterranean fever. Erythrocyte sedimentation rate (ESR), c-reactive protein (CRP), serum amyloid A (SAA) and fibrinogen levels increase during attack and return to normal values after the attack (3,4). Biomarkers that are useful on the course of the disease and the development of amyloidosis are still needed. Objectives In this study, we aimed to observe the changes reflected in the hemogram parameter values during the remission and attack periods of FMF patients. Methods Study included 67 patients who met the FMF Tel-Hashomer criteria. The patients with available results of both remission and attack periods were included in the study.Since the distribution of all continuous variables used in the study as a statistical method was not suitable for normal distribution, Wilcoxon T test was used to investigate the difference in hematological variables during remission and attack periods Results 21 of the patients were male and 46 were female. The mean age of the male patients was 41.57 ± 11.08 and the female patients were 35.84 ± 11.75. The mean age of all patients was 38.18 ± 11.20 years. There was a statistically significant difference between neutrophils in the comparisons of hematological variables between remission and episode periods of familial Mediterranean Fever patients (p Conclusion Our study does not support that neutrophil/lymphocyte ratio, MPV and PDW values may be helpful in the attack. Significant differences in neutrophil counts suggest that further studies are needed to show neutrophil cell morphology, number, and function in FMF patients. References [1] Ozen S, Batu ED, Demir S. Familial Mediterranean Fever: Recent Developments in Pathogenesis and New Recommendations for Management.Front Immunol. 2017 23;8:253. [2] Sonmez HE, Batu ED, Ozen S. Familial Mediterranean fever: current perspectives.J Inflamm Res. 2016 17;9:13-20. [3] Ozer S, Yilmaz R, Sonmezgoz E, Karaaslan E, TaSkin S, Butun I, Demir O. Simple markers for subclinical inflammation in patients with Familial Mediterranean Fever.Med Sci Monit. 2015 23;21:298-303. [4] Tunca M, Kirkali G, Soyturk M, et al. Acute phase response and evolution of familial Mediterranean fever. Lancet. 1999;353:1415. Disclosure of Interests None declared

Background Familial Mediterranean fever (FMF) is known the most common monogenic autoinflammatory disease. Its prevalence is reported high from the eastern Mediterranean areas (1) The disease is characterized by episodes of fever, serositis, arthritis,renal complications and other different clinical manifestations (2) Objectives Here, we aimed to present our data of our 139 FMF patients for demonstrating the demographic and clinical features of the study group,from North-western part of Turkey. Methods A total of 139 FMF patients who were diagnosed and treated in the department of Internal medicine/Rheumatology, Sakarya University (North-western area of Turkey) were included in the study and the demographic and clinical characteristics of the patients were examined. Results The mean age of the patients was 39.02 ± 11.3. Male gender was 42 (30.2%) and female gender was 97 (69.8%).107 (77%) of patients had fever and 32 (23%) had no history of fever. 127 (91.4%) patients complained about peritonitis, 27 (%19.4) patients had pleuritic pain, 19 (13.7%) patients had erysipelas like erythema and 53 (38.1%) patients had arthritis attack. 34 (24.5%) patients also had sacroiliitis. The ratio of resistance of treatment response to colchicine drugs that can be available in Turkey (Colchicum dispert®), 6 (4.3%) was determined. Interestingly these patients responded to the colchicine drugs available from some other countries from Europe (as Colchicine-opocalcium® and Colchicina-lirca®) None of our patients needed anti-IL1 therapies. The rate of amyloidosis was 5 (3.6%). Conclusion FMF is a disease with high morbidity and mortality, 95.7% of the patients in our region have response to colchicine drugs which is available in our country. The remaining patients have also response to colchicine available from some other countries. None of our patients had anti-IL1 therapies. References [1] ozen S, Batu ED, Demir S. Familial Mediterranean Fever: Recent Developments in Pathogenesis and New Recommendations for Management. Front Immunol. 2017 23;8:253. [2] Alghamdi M. Familial Mediterranean fever, review of the literatureClin Rheumatol. 2017;36:1707-1713. Disclosure of Interests None declared

Eriskin still hastaligi (ESH), bilinmeyen nedenlere bagli gelisen multisistemik inflamatuvar bir hastaliktir. Ates, artrit ve govdede tipik olarak gorulen somon renkli dokuntulerle karakterizedir. Tani genellikle benzer bulgularla seyreden hastaliklarin dislanmasina ek olarak klinik ve laboratuvar bulgularinin birlikteligi ile konur. Farenjit, lenfadenopati ve lokositoz da dahil olmak uzere nonspesifik hematolojik bulgular mevcut olabilir. Reaktif trombositoz ESH’da beklenen bir durumken 1 milyonu gecen trombosit yuksekligi ise nadir gorulen bir laboratuvar bulgusudur. Bu olguda eriskin still hastaligi ve eslik eden belirgin reaktif trombositoz ile takip ettigimiz 33 yasinda bir erkek hasta sunulmaktadir

Rheumatoid arthritis (RA) is a chronic, autoimmune inflammatory disorder which can also affect cardiovascular system. Cardiovascular system involvement can be seen in many forms such as pericarditis, myocarditis, coronary artery disease or heart failure. Diastolic dysfunction is especially important for preserved ejection fraction heart failure patients. With this study we aim to investigate echocardiographic diastolic dysfunction parameters in RA patients.77 RA patients and 76 control subjects without coronary artery disease, heart failure, valvular heart diseases were included for the study and echocardiography was performed. Interventricular septum (p=0.041), posterior wall diameter (p=0.008), left ventricular mass index (p=0.037) values were significantly higher and E wave (p=0.000), E/A (p=0.000), e’ (p=0.018) values were significantly lower in RA patients compared to control group. In conclusion echocardiographic diastolic dysfunction parameters were declined in RA patients.

Background:Although psoriatic arthritis (PsA) may be seen at any decades, juvenil onset PsA is relatively rare. Moreover, there were no more data about clinical features, treatments, and course in juvenile PsA when they reached to adult age.Objectives:The objective of this study was to assess and compare demographic and clinical features for juvenile onset PsA and adult onset PsA.Methods:PsART-ID is a multicenter, international database, investigating the disease characteristic in real life (1). Briefly, demographic data, PsA subtypes, uveitis, enthesitis, dactylitis, Co-morbidities, disease activity scores (TJC, SJC, VAS-pain, VAS patients and physician global assessments, VAS-fatigue, BASDAI), and functional status (HAQ-DI, BASFI) were recorded. Psoriasis and PsA starting age were noted, as well. Patients were classified as juvenile PsA or juvenile PsO (under 18 years old). Results were compared regarding to juvenile versus adult onset age.Results:Overall, 1644 PsA patients were included to study, 301/1644 (18.3%) patients had juvenile onset psoriasis. Of 39/1644 (2.4%) patients had juvenile onset PsA, as well. As expected, juvenile onset PsA patients were younger, however PsA disease duration were longer than adult onset PsA patients. There were no any difference between demographic and clinical data, except BMI and enthesitis were less frequently at the juvenile onset PsA groups. Although, ever csDMARD using were similar between two groups, however, juvenile onset PsA patients were used more frequently bDMARDs.Table.Comparison of demographic and clinical characteristics of juvenile and adult-onset psoriatic arthritisJuvenile onsetAdult onsetpN (%)39 (2.4)1605 (97.6)Female Sex n (%)24 (61.5)1006 (62.7)0.884PsA beginning age mean (SD)13.3 ± 3.8542.3 ± 12.9Current age mean (SD)26.6 ±10.747.3 ±13.07Duration of psoriasis (years)17.10 ± 11.2614.75 ± 11.780.124Duration of psoriatic arthritis (years)13.5 ±115.06 ± 6.7Cigarette smoking (ever) n (%)15/38641/14940.72Education duration/year (mean,SD)10.09 ± 3.679.52 ± 4.810.464BMI (kg/m2) (mean, SD)24.5 ±5.128.3 ± 5.21Family history of PsO/PsA n (%)15 (38.5)559 (34.9)0.642Nail involvement n (%)18 (46.2)762 (47.5)0.864Dactilitis n (%)9 (23.7)367 (24)0.958Entesitis n (%)3 (7.9)384 (25.7)0.013Uveitis n (%)-13 (4.3)0.713Axial involvement (%)15 (38.5)464 (29)0.199Methotrexate36 (92.3)1348 (84)0.162Sulfasalazine17 (43.6)612 (38.1)0.488Leflunomide14 (35.9)379 (23.6)0.076Biologic DMARDs102 (33.9)358 (26.8)0.013Conclusion:Although psoriasis may be seen frequently in the juvenile age, juvenile onset PsA was not so frequent in our PsA cohort. Although, ever csDMARD using were similar between two groups, however, juvenile onset PsA patients were used bDMARDs more frequently.References:[1]Kalyoncu U et al. The Psoriatic Arthritis Registry of Turkey: results of a multicenter registry on 1081 patients. Rheumatology. 2017;56:279-286.Disclosure of Interests:Serpil ERGULU EŞMEN: None declared, Ozun Bayindir: None declared, esen kasapoğlu: None declared, Sibel Bakirci: None declared, Dilek Solmaz: None declared, Gezmiş Kimyon: None declared, Atalay Doğru: None declared, Ediz Dalkiliç: None declared, Cem Özişler: None declared, Meryem Can: None declared, Servet Akar: None declared, Emine Figen Tarhan: None declared, Sule Yavuz: None declared, Levent Kiliç: None declared, Orhan Küçükşahin: None declared, Ahmet Omma: None declared, Emel Gönüllü: None declared, Fatih Yildiz: None declared, Duygu Ersözlü: None declared, abdurrahman tufan: None declared, Muhammet Çinar: None declared, Abdulsamet Erden: None declared, Sema Yilmaz: None declared, Seval Pehlevan: None declared, Mehmet Tuncay Duruöz: None declared, Sibel Aydin: None declared, Umut Kalyoncu Consultant of: Abbvie, Amgen, Janssen, Lilly, Novartis, UCB

Background:Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent attacks of fever, peritonitis, pleuritis, arthritis, and skin eruption (1). It is shown by studies that chronic diseases like diabetes mellitus, chronic heart disease, hypertension which other than inflammatory – rheumatologic disease increase depression and anxiety (2). There are a few studies evaluating depression and anxiety in FMF patients, and these results are conflicting (3,4).Objectives:To assess the frequency of depression and anxiety in patients with Familial Mediterranean Fever (FMF)Methods:In this study, 77 FMF patients aged 18 and over who were followed up in Sakarya University Education and Research Hospital, Department of Rheumatology, and 78 healthy volunteers aged 18 and over as thecontrol group. Beck depression scale and Beck anxiety scale were used to depression and anxiety, respectively. Beck’sdepression scale was evaluated as 9 and below normal, 10-16 mild depression, 17-29 moderate depression, 30-63 severe depression. Beck anxiety scale was evaluated as 0-8 normal, 8-15 mild anxiety, 16-25 moderate anxiety, 26 and above severe anxiety.FMF disease severity was determined by Pras scoring.Results:The study group, comprised 77 diagnosed with FMF with a meanage of 37.18 and a control group comprised of 78 healthy controls (C) with a meanage of 35.32 (p=0,058). İn studygroup (P) %63.6, control group (C) %53.8 as female. %36.4 of thestudy group(C), %46.2 of the control group are male. (p=0,216). The prevalence of depression was significantly higher in FMF patients compared to the control group (in order P;C: normal %24,7; %47,4, mild depression: %40.3; %26.9, moderate depression %26; %19.2, severe depression %11.7; %6.4 pConclusion:Both anxiety and depression frequency are increased in FMF patients compared to healthy controls.References:[1]Livneh A, Langevitz P, Zemer D et al. (1997) Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum 40 (10), 1879–85.[2]Alonso J, Ferrer M, Gandek B, Ware JE Jr, Aaronson NK, Mosconi P, Rasmussen NK, Bullinger M, Fukuhara S, Kaasa S, Leplège A, IQOLA Project Group (2004) Health-related quality of life associated with chronic conditions in eight countries: results from the International Quality of Life Assessment (IQOLA) Project. Qual Life Res 13:283–298[3]Makay B, Emiroglu N, Unsal E (2010) Depression andanxiety in children and adolescents with familial Mediterranean fever. Clin Rheumatol 29, 375–9.[4]Giese A, Ornek A, Kilic L, Kurucay M, Sendur S. N., Lainka E, Henning B. F. Anxiety and depression in adult patients with familialMediterranean fever: a study comparing patients living in Germany and Turkey. International Journal of Rheumatic Diseases 2017; 20: 2093–2100Disclosure of Interests: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 Psoriatic arthritis (PsA) has a genetic background, approximately 40% of patients having a family history of psoriasis or PsA in first-degree relatives, which may impact the disease features. Objectives The aim of this study was to evaluate the effects of family history of psoriasis or PsA on the disease phenotypes. Methods The demographic and clinical data were retrieved from the longitudinal, multicenter PsArt-ID (Psoriatic Arthritis-International Database). Family history of psoriasis and PsA were investigated for 1st and 2nd degree relatives separately. The effect of the family history of psoriasis and/or PsA on disease phenotypes and severity were analysed, calculating the relative risks (RR). Results 1393 patients had the data for family history, 444 (31.9%) of whom was positive for psoriasis and/or PsA. The majority of the family history was only psoriasis (333/444; 75%) and 58.5% (260/444) of the patients had first-degree relatives affected. There was no differences in maternal or parental transmission rates however women had more psoriasis and/or PsA in their family (67.3% vs 32.7% p: 0.028). Patients with a family history had an earlier onset of age for psoriasis (29±14.8 vs 31±14.9 p: 0.007), more frequent nail involvement (50.7% vs 29.6% p: 0.032), more frequent enthesitis (28.2% vs 17.7% p Conclusions The family history of psoriasis and PsA has impacts on skin phenotypes, musculoskeletal features and the disease severity. The differences between family history of psoriasis and PsA and pustular vs plaque phenotypes may point out to a different genetic background and pathogenic mechanisms in these subsets. Disclosure of Interest None declared

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. 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 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

To investigate whether there is a difference between male and female patients with Behçet's disease (BD) in terms of hypercoagulability by using modified rotational thromboelastograhic (ROTEM) analysis.126 BD patients (71 male, 55 female; mean age: 41±9 yrs) who met ISSG criteria for BD were included into the study. 23 patients with vasculitis (16 female, 7 male; mean age 49±16 yrs), and 25 healthy individuals (11 female, 14 male; mean age: 37±10 yrs) were included to the study as disease and healthy control (HC) group, respectively. Clotting time (CT), clot formation time (CFT) and maximum clot firmness (MCF) were determined by INTEM and EXTEM analyses. As a marker of vascular endothelial injury, along with inflammatory markers, vWFag levels were investigated in patients and HC group.Extem-CFT was shorter in only vasculitic group compared to HC group. Intem-CFT was found to be shorter in BD patients and vasculitis group compared to HC. Intem-MCF was significantly longer in male BD patients than female BD patients. Extem-CFT was found to be shorter in male BD patients compared to female BD patients. Extem-MCF was statistically longer in male BD patients. In inactive male BD patients, while Intem-CFT was shorter than HC individuals, Intem-MCF and Extem-MCF were statistically longer than HC (p0.02, p0.03), respectively. However, no significant differences were found between inactive female BD patients and HC in terms of all ROTEM parameters.These results support that male BD patients have a hypercoagulable state compared to female BD patients, which may explain why male patients are prone to thrombotic complications.

Cup-like morphology is defined as cup-like nuclear invagination spanning ≥25% of the nuclear diameter in10% of blasts. Studies have shown that FLT3 ITD and normal cytology are strongly associated with cup-like morphology in acute myeloid leukemia (AML) patients. Herein we describe a patient with cup-like blasts that was diagnosed and treated for common acute lymphoblastic leukemia (ALL). In contrast to the literature, the presented case was Philadelphia chromosome positive and FLT3 ITD negative.Cup-like morfoloji, blastların %10’unda fazlasında bulunan, blast çekirdeği çapının, en az %25’ini kapsayan, çekirdeğin içe doğru kıvrılması olarak tanımlanır. Çalışmalarda AML hastalarındaki cup-like morfolojinin, FLT3 ITD ve normal sitogenetik ile güçlü bir ilişkisi olduğu gösterilmiştir. Bu çalışmada Common akut lenfoblastik lösemi tanısı alan ve tedavisi buna göre düzenlenen, cup-like blastları olan bir hasta sunulmuştur. Literatürün aksine bizim hastamızda Philadelphia kromozomu pozitif ve FLT3 ITD negative tespit ettik.

Amaç: Akut abdominal ağrı toplumda çok sık görülmektedir ve acil polikliniğe başvuran hastaların %5%10'unu oluşturmaktadır. Fakat hipofizer yetmezlik gibi cerrahi dışı başka patolojiler olaya dahil olabilir ya da hastalıkla birlikte gelişen stresli durum hipofizer yetersizliği ortaya çıkarabilir. Gereç ve Yöntemler: Bu çalışmada tek bir merkezde karın ağrısı yakınmaları olan ve hipofizer yetersizlik saptanan hastalar değerlendirildi. Bulgular: Hipofizer yetmezlik tespit edilen tüm hastalarda tedavi sonrası karın ağrısı geriledi. Sonuç: Sonuç olarak özellikle geriatrik popülasyonda karın ağrısı nedenlerinin multifaktöryel olabileceği unutulmamalıdır., Objective: Acute abdominal pain is a common symptom and constitutes 5% -10% of patients admitted to the emergency department. But non-surgical events such as pituitary insufficiency also may be clinically evident together or pituitary insufficiency may occur during the stressful situations. Material and Method: In this study, we evaluated five patients that abdominal pain with pituitary insufficiency in a single center. Results: After treatment, abdominal pain regressed all patients finding pituitary insufficiency. Conclusions: Eventually, especially in geriatric populatıon abdominal pain causes for it should be noted that it might be multifactoriel.

Objective: Vascular events are a common complication in patients with polycythemia vera (PV) and essential thrombocythemia (ET). This study aimed to analyze the association between PAI-1 4G/5G and ACE I/D gene polymorphisms, and leukocytosis with thrombosis in patients with PV and ET. Material and Methods: In total, 64 patients with ET and PV were evaluated. Arterial or venous thrombosis, such as cerebral transient ischemic attack, ischemic stroke, myocardial infarction, peripheral arterial thrombosis, deep venous thrombosis, and pulmonary embolism, were defined as a vascular event. DNA samples were screened for mutations via reverse hybridization strip assay. Results: In terms of PAI-1 gene polymorphism, the frequency of the 4G and 5G allele was 48.5% and 51.5%, respectively. The ACE allele frequency was 51.2% and 48.8% for D and I, respectively. There wasn’t an association between occurrence of vascular events and the frequency of any allele. In terms of occurrence of vascular events, there weren’t any significance differences between the patients that were carrying the ACE D/D homozygous allele to ACE I/D and those that carried the I/I allele (P = 0.93). There wasn’t a significant difference in occurrence of vascular events between the PAI-1 5G/5G homozygote allele carriers, and the 4G/5G and 4G/4G allele carriers (P = 0.97). Vascular events were significantly more common in the patients with leukocytosis (leukocyte count >10 × 109 L–1) than in those without leukocytosis (leukocyte count ≤10 × 109 L–1) (P = 0.00). Age >60 years was also a significant risk factor for occurrence of vascular events(P = 0.008). Conclusion: PAI-1 and ACE gene polymorphisms were not considered new risk factors for thrombosis in PV and ET patients. On the other hand, leukocytosis at diagnosis was associated with the occurrence of vascular events in the patients with ET and PV.

Aim: Acute myeloblastic leukemia (AML) is a malign and clonal disease of the bone marrow resulting in neoplastic conversion of hematopoietic progenitor cells. This study was planned to evaluate effects of G-CSF use on neutropenia duration, infection frequency, febrile neutropenia duration, duration of antibiotic and/or antifungal use and cost in AML treatment, Amaç: Akut miyeloid lösemi (AML) kemik iliğinin habis ve klonal bir hastalığı olup hematopoetik öncül hücrelerin neoplastik dönüșümü ile gelișir. Bu çalıșma; AML tedavisinde kullanılan G-CSF’ün nötropeni süresi, enfeksiyon sıklığı, febril nötropeni süresi, antibiyotik ve/veya antifungal kullanım süresi ve maliyet üzerine etkisini araștırmak üzere planlandı. Yöntemler: Çalıșmaya toplam 40 hasta alındı (19 kadın, 21 erkek, yaș ortalaması 42). Hastalar G-CSF kullanılan ve kullanılmayan iki gruba ayrıldı. Nötropeni gelișme zamanı, nötropeni süresi, ateșin ortaya çıkıș zamanı, ateșin süresi, kültürle ispatlanan enfeksiyon, antibiyotik ve antifungal kullanım süresi, hastanede yatıș süresi, antibiyoterapi maliyeti ve toplam maliyet verileri iki grup arasında kıyaslandı. Bulgular: G-CSF grubunda; nötropeni süresi, ateș ortaya çıkıș zamanı, hastane yatıș süresi, ateș süresi, antibiyotik ve antifungal tedavi süresi ve antibiyoterapi maliyeti azaldı. G-CSF grubunda; antibiyoterapi dıșı günlük hastane maliyeti ve toplam maliyette hafif azalma mevcuttu (P: 0,751). Sonuç: AML’li hastaların pekiștirme tedavisinde kullanılan yüksek doz ARA-C protokolüne G-CSF grubunda eklenmesi nötropeni ve hastanede yatıș süresini anlamlı olarak kısaltır (p

An effective treatment strategy for Felty syndrome (FS) has not been developed so far. In this article, three cases with FS, who responded to different treatment modalities, have been presented. Case 1 was a 52-year-old male patient who initially received methotrexate, and then, he was switched to granulocyte colony-stimulating factor (G-CSF) and cyclosporine treatment when his neutropenia was further deteriorated. The patient needed monthly doses of G-CSF for nearly 6 months, and his steroid dose was increased. Afterwards, his neutropenia improved with cyclosporine, methotrexate, and hydroxychloroquine combination treatment. Case 2 was a 78-year-old female patient who was started on leflunomide, hydroxychloroquine, and 60 mg methylprednisolone. Case 3 was a 69-year-old female patient who was first treated with 32 mg methylprednisolone, G-CSF, and then with cyclosporine. Neutropenia of both patients improved, and their health status normalized at 2 months. Different treatment strategies have been tried for the management of FS; disease-modifying anti-rheumatic drugs have been used successfully alone or in combination with G-CSF. As seen in the last case, it should be kept in mind that patients can present predominantly with symptoms of infection or hematologic disorders.