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Takayasu's arteritis (TAK) is a chronic vasculitis, affecting predominantly the aorta and/or its major branches. The aim of this study was to compare the differences between childhood and adult onset TAK.We retrospectively evaluated 179 TAK patients followed between August 2005 and July 2019. Demographic characteristics, laboratory features, disease activity, echocardiographic data at diagnosis and treatment regimens in the disease course were compared between the paediatric and adult onset patients.Twenty-five paediatric-onset (18 years of age at diagnosis) and 154 adult-onset patients (≥18 years of age at diagnosis) were enrolled. The mean age at diagnosis for children and adults were 13.6±4 and 35.6±13, respectively. Paediatric onset TAK patients had more intense inflammation at the time of diagnosis reflected in their clinical findings. Acute phase reactants were high in all paediatric patients and significantly higher in patients with paediatric-onset TAK (p=0.006 and p=0.005, respectively). Abdominal predominant disease was more common in the paediatric group, in contrast, focal disease and aortic arch predominant disease were more common in the adult group. Ascending aortic dilatation, left ventricular hypertrophy and moderate-severe aortic insufficiency were more frequent in echocardiography findings of paediatric onset TAK patients. In comorbidities, hypertension was more common in paediatric TAK patients during follow-up, whereas cerebrovascular disease was more common in adult patients.Our paediatric onset TAK patients presented with a more severe inflammation and more widespread vascular involvement. Multicentre studies from different geographic areas are needed to verify our observation and understand the underlying causes.

Objective Persistent inflammation is an insidious and less studied feature of FMF. We investigated clinical determinants of persistent inflammation and its associations with individual damage items. Methods This is a cross-sectional analysis of 917 FMF patients, who fulfilled the Tel Hashomer criteria and had at least 6 months’ follow-up. Patients were stratified based on whether they had persistent inflammation. We used logistic regression analysis to investigate independent predictors of persistent inflammation and the associated individual damage items. Results One hundred and forty-two (15%) patients had persistent inflammation. Active FMF (54%) was the most prominent reason for the persistent inflammation. Spondylarthritis (16%), other inflammatory arthritis (8%) and IBD (2%) were other frequent reasons. Male gender, history of exertional leg pain, inflammatory comorbidities, M694V homozygosity, colchicine resistance, lower education levels and musculoskeletal attack dominance were found to be the independent predictors of persistent inflammation. Earlier disease onset led to a tendency towards persistent inflammation. Patients with persistent inflammation were more likely to suffer damage. There is an increased risk of developing proteinuria, amyloidosis and renal insufficiency. Conclusion We identified, for the first time, the predictors of persistent inflammation in adult FMF patients and related individual damage items of the Autoinflammatory Disease Damage Index. Persistent inflammation is insidious and one of the chief causes of damage; therefore, especially patients with these predictors should be followed up more closely. If detected, underlying inflammatory comorbidities should be assessed meticulously as early detection and proper treatment strategies may favourably impact the natural history of the disease.

Resume Objectifs L’arterite de Takayasu et la spondyloarthrite sont deux maladies inflammatoires distinctes qui touchent les memes classes d’âge. Le nombre croissant de publications decrivant des cas coincidents d’arterite de Takayasu et de spondyloarthrite a suscite des hypotheses quant a l’association de ces deux affections. L’objectif de cette etude est d’evaluer l’incidence des maladies du spectre des spondyloarthropathies chez les patients presentant une arterite de Takayasu. Methodes Les caracteristiques cliniques et demographiques detaillees de patients presentant une arterite de Takayasu ont ete enregistrees a la recherche de caracteristiques de spondyloarthropathie conformes aux recommandations de l’Assessment of SpondyloArthritis international Society (ASAS). Les patients ont ete interroges sur la presence de rachialgie inflammatoire, d’enthesite, d’uveite, de maladie inflammatoire chronique de l’intestin et d’arthrite peripherique, et les examens appropries de recherche de HLA-B27, radiographie standard et imagerie par resonance magnetique de l’articulation sacro-iliaque ont ete realises. Resultats Au total, 69 patients (65 femmes, 94,2 %) presentant une arterite de Takayasu ont ete recrutes. Une etude detaillee a determine que 14 (20,3 %) d’entre eux remplissaient les criteres de spondyloarthropathie de l’ASAS. La recherche de HLA-B27 s’est averee positive pour deux de ces patients (14,2 %). Les types 1 et 2 de l’arterite de Takayasu etaient plus frequents chez les patients presentant a la fois l’arterite et une spondyloarthropathie que chez ceux sans spondyloarthropathie. Les patients ayant besoin d’une biotherapie etaient plus nombreux dans le groupe presentant les deux affections que chez ceux ayant uniquement une arterite de Takayasu (64,3 % contre 29,1 %, p = 0,014), en raison du caractere refractaire de l’arterite. Conclusion Nos resultats suggerent l’existence d’une association entre arterite de Takayasu et spondyloarthropathie.

OBJECTIVE To describe the associations between autoantibodies, clinical presentation, and outcomes among patients with systemic sclerosis (SSc) in order to develop a novel SSc classification scheme that would incorporate both antibodies and the cutaneous disease subset as criteria. METHODS Demographic and clinical characteristics, including cutaneous subset, time of disease and organ complication onset, and autoantibody specificities, were determined in a cohort of SSc subjects. Survival analysis was used to assess the effect of the autoantibodies on organ disease and death. RESULTS The study included 1,325 subjects. Among the antibody/skin disease subsets, anticentromere antibody-positive patients with limited cutaneous SSc (lcSSc) (n = 374) had the highest 20-year survival (65.3%), lowest incidence of clinically significant pulmonary fibrosis (PF) (8.5%) and scleroderma renal crisis (SRC) (0.3%), and lowest incidence of cardiac SSc (4.9%), whereas the frequency of pulmonary hypertension (PH) was similar to the mean value in the SSc cohort overall. The anti-Scl-70+ groups of patients with lcSSc (n = 138) and patients with diffuse cutaneous SSc (dcSSc) (n = 149) had the highest incidence of clinically significant PF (86.1% and 84%, respectively, at 15 years). Anti-Scl-70+ patients with dcSSc had the lowest survival (32.4%) and the second highest incidence of cardiac SSc (12.9%) at 20 years. In contrast, in anti-Scl-70+ patients with lcSSc, other complications were rare, and these patients demonstrated the lowest incidence of PH (6.9%) and second highest survival (61.8%) at 20 years. Anti-RNA polymerase antibody-positive SSc patients (n = 147) had the highest incidence of SRC (28.1%) at 20 years. The anti-U3 RNP+ SSc group (n = 56) had the highest incidence of PH (33.8%) and cardiac SSc (13.2%) at 20 years. Among lcSSc patients with other autoantibodies (n = 295), the risk of SRC and cardiac SSc was low at 20 years (2.7% and 2.4%, respectively), while the frequencies of other outcomes were similar to the mean values in the full SSc cohort. Patients with dcSSc who were positive for other autoantibodies (n = 166) had a poor prognosis, demonstrating the second lowest survival (33.6%) and frequent organ complications. CONCLUSION These findings highlight the importance of autoantibodies, cutaneous subset, and disease duration when assessing morbidity and mortality in patients with SSc. Our novel classification scheme may improve disease monitoring and benefit future clinical trial designs in SSc.

Objective: The aim of this multi-center study was to assess predictive factors for work-day loss as an indirect cost element in Behçet's syndrome (BS). Methods: In this cross-sectional, multi-center study, 834 BS patients (F/M: 441/393, age mean: 38.4 ± 10.9 years) were included. Data were collected by a questionnaire regarding treatment protocols, disease duration, smoking pattern, frequency of medical visits during the previous year and self-reported work-day loss during the previous year. Results: Work-day loss was observed in 16.2% of patients (M/F: 103/32). The percentages of being a smoker (81.8%), using immunosuppressive (IS) medications (82%), and having disease duration

Objective: To identify several clinical and/or laboratory parameters which can differentiate adult-onset Still's disease (AOSD) from other causes of fever of unknown origin (FUO) and create a clinician-friendly algorithm for this purpose. Methods: FUO patients hospitalized between March 2015 and September 2017 were recruited prospectively. AOSD patients diagnosed between 2001 and 2017 in our department were analyzed. Clinical and laboratory parameters were recorded for all patients. A multivariate analysis was performed to identify possible parameters related to the discrimination of AOSD from FUO. Results: We recruited 69 AOSD patients (51 females, 74%) and 87 patients (43 females, 49.4%) evaluated for FUO. Median ages were 45 (30-57) and 45 (30-62), respectively. Arthralgia, rash, sore throat, neutrophilia, serum ferritin level higher than 5 times of the upper limit, and elevated lactate dehydrogenase levels were associated with the likelihood of diagnosing AOSD; on the other hand, the number of daily fever peaks equal or greater than 3 was associated with the unlikelihood of diagnosing AOSD. After the clinical feasibility assessment of possible parameters derived from the multivariate analysis, in the setting of fever, two clinical (arthralgia, sore throat) and two laboratory (ferritin level, neutrophilia) parameters were selected to develop an algorithm for discrimination of AOSD and FUO. Conclusion: Presence of arthralgia, hyperferritinemia, sore throat, and neutrophilia suggests AOSD in patients presenting as FUO. This study proposes a clinician-friendly algorithm for the first time in current literature to discriminate AOSD from other causes of FUO.

Background: Gastroinestinal involvement in Behcet’s disease impacts morbidity and mortality. The diagnosis of gastrointestinal disease requires comprehensive evaluation with endoscopic and radiologic examinations which is costly and impractical. A biomarker is essential for non-invasive detection. Fecal S100A12 is an established biomarker in gastrointestinal inflammatory diseases and its serum levels are known to increase in Behcet’s Disease. In this study, we aimed to tests fecal S100A12 levels in Behcet’s patients, its relation with gastrointestinal symptoms to its potential as a biomarker in gastrointestinal involvement. Methods: We prospectively enrolled 48 cases of Behcet’s disease patients fulfilling International Study Group criteria from a university hospital. We excluded patients with other autoimmune conditions, active or recent infection, using NSAIDs and antibiotics. Control group was selected from volunteers who had applied to the rheumatology outpatient clinic. Patients with BD were categorized into 5 groups according to organ involvement. Also we evaluated and recorded disease activity with the BDCAF 2006. Fecal S100A12, fecal calprotectin and acute phase reactants were also collected. Results: Fecal calprotectin levels were six-fold higher in BD than controls (p

Background: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by arterial and/or venous thrombosis accompanied by persistently elevated levels of antiphospholipid antibodies. We aimed to evaluate pulmonary manifestations of APS. Method: We retrospectively reviewed the data of patients who were followed in the department of rheumatology with the diagnosis of APS between October 2010 and May 2017. Demographic data, clinical, radiological and laboratory findings were recorded. Results: The study included 67 patients (56 female/11 male) with a mean age of 39±13 years. Pulmonary manifestations such as parenchymal and/or vascular involvement were seen in 12 (17.9%) patients. There were no significant differences between patients with and without pulmonary manifestations in terms of age (p=0.46), comorbidities (p=0.48) and APS duration (p=0.661). Acute pulmonary thromboembolism (PE) was determined in 11 (16.4%), alveolar hemorrhage in 2 (3%) patients. Four patients with acute PE (36%) developed chronic thromboembolic pulmonary hypertension (CTEPH). One patient developed both CTEPH and diffuse alveolar hemorrhage after acute PE during follow up. Antiphosholipid antibody IgM was highly positive in patients with PE compared to patients without PE (p=0.005). Other antiphosholipid antibodies and lupus anticoagulant were not significantly different in patients with and without PE. None of the patients were deceased due to pulmonary manifestations of APS. Conclusion: PE was the most common pulmonary manifestation of APS. The development of CTEPH was high among APS patients. Patients with APS should be closely followed for the onset of PE and CTEPH.

Background Systemic sclerosis (SSc) is typically manifests with SSc-specific antibodies (SSc-Abs): anti-topoisomerase I (ATA), anti-centromere (ACA), anti-RNA polymerase III (ARA), anti-U3RNP (u3RNP), anti-U1RNP (U1RNP), anti-PmScl (PmScl), anti-Ku (Ku) and anti-Th/T0 (Th/T0), each being characterised by distinct clinical features and prognosis. The presence of > 1 SSc-Abs is rare with minimum data about these patients’ clinical phenotype in SSc Methods The autoantibody profiles of 2,799 SSc patients from February 2001 to June 2017 were retrospectively reviewed. Patients with >1 SSc-Abs were identified. Clinical features were collected and compared to historical cohorts of SSc patients with single SSc-Ab positivity. Patients were excluded if treated prior to their immunology test with rituximab, iv immunoglobulins or stem cell transplantation. Statistical analysis was performed using Fisher exact test. Results 72 patients (2.6%) with >1 SSc-Ab were identified. Full clinical data were available for 63 patients. 60 patients (2.1%) had double Ab positivity and 3 patients had triple Ab positivity (0.1%). 13 Ab combinations were present. U1RNP and ATA was the most frequent combination (35%), these patients were significantly younger(51.38 years) than both U1RNP (58.64 years,p=0.050) and ATA (62.03 years,p=0.002) patients and more commonly of diffuse subset (dcSSc)(p = 0.001 and p = 0.041 respectively). Compared to ATA patients overlap features were more frequent (43% vs 15%, p = 0.004) including inflammatory arthritis (p = 0.025) and myositis (p = 0.013) (Table 1). U1RNP and ACA had a significantly higher prevalence of pulmonary arterial hypertension compared to U1RNP (p = 0.039) and ACA (p = 0.022) patients, and compared to ACA patients they were younger (57.88 vs 68.75,p=0.015) with a higher frequency of myositis (p = 0.001). U1RNP and ARA patients were more frequently dcSSc subtype compared to U1RNP patients (75% vs 21%, p = 0.040). U1RNP and PmScl patients had a higher prevalence of myositis compared to U1RNP patients (p = 0.006). ATA and ACA patients behaved similarly to ATA patients with a significantly higher prevalence of lung fibrosis (p = 0.006) and myositis (p = 0.041) compared to ACA. ACA and PmScl (7%) had higher prevalence of myositis compared to ACA patients (p = 0.04). Conclusion Coexistence of hallmark autoantibodies is exceedingly rare in SSc patients. When combined, both SSc-Abs have the potential to synergistically interact and modify the clinical phenotype. Disclosures C. Campochiaro None. K.E.N. Clark None. L. Host None. A. Sari None. C.P. Denton None. V.H. Ong None.

Background Iron deficiency (ID) is more prevalent in systemic sclerosis (SSc) patients with pulmonary hypertension (PH) than those without and associated with worse prognosis. Few data are available about the relationship between serum iron parameters and pulmonary haemodynamics. Methods Right heart catheterisation (RHC) reports of SSc patients were retrospectively reviewed. Subjects were included in the study if they had serum iron studies done within 12 months of RHC and were either diagnosed as 1) pulmonary arteral hypertension (PAH, group 1 PH), defined as mean pulmonary artery pressure (mPAP)≥25 mmHg at rest with pulmonary artery wedge pressure ≤15 mmHg; or 2) no PH. Patients with gastrointestinal disorders that may cause low iron levels and glomerular filtration rate below 60 ml/min/1.73m2 were excluded. We recorded serum iron concentration (µmol/L), total iron-binding capacity (TIBC, µmol/L), transferrin saturation (TS, %), ferritin (µg/L), red cell distribution width (RDW, %), as well as mPAP (mmHg) and pulmonary vascular resistance (PVR, dynes/sec/cm-5). Univariable assocıatıons were assessed using Mann-Whitney test and Spearman's correlation as appropriate. Multiple regression of log-transformed mPAP and PVR and Cox regression were used to compare iron and RDW association with haemodynamics and survival. Results We included 122 subjects in the analysis, 84% were female and mean age at RHC was 57 years. The majority (73%) had limited cutaneous SSc and 34% carried anti-centromere antibodies, followed by 18% with anti-Scl70 and 8% with anti-U3RNP.At RHC, 53/122 (43%) of the patients were diagnosed with PAH. Among them we observed substantially lower levels of serum iron (8.7 vs. 12.1 µmol/L, p < 0.001), TS (15.3% vs. 22.5%, p < 0.001), ferritin (68.1 vs. 112.5 µg/L, p = 0.07), significantly higher RDW (16.4% vs. 14.8%, p < 0.001) and no difference in TIBC (57.4 vs. 54.3 µmol/L, p = 0.163), compared to subjects in whom PH was excluded. mPAP showed moderately strong negative correlation with iron concentration (Spearman's rho=-0.35, p < 0.001) and TS (Spearman's rho=-0.39, p < 0.001) and positive correlation with RDW (Spearman's rho=0.46, p < 0.001).PVR was significantly inversely correlated with iron concentration (Spearman's rho=-0.30, p = 0.001), TS (Spearman's rho=-0.37, p < 0.001 and ferritin (Spearman's rho=-0.22, p = 0.016), while it showed positive correlation with TIBC (Spearman's rho=0.28, p = 0.003) and RDW (Spearman's rho=0.37, p < 0.001).Compared to serum iron indices, RDW is a better predictor of mPAP and PVR. In addition, iron levels did not demonstrate any association with survival, while risk of death was significantly higher for patients with higher RDW (HR = 1.18, p = 0.002) and the association remained after adjusting for presence of PH (HR = 1.16, p = 0.013). Conclusion To our knowledge, this is the first study to demonstrate a significant relationship between ID state and haemodynamic measures of SSc-PAH. Our data also support RDW, as an indicator for functional iron deficiency, may serve as a biomarker for severity of pulmonary vasculopathy in SSc. Disclosures A. Sari None. S.I. Nihtyanova None. B.E. Schreiber None. G. Coghlan None. C.P. Denton None. V.H. Ong None.

Introduction/objectives Familial Mediterranean fever (FMF) is characterized by recurrent attacks of fever, serositis, and arthritis, but some patients may experience long-term complications of disease such as infertility/subfertility. The published data about FMF-associated infertility is still limited. The aim of this study is to investigate the frequency and to determine potential factors for FMF-associated infertility/subfertility. Methods We enrolled 971 adult patients with FMF. We defined infertility as the failure to conceive after 12 months of regular, unprotected intercourse. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics and genotype data (if available), disease complications, laboratory parameters, and treatment features were recorded. Results There were 582 subjects eligible for the present study (mean age 41.05 +/- 10.6 years, 65.8% female). MEFV mutations were available in 482 subjects, and 74.9% of subjects were harboring M694 V mutation (25.1% homozygous for M694 V). Infertility was present in 64 patients (14.6% of females and 4% of males). Multivariate analysis showed female sex [odds ratio (OR), 4.47; 95% confidence interval (CI95%) 1.75-11.42; p = 0.002], FMF disease onset < 20 years [OR, 2.99; (CI95% 1.04-8.61);p = 0.04], disease severity (ISSF) [OR, 4.81; (CI95% 2.28-10.17); p < 0.001], and colchicine nonresponse [OR, 2.80; (CI95% 1.17-6.74) p = 0.021] were the independent predictors of infertility. We also observed reversal of infertility in five patients who were treated with IL-1 beta antagonists. Conclusion Disease severity, FMF disease onset < 20 years, colchicine nonresponse, and female sex were found to be the independent predictors of infertility. The value of effective therapeutic interventions must be determined to treat infertility in these patients.center dot The prevalence of infertility increased in female patients with FMF.center dot Female sex, FMF disease onset < 20 years, disease severity, and colchicine nonresponse were risk factors for FMF-associated infertility.center dot With effective treatment of FMF, reversal of infertility was observed in five patients.

There is a need for better definition of polyarteritis nodosa (PAN) subphenotypes and the influence of ethnicity and geography. This study is aimed to study the demographic and clinical features of PAN cohorts from the UK and Turkey (TR) and to compare and contrast disease characteristics. A retrospective survey of databases from two vasculitis centres between 1990 and 2016 for PAN patients fulfilling the EMEA Vasculitis Classification algorithm. All paediatric-onset adult patients met the Ankara 2008 (EULAR/PReS endorsed) criteria for childhood PAN. Those with typical angiographic and/or histopathologic findings consistent with PAN were included. 93 (M/F: 51/42) patients (UK: 47, TR: 46) were included. Three were HBV-related, 20 (21.5%) had paediatric onset and 16 (16.5%), cutaneous PAN. TR patients had younger age of disease onset 44 (28.5-59.0) vs. 24.5 (11.8-40.5), p = 0.002. Twelve (26%) of TR patients had monogenic disease (Familial Mediterranean Fever association (n = 7), deficiency of adenosine deaminase 2, DADA2, (n = 5). No difference was found in phenotype between paediatric and adult onset patients except for frequency of cutaneous lesions (p = 0.002). During a median 67.5 (32-126) months follow-up, 13 patients died (12.7% in UK vs. 15.2% in Turkish cohorts). No difference was found between two cohorts in relation to relapse rate, death and vasculitis damage index. This study defined a diagnosis of PAN according to the EMEA algorithm. The TR group had a younger age of disease onset and more cases of monogenic disease; however, disease extent, relapse rate, damage index and death rates were similar between groups.

Aim To determine the frequency and define the causes of pulmonary hypertension (PH) in patients with Takayasu arteritis (TA). Method Sixty-four TA patients were evaluated by transthoracic echocardiography (TTE). Having an estimated systolic pulmonary arterial pressure (sPAP) ≥40 mm Hg by echocardiography or if performed, mean PAP ≥25 mm Hg in right heart catheterization was defined as PH. Clinical, imaging and laboratory results of the TA patients were obtained from hospital files. Result In total, seven (10.9%) patients had PH. Four patients had PH due to left-sided heart disease (group 2 PH), three patients due to pulmonary arterial involvement (PAI; group 4 PH) and one patient due to atrial septal defect (group 1 PH). In one patient, combination of PAI, aortic insufficiency and pulmonary venous return anomaly was present and he was considered to have both group 2 and group 4 PH. PAI was more frequent (42.9% vs 15.7%) in patients with PH but the difference was not statistically significant. The percentage of patients treated with cyclophosphamide and/or biologics was higher in the group with PH as compared to the group without PH (P = 0.015). One patient with group 4 PH had been on pulmonary arterial hypertension (PAH)-specific agents for 8 years. Conclusion Pulmonary hypertension is not infrequent in TA patients and all the potential causes of PH should be carefully evaluated. Patients with severe or treatment-resistant disease are prone to have PH. PAH-specific agents may be effective in patients with group 4 PH.

Background:Postthrombotic syndrome(PTS) defines chronic manifestations of venous insufficiency following deep vein thrombosis(DVT).It is the most frequent and disabling complication of DVT.Objectives:Aim of the study is to describe clinical characteristics and predictors of PTS,severe PTS among Behçet’s disease(BD)patients with known low extremity DVT.Also,to depict venous Doppler ultrasonography(US)findings and its association with the clinical characteristic and treatments.Methods:This retrospective multicenter study included 205(166men,39women;mean age 39±9.5 years)BD patients with DVT history.The Villalta scale was used to assess the presence and severity of PTS.Doppler US of bilateral legs are performed within 1 week of clinical evaluation.Total number of vessels with reflux,thrombi,recanalization and collaterals were calculated and presented as scores.Results:Of 205 BD patients with known DVT history; 127(62%) had PTS and 18% had severe PTS diagnosed by Villalta scale.(Table 1)Table 1.Demographic and Clinical Characteristics of PatientsEntire Study Population (n:205)PTS present (n:127)PTS absent (n:78)P valueSex(male,%)166,81%101,79.5%65,83.3%.584History of smoking,%105,52%64,51.2%41,53.2%.895BMI(n:198)26.2±4.626.7±525.6±3.8.123Disease duration,years9(5-15)10(5-16)8(5-13).205DVT following time years6(3-11)7(3-11.5)5(3-9).179Current age39±9.540.5±9.636.8±8.8.012Number of vascular events1(1-2)1(1-2)1(1-2).723VeinesQoL total82±16.375.8±1492.2±14.6VeinesQoL symptom37.5±934.4±8.342.6±7.7Doppler Findingsİliofemoral thrombi41,26.5%32,35%9,17%.014Bilateral involvement62,43.4%45,49%17,33%.080All median values presented with interquartileranges(IQR)Table 2 summarize multivariate logistic regression analyses for the risk of having PTS-moderate/severePTS.Table 2.Multivariate Regression Analysis of presence of PTSOdds Ratio(with 95%CI)PCurrent age1.05(1.01-1.10).048BSAS1.06(1.04-1.10).000Bilateral Doppler USG involvement2.81(1.18-6.67).019İliofemoral thrombi2.74(1.02-7.38).045Moderate/severe PTSOdds RatiopBody mass index1.09(1.001-1.19).048IS0.10 (0.02-0.05).005Severe PTS patients had increased reflux(p=.027) compared to mild group and decreased recanalization scores(p=.013)compared to moderate group(Figure1)Patients treated with AC+IS had increased recanalization(p:.078),collateral scores compared patients treated with only ISs(p=.004)(Figure2)Conclusion:After an acuteDVT,BD patients faced with high risk of having PTS,severe PTS.ISs decreases the risk of having PTS.Our results also suggest that AC treatment may decrease severity of PTS by increasing recanalization of thrombi.Figure 1.US scores for PTS absent and present group Reflux score(p=.054) and thrombosis scores(p=.02)Figure 2.US scores for treatment groupsReferences:Disclosure of Interests:None declared

Familial Mediterranean fever (FMF) is characterized by recurrent short-lived/self-limiting inflammatory attacks. Besides these, a substantial number of patients with FMF present with a variety of other inflammatory diseases; however, this issue has not been systematically studied previously. Hence, we aimed to investigate the frequency of inflammatory comorbid diseases in a large FMF cohort. All patients were recruited from "FMF in Central Anatolia (FiCA) Cohort", comprising 971 (mean age 35.3 +/- 12 years, 61.5% female) adult subjects. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics, MEFV gene mutations, and comorbid inflammatory diseases were meticulously questioned, and laboratory features and genotype data were retrieved from hospital records. There were comorbid inflammatory diseases in 205 (21.1%) patients. The most common inflammatory disease was spondyloarthritis (12.9%). Other remarkable inflammatory disorders were psoriasis, immunoglobulin A vasculitis/Henoch-Schonlein purpura, Behcet's disease and inflammatory bowel diseases. Cryptogenic organizing pneumonia is a newly defined entity in our cohort which is seemed to be associated with FMF (0.3%). Number of patients with persistent inflammation was higher in those with comorbid diseases (p < 0.001). Our results suggest that FMF is commonly associated with other inflammatory diseases. Therefore, clinicians should be cautious about comorbid inflammatory diseases in FMF patients, particularly in those with persistent inflammation. Identification of pathogenic pathways linking FMF to these diseases warrants further investigations.

Background: Scleroderma is a connective tissue disease that is characterized by fibrosis of the skin.The modified Rodnan Skin Score (mRSS) is a measure generally used to assess the skin thickness in patients with scleroderma.Data on the effectiveness of the mRSS training courses differ in the literature. Objectives: The objective of our study was to evaluate the effectiveness of the mRSS training course in rheumatology fellows in the rheumatology departments. Methods: The study included 6 fellows from the departments of rheumatology.Participants were given a 1-hour-long theoretical training, including dermal involvement, and mRSS assessment by 3 rheumatology experts experienced in scleroderma, which was followed by an applied training on 4 patients for one-hour.Participants scored two patients before and after training on a form, which included 17 domains with a total score-range between “0” and “51”. Then using the SPSS15 software program, inter-rater reliability was assessed with intraclass correlation(ICC) analysis for both pre- and post-training mRSS.Fleiss’ kappa was used to measure the degree of agreement according to 12 Rodnan score areas before and after the training. Results: The ICC value for pre-training and post-training total Rodnan scores was 0.867 (95% CI-0.625-1.00, P=0.05), and 0.905(95% CI 0.045-1.00, P=0.02), respectively. Individual analysis of score areas showed that after the training there was an increase in degree of agreement in some of these areas, while there was no difference in one area, and it decreased in others (Table 1). Conclusion: Several studies have demonstrated the applicability, reliability, and validity of mRSS, a measure of dermal involvement, and that evaluation of the score requires experience, and an attentive learning process.In the literature, the inter-rater ICC values during previous training courses are reported to range between 0.378 and 0.92(1). These studies show differences in terms of the number and experience of participants, number of patients, course-length, and repeated courses. Similar to the observation by Ionescu et al(2), the pre-training inter-observer ICC value was very high(0.867), and it increased to 0.905 after the training. Presence of an increase in some areas, and reduction in some others in individual analysis of areas after the training may suggests the need for repeated training. Our limitations were low number of participants and patients. We obtained very good inter-rater ICC values in the mRSS training course given to rheumatology fellows; however we may benefit from new studies for optimization of these conclusions by increasing the numbers of trainees, patients, and the lenght of courses. References [1] Czirjak, L., et al., the EUSTAR model for teaching and implementing the modified Rodnan skin score in systemic sclerosis. Ann Rheum Dis, 2007. [2] Ionescu, R., et al., Repeated teaching courses of the modified Rodnan skin score in systemic sclerosis. Clin Exp Rheumatol, 2010. Disclosure of interests: None declared

Background: Takayasu Arteritis (TAK) is a rare idiopathic granulomatous large vessel vasculitis and it is a clinically heterogenous disease. Various classifications and subsets which based on distribution of arterial lesions were defined in the literature in order to exhibit and predict different disease courses. Objectives: We aimed to validate a new disease subsets developed by Goel et al in our TAK series (1). Methods: We retrospectively evaluated the medical records of 164 TAK patients followed at Department of Rheumatology and Department of Pediatric Rheumatology in Hacettepe University, Ankara, Turkey between August 2005 and October 2018. The pediatric-onset ( Results: There were forty-two (25.6%), sixty-three (38.4%) and fifty-nine (36%) patients in Cluster 1, 2 and 3, respectively. Demographic and clinical data was summarized in Table-1. Cluster 1 included approximately half of patients (45.8%) with pediatric-onset TAK (p= 0.048). Baseline acute phase reactants (Erithrocyte Sedimentation Rate:ESR and C-reactive protein:CRP) and age at disease diagnosis were slightly lower in Cluster 1, however none of them did not reach statistical significance (p= 0.94, p=0.99 and 0.56, respectively). In contrast, cerebrovascular accident rates were slightly higher in Cluster 2 (p=0.24). Although anti-TNF biological agents were more frequently used for treatment in Cluster 1 (p=0.004), cyclophosphamide and/or overall biological agents usage was similar in three groups (p=0.15). Conclusion: Decision tree which defined by Goel et al. was applied to our single center pediatric and adult TAK cohort. In contrast to original validation cohorts, there was no difference among three clusters of our cohort except pediatric-onset disease and anti-TNF usage. Dissimilarities among the cohorts in terms of new classification system may be caused either number of patients or ethnic/regional differences. Reference [1] Goel R, Gribbons KB, Maksimowicz-McKinnon K, Hoffman GS, Kumar S, Joseph G, et al., editors. Discovery and Validation of Novel Disease Subsets in 806 Patients with Takayasu’s Arteritis across Four International Cohorts. ARTHRITIS & RHEUMATOLOGY; 2018: WILEY 111 RIVER ST, HOBOKEN 07030-5774, NJ USA. Disclosure of Interests: Ertugrul Cagri Bolek: None declared, Selcan Demir: None declared, Alper Sari: None declared, Ummusen Kaya Akca: None declared, Levent Kilic: None declared, Ali Akdogan: None declared, Sedat Kiraz: None declared, Omer Karadag: None declared, Seza Ozen Consultant for: Seza Ozen is receiving consultancy fees from Novartis, Speakers bureau: Roche

Objective Psoriatic Arthritis Impact of Disease 12-item questionnaire (PsAID-12) has been developed to be used in psoriatic arthritis in daily practice. The aim of the present study was to assess the change values of PsAID-12 in PsA patients continuing or discontinuing anti-TNF treatment. Methods We recruited patients from the Hacettepe University biological database (HUR-BIO). Overall, 70 PsA patients had PsAID-12 score before the initiation of the first anti-TNF treatment. Stopping or switching the anti-TNF treatment due to inefficacy was definitely considered a negative response. Changes were evaluated by the comparison with the baseline PsAID-12 score in compliance with the favorable and unfavorable responses to anti-TNF treatments. The standardized response mean (SRM) was used for determining the response. Results Seventy (78.6% female) patients were analyzed and their mean age was 45.5 years (12.0 years). The mean follow-up duration was 18.3 months (12.6 months). At baseline, the mean PsAID-12 score was 6.6 (1.5). Physicians stopped or switched the treatment in 28 patients (40.0%) due to the inefficacy of anti-TNF treatment. The ΔPsAID-12 score was 0.25 (1.71) in the patients discontinuing anti-TNF treatment and 3.52 (2.31) in the patients continuing their anti-TNF treatment (p

Rheumatoid arthritis (RA)-associated lung involvement is a cause of mortality. This study aimed to evaluate mortality rate and mortality-associated factors in RA patients with high-resolution computed tomography (HRCT)-proven lung involvement.Patients followed-up for RA between 2010 and 2018 were evaluated regarding HRCT-proven lung involvement. The present study was designed as a single-centre, retrospective and descriptive study. The HRCT reports of patients were re-evaluated for three major patterns: UIP, nonspecific interstitial pneumonia (NSIP), and isolated airway disease (AD). Mortality rates and its associated factors (demographic characteristics, RA-related factors and lung-involvement-related factors) were determined.The study included 156 patients (females, 68.3%) with radiologically confirmed RA-associated lung disease. The mean age was 55.5 (12.1) years at RA diagnosis and 62.7 (9.7) years at the diagnosis of lung involvement. The patterns of lung involvement on HRCT were UIP in 89 (57.0%) patients, NSIP in 51 (32.7%) patients, and isolated AD in 16 (10.3%) patients. The RA patients were followed-up for a mean of 10.2 (7.4) years and they were followed-up for a mean of 4.5 (3.7) years after interstitial lung disease (ILD) diagnosis. Overall, 40 (25.6%) patients died. The 5-year survival rate was 78%. Multivariate analysis revealed UIP pattern (log-rank test, P0.01), pleural effusion (log-rank test, P0.05), and a shorter time interval (3 years) between the diagnoses of RA and RA-ILD (log-rank test, P0.01) to be independent predictors of mortality.In addition to the UIP, a known risk factor, pleural effusion and the short time between the diagnoses of RA and ILD were also found to be associated with mortality.

Background:Colchicine is the mainstay of treatment in FMF. However, in daily practice it is not easy to maintain effective colchicine doses in substantial number of patients, due to its side effects.Objectives:It was aimed to investigate prevalence and risk factors for colchicine side effects that limit optimal drug dosing and permanent discontinuation.Methods:All patients were recruited from “FMF in Central Anatolia” (FiCA) cohort, 915 adult subjects with minimum follow up time of 6 months and had compliance of treatment were included. Demographic and anthropometric data, FMF disease characteristics, disease severity, complications and treatment features were recorded on a web based registry. Prevalence of colchicine intolerance and characteristics of intolerant patients were analyzed.Results:Effective colchicine doses cannot be maintained in 172 (18.7%) subjects. Main side effects that limit optimal dosing were as follows; diarrhea in 99 (10.8%), elevation in transaminases in 54 (5.9%), leukopenia in 10 (%1.1), renal impairment in 14 (1.3%), myopathy in 5 (0.5%) and allergic skin reaction in two. Colchicine had to be permanently ceased in 18 (2%) patients because of serious toxicity. Male gender and obesity were found to be associated with liver toxicity and having normal body weight was associated with diarrhea. Chronic inflammation and proteinuria were more common in colchicine intolerant patients and they had reported more frequent attacks compared to those tolerating optimal doses.Conclusion:Colchicine intolerance is an important problem in daily clinical practice, mainly due to diarrhea and liver toxicity. Suboptimal colchicine dosing associated with complications.References:[1] Sönmez, H.E., E.D. Batu, and S. Özen,Familial Mediterranean fever: current perspectives.Journal of inflammation research, 2016.9: p. 13.[2] Sari, İ., M. Birlik, and T. Kasifoğlu,Familial Mediterranean fever: an updated review.European journal of rheumatology, 2014.1(1): p. 21.[3] Ozen, S., et al.,EULAR recommendations for the management of familial Mediterranean fever.Annals of the rheumatic diseases, 2016.75(4): p. 644-651.Table 1.Prevalence of all side effects of colchicine and reasons for drug discontinuationSide effectAll side effectsN=172*Permanent cessationN=18*Diarrhea9911Liver toxicity544Leukopenia101Muscle toxicity52Skin reaction2-Nausea4-Infertility2-* some patients had more than one clinically significant side effectTable 2.Disease course in colchicine tolerant and intolerant patientsColchicine TolerantN=743Colchicine IntolerantN=172p valueChronic inflammation115 (15.4%)45 (26.1%)Number of attacks in the last year4.05±6.087.60±9.6Proteinuria44 (5.9 %)20 (11.6%)0.025Amyloidosis33 (% 4.4)23 (13.3%)ADDI (median)1 (1)1 (1)ADDI: auto-inflammatory disease damage index, FMF: familial Mediterranean feverDisclosure of Interests:Hasan Satiş: None declared, Berkan Armagan: None declared, Erdal Bodakci: None declared, Nuh Atas: None declared, Alper Sari: None declared, Dilek Yapar: None declared, Nazife Sule Yasar Bilge: None declared, reyhan bilici salman: None declared, Gözde Kübra Yardimci: None declared, Hakan Babaoglu: None declared, Levent Kiliç: None declared, mehmet akif ozturk: None declared, Berna Goker: None declared, seminur haznedaroglu: None declared, Umut Kalyoncu Consultant of: Abbvie, Amgen, Janssen, Lilly, Novartis, UCB, Timuçin Kaşifoğlu: None declared, abdurrahman tufan: None declared

Background:Serum autoantibodies closely reflect patterns of organ involvement and disease progression in systemic sclerosis (SSc). The entire autoantibody profile is less well defined in many cohorts and the data regarding their clinical associations and frequencies is limited.Objectives:To determine the autoantibody profile of patients with SSc, as well as their clinical associations, in well-characterized inception- cohort with disease duration less than 3 years.Methods:Serum samples of 100 patients out of 105 enrolled in the study were analyzed for ANA patterns with indirect immunofluorescence (IIF) assay using HEp-20-10/primate liver mosaic IIFT kit. Sera of 96 patients were subjected to commercial line immunoassay to quantify autoantibodies against 13 different autoantigens.Results:92 (92%) out of 100 patients were positive for ANA by IIF (Table 1). The speckled staining was the most pattern followed by nucleolar in 10 patients, centromere in 4, reticular in 1, nuclear in 2 and homogenous in 1. All patients (n= 96) patients were positive for at least 1 autoantibody by immunoblotting (Table 2). Twenty-two (49%) of patients with antiTopo I, 12 (44%) of the patients with antiCENP and 4 (22%) of the patients with antiRNAPIII were single positive. There was no difference in terms of the clinical findings when the patients with single and coexpression of these antibodies were compared. The distributions of the most frequent autoantibodies are shown in Figure 1. Interstitial lung disease was more frequent in the patients positive for anti-Topo I (78.8%) and anti-RNAPIII (27.3%). One of the two patients with breast cancer was anti-RNAPIII positive and none of the patients have diagnosed scleroderma renal crisis. Anti-Topo I was more common in patients with dcSSc (75%) and anti-CENP in lcSSc (46.4%).Table 1.Demographic, clinical and laboratory characteristics of the SSc patients.Sex Female N, %91 (86.7%) Male N, %14 (13.3) Female-to-male ratioAge, mean±SD years48.6±12.7Disease duration, mean±SD years2±1.4Disease classification N, % Diffuse39 (36.5%) Limited65 (62.5%) Sine scleroderma1 (1%)Interstitial lung disease37 (34.3%)Pulmonary arterial hypertension3 (2.8%)Scleroderma renal crisis0Digital ulcer14 (13.3%)Raynaud phenomenon105(100%)Telengiectasia31 (28.8%)Calcinosis1 (1%)Malignancy3 (2.9%)Antinuclear antibody profile N*, % Positive92 (92%)Staining pattern Speckled65 (65%) Nucleolar13 (13%) Centromere29 (29%) Homogeneous3 (3%) Reticular3 (3%)Table 2.Numbers and combinations of autoantibodies identified in the 96 SSc patients.Topo-ICENPRNAP IIIFibrillarinNOR90Th/ToPm/SclKuPDGFRRo52Topo-I22170159507CENP12202222011RNAPIII40121307Fibrillarin0000000NOR90011002Th/To04202Pm/Scl4306Ku102PDGFR00Ro522Single positive221240004102Total452718058179024Figure 1.Diagram of disease-related antibodies against the four main autoantibodies [anti-centromere (antiCENP) anti-Topoisomerase I (antiTopo I), anti-RNA polymerase III (antiRNAP III) and anti-Ro52).Conclusion:We presented the clinical and serologic features of the Turkish SSc patients from a new inception cohort. Clinical features of the SSc patients with single or multiple antibody positivity were not different.References:NoneDisclosure of Interests:None declared

Objectives Takayasu arteritis and Spondyloarthritis are two distinct inflammatory diseases that affect the same age periods. Increasing number of reports on co-incident Takayasu arteritis-spondyloarthritis cases in literature raised the hypotheses about their association. The purpose of this study is to evaluate the incidence of spondyloarthropathy spectrum diseases in Takayasu arteritis patients. Methods Detailed clinical and demographic features of Takayasu arteritis patients were recorded and all were screened meticulously for the presence of spondyloarthropathy features following recommendations of Assessment of SpondyloArthritis international Society. Patients were questioned for inflammatory back pain, enthesitis, uveitis, inflammatory bowel disease, peripheral arthritis, and investigated accordingly with HLA-B27, plain X-rays and sacroiliac magnetic resonance imaging. Results A total of 69 Takayasu arteritis patients (65 female, 94.2%) were enrolled. After detailed investigation, 14 (20.3%) Takayasu arteritis patients fulfilled the Assessment of SpondyloArthritis international Society criteria for Spondyloarthropathy. Two of 14 (14.2%) spondyloarthropathy patients were positive for HLA-B27. Type 1 and type 2 Takayasu arteritis were more common in patients with diagnosis of both Takayasu arteritis and spondyloarthropathy than those without spondyloarthropathy. Most of patients with diagnosis of both these diseases required biologic therapies than patients with diagnosis of Takayasu arteritis alone (64.3% vs 29.1%, P = 0.014) due to refractory Takayasu arteritis. Conclusion Our results suggest a significant association between Takayasu arteritis and spondyloarthropathy. Possible shared genetic or immunopathogenic processes may explain this association, which merits further investigations.

Background Drug survival rate is generally accepted as a reliable indicator of both efficacy and safety profile of a biological DMARD. Objectives To evaluate survival rates of certolizumab-pegol (CZP) in ankylosing spondylitis (AS) and non-radiographic axial spondyloarthritis (nrAxSpA) patients registered in HUR-BIO (Hacettepe University Rheumatology Biologic Registry). Methods HUR-BIO is a monocentric database of biologics including 2058 SpA patients (226 were prescribed CZP) by June 2017. All AS (n=142) and nrAxSpA (n=54) patients in HUR-BIO prescribed CZP at least once were enrolled in the study. Twenty-three patients who was already on CZP before admitting Hacettepe University were excluded. Patients prescribed CZP within 6 months period before analysis defined as drug continuing. Demographic, clinical and laboratory data of AS and nrAxSpa patients were evaluated. Kaplan-Meier analysis was used to estimate CZP survival rates. Results In total, 124 AS and 49 nrAxSpA patients were analysed. Baseline characteristics of patients were shown in Table. Median duration of CZP usage was 7.54 (3–26.5) months in AS and 6.27 (3–26.7) months in nrAxSpA group(p=0.53). CZP survival was similar between AS and nrAxSpA patients (figure 1). Fourty-nine (27.2%) patients had used at least one TNFi and 38 (21.9%) patients had used more than one TNFi before CZP. There was no difference in drug survival between those who used TNFi and those who did not use TNFi before CZP (figure 2). BASDAI 50 response was reached in 27.6% of AS and 36.0% of nrAxSpA patients and at the last control visit (p=0.44). Conclusions In this single centre biological SpA cohort, adherence to CZP therapy seems to be high with a first year survival rate of 83% and there was no difference between AS and nrAxSpA patients. Disclosure of Interest None declared

Objectives IgA Vasculitis is a leukocytoclastic vasculitis involving small vessels with depositions of immune complexes containing IgA. IgA Vasculitis is a predominantly paediatric vasculitis. There is limited data for the prognosis of adult IgA Vasculitis, with also no damage assessment. In this study, we aimed to evaluate the clinical characteristics, treatment, outcome and damage of patients with adult IgA Vasculitis. Methods We assembled a retrospective cohort of patients with adult IgA Vasculitis from tertiary Rheumatology Centres in Turkey. The demographics, clinical characteristics, treatment and outcomes of patients were abstracted from medical records. Birmingham Vasculitis Activity Score (BVAS), prognostic Five Factor Score (FFS) and vasculitis damage index (VDI) were calculated. Results The study included 103 (male/female: 67/36) patients with adult IgA Vasculitis. The mean age was 42.6±17 years. Infection history within 6 weeks before presentation was present in 40 (38.8%) patients (32 upper respiratory tract, 3 urinary tract, 2 gastrointestinal, 3 others). Cutaneous manifestations and arthritis/arthralgia were the most common clinical manifestations (table 1). 92 (89.3%) patients were treated with oral glucocorticoids (GC). Pulse GC treatment was also given to 29 (28.1%) patients. As additional immunosuppressive agents, azathioprine was given to 36 (34.9%) and pulse cyclophosphamide to 13 (12.6%) patients. Fifty-nine patients (58.2%) had follow-up of mean 35.6 months. Eleven (18.6%) patients relapsed during follow-up. While 5 relapses were major, six of them were minor relapses. At the last visit, disease status was evaluated as active or treatment failure by the treating physician in 7 (11.8%) patients. The rate of chronic renal failure was 8.3%(n=5). Mortality was 1.6% (n=1) during follow-up, due to pneumonia. The mean VDI score was 0.3 in the last visit. Twelve (20.3%) patients had at least one damage item at the end of follow-up period. Conclusions Our results showed that approximately one fifth of patients with adult IgA Vasculitis had relapses during follow-up and had at least one damage item at the end of follow-up. Although, 31% of patients had FFS≥1, the mortality rate was observed to be very low in the present study. Disclosure of Interest None declared

In patients with systemic sclerosis (SSc) gastrointestinal (GI) involvement, nutritional status and medications may lead to cobalamin (Vit B12) deficiency. We aimed to determine the frequency and the potential causes of Vit B12 deficiency in SSc patients.We conducted a cross-sectional analysis of 62 SSc patients in a single center in 1 year period. Medical history and physical examination of patients were reevaluated. Data about organ involvements were obtained from hospital file records. The nutritional status of the patients was assessed with Malnutrition Universal Screening Tool (MUST). Vit B12, homocysteine (except in three patients) and Helicobacter Pylori Immunoglobulin G (H. Pylori IgG) levels were measured in all patients. Vit B12 deficiency was considered as serum Vit B12 level200 pg/mL or being on Vit B12 replacement therapy. Serum Vit B12 levels of the patients were also grouped as low (200 pg/mL), borderline (200-300 pg/mL) and normal (300 pg/mL). Plasma homocysteine levels of the patients were classified as elevated (9 μmol/L) and hyperhomocysteinemia (15 μmol/L). Mann-Whitney U and Kruskal-Wallis tests were used to compare parameters among the groups. Correlation was tested by Spearman's correlation coefficient.Forty-four (71.0%) patients were defined as Vit B12 deficient; 22 had Vit B12 level200 pg/mL (four were on Vit B12 replacement therapy) and the remaining 22 had Vit B12200 pg/mL and were already on Vit B12 replacement therapy. The percentage of the patients with hyperhomocysteinemia was significantly higher in the group with Vit B12200 pg/mL as compared to other groups (P = 0.004) but only 33.3% (7/21) of the patients with Vit B12200 pg/mL had hyperhomocysteinemia. There were no statistically significant differences between patients with and without Vit B12 deficiency regarding age, mean disease duration, MUST scores, mean hemoglobin levels, H. Pylori IgG positivity and organ involvements (P0.05 for all).Vit B12 deficiency is frequent in SSc and has multiple causes. All patients should be monitored for Vit B12 deficiency. The homocysteine levels seem unlikely to be helpful for confirmation of Vit B12 deficiency.

Objective: Familial Mediterranean fever (FMF) is a periodic fever syndrome caused by MEFV mutations. FMF may be associated with psoriasis in some cases. The prevalence of psoriasis in the normal Turkish population is 0.42%. We aimed to investigate the prevalence of psoriasis among FMF patients and their relatives. Methods: FMF patients followed at Hacettepe University Adult and Pediatric Rheumatology Departments between January and August 2016 were included. FMF patients/their relatives were accepted to have psoriasis if the diagnosis was made by a dermatologist. Results: A total of 351 FMF patients (177 adults; 174 children) were included. The median (min–max) age of adult and pediatric patients was 35 (19–63) and 10 (2–18) years, respectively. Thirteen (3.7%) FMF patients (11 adults, 2 children) had psoriasis. Psoriasis was more common in adult than pediatric patients (p = 0.02). Psoriasis was present in 22 (12.4%) of adult and 9 (5.2%) of pediatric patients’ relatives (p = 0.023). The frequency of psoriasis in ≥1 relatives of FMF patients was found to be 8.8%. Abdominal pain and fever were significantly higher, and arthralgia, arthritis, pleural chest pain, and pericarditis were significantly less frequent in the pediatric group than in adults (p

Objective: Immunoglobulin A vasculitis/Henoch–Schönlein purpura (IgAV/HSP) is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA. It is the most common primary systemic vasculitis of childhood and is much less common in adults. Our aim was to investigate the differences and similarities between adult and paediatric patients with IgAV/HSP. Method: We retrospectively evaluated the medical records of 35 adult and 159 paediatric (˂ 18 years old) patients with a clinical diagnosis of IgAV/HSP who were seen at the Departments of Rheumatology and Pediatric Rheumatology, Hacettepe University, Ankara, Turkey. The paediatric and adult patients were classified with IgAV/HSP according to the Ankara 2008 and American College of Rheumatology 1990 criteria, respectively. Results: Upper respiratory tract infection was a common predisposing factor for both adults (34.3%) and children (21.4%). Creatinine and C-reactive protein were higher; and skin biopsy, hypertension, renal involvement, haematuria, proteinuria, and renal insufficiency at diagnosis were more frequent in adults than in children. Thrombocyte count was higher in children than in adults. Follow-up without treatment and complete recovery were more frequent in children, while persistent haematuria, chronic renal failure, relapse, and the use of corticosteroids/azathioprine were more frequent in adults. The only independent predictive factor for relapse was persistent haematuria. Conclusion: Various clinical and laboratory characteristics differ between children and adults with IgAV/HSP. Overall, IgAV/HSP has a self-limiting course in children but represents a more severe form of disease in adults, with more severe renal involvement. Persistent haematuria is a predictive factor for relapse.

First, thanks for this interesting article that tries to answer a difficult clinical problem of patients with fever of unknown origin (FUO) and inflammation of unknown origin (IUO) who are discharged without diagnosis.1 This study is very important in creating a diagnostic algorithm for FUO and IUO. We wanted to address some of the …

The objective of this study was to report the experience with rituximab treatment in a case series of patients with long-standing systemic sclerosis-associated interstitial lung disease (SSc-ILD).We reviewed the charts of 197 SSc patients. Fourteen patients who received rituximab for SSc-ILD participated in this analysis. Pulmonary function tests, high-resolution thorax computed tomography and modified Rodnan skin scores were evaluated at baseline and end of the follow-up.Median age was 53.2 years (interquartile range, 46.8-55.5 years), and median disease duration was 9.1 years (interquartile range, 5.1-13.6 years). At the end of median follow-up (15 months), although the median forced vital capacity value increased compared with baseline, the change was not statistically significant (52.5 vs. 58.0, P = 0.065). Forced vital capacity was improved in 4 patients and stabilized in 10 patients. High-resolution computed tomography was stable in 7 patients and worsened in 3 patients. Modified Rodnan skin scores remained stable at the end of follow-up (8.0 vs. 6.0, P = 0.6).The improvement or stabilization of pulmonary disease was observed in most SSc patients with longer disease duration and worse pulmonary function. Rituximab might be useful in this patient group who is resistant to conventional immunosuppressive treatments.

First, thank you for this article which tries to answer a difficult clinical problem about anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis remission phase treatment.1 We believe that the study will be a milestone in ANCA-associated vasculitis maintenance treatment paradigm. To improve clinical implications, we wanted to ask about some important points in the study. As seen in the literature,2 the Five Factor Score (FFS) is an easily …

Objective Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium/small arteries. We aimed to examine the characteristics of adult- and childhood-onset PAN. Methods Fifteen pediatric (˂ 18 years) and 22 adult PAN patients who fulfilled the Ankara 2008 and American College of Rheumatology 1990 criteria, respectively, were included in the study. Results Five children had cutaneous and all the rest of the patients had systemic PAN. Weight loss was more common (59.1% vs. 20%, P = 0.041) and presence of an angiography at diagnosis was more frequent (81.8% vs. 33.3%, P = 0.003) in adults than children. Arthralgia/arthritis and skin involvement were more common in children (86.7% vs. 59.1%; 93.3% vs. 72.7%, respectively) while renal and neurologic involvement were more frequently observed in adult patients (50% vs. 20%; 59.1% vs. 40%, respectively) (P > 0.05 for all). Cutaneous PAN patients were treated with corticosteroids only. All but one adult patient received cyclophosphamide while mycophenolate mofetil was used in five and cyclophosphamide was used in four children as induction treatment. The median duration of induction treatment was longer in adults than children (12 vs. 3 months, respectively; P = 0.004). The most common maintenance drug was mycophenolate mofetil in children and azathioprine in adults. The mortality rate was 13.6% (n = 3) and 0% in adults and children, respectively. Conclusion To our knowledge, this is the first study comparing characteristics of adult and childhood onset PAN. Our results have suggested that juvenile PAN had a more benign course (with less renal and neurologic involvement, shorter duration of induction treatment) than adult onset PAN.

Background Lungs are one of the target organs in rheumatoid arthritis (RA) patients. Interstitial lung disease (ILD), rheumatoid nodules, pleural effusion, bronchiectasia and small airway disease are common manifestations of lung involvement. Objectives Objective of this study was to evaluate distribution of Thorax CT findings and treatment features in RA patients registered in a biologic registry. Methods HUR-BIO (Hacettepe University Rheumatology Biologic Registry) is a prospective, single center database of biological treatments including 1229 RA patients by August 2016. Demographics, clinical and serological features of patients and thorax CT results performed in Hacettepe University between 2000 and 2016 were retrospectively evaluated. Patients whose CT was reported as small airway disease or ILD by radiologists, were evaluated as a subgroup. Data included type of biological treatments before diagnose of ILD and at last control as well. Results Among 1229 patients (78.8% female), mean age was 53.3±12.9 and disease duration was 11.4±7.9. Thorax CT had been performed in 203 (16.5%) patients. Distribution of pathologic findings in thorax CT was represented in Table 1. 29 patients (%2.4) were diagnosed as ILD with regard to CT findings. Patients with ILD were older (64.5±6.7 vs 60.6±10.1, p=0.039) and more frequently positive for rheumatoid factor (RF) (25/27 (%92.6) vs 77/121 (%63.3), p=0.003) than patients without ILD. ILD was detected before beginning of biologics in 11 (37.9%) patients and during biologic treatment in 18 (62.1%) patients. Honeycombing was seen in 10 (34.5%) patients. First choice of biologic agent was RTX in 10 (34.5%) and TNFi in 19 (65.5%) patients. In ILD patients, mean follow-up duration was 51±31 months and first biologic was switched to another in 16 (55.2%) of patients. At the last follow-up visit 19 (65.5%) patients were on rituximab, 3 (10.3%) patients were on abatacept and 7 (24.1%) were on TNFi. Small airway disease was detected in 19 (9.3%) patients. History of smoking was present in only 4 (21.0%) of patients with small airway disease. Conclusions Bronciectasia, rheumatoid nodules, small airway disease and ILD were the most frequent CT findings of RA patients. Rituximab was the choice of first biologic treatment in one third of patients, however two-thirds of patients were on rituximab therapy at the last follow-up visit. Lung involvement stands out as an important factor in selection of biologics. Disclosure of Interest None declared

Background A significant part of patients with inflammatory arthritis are at their reproductive ages. Biological drugs are one of the important treatment options for inflammatory artritis which can cause fetal morbidity and mortality. Management of arthritis during pre-conceptional period is one of the challenges in rheumatology. Objectives This study aims to assess whether patients under biological treatment share their conception intents with their physicians. Methods 1580 patients admitted to our outpatient clinic between July 2015 and July 2016 who were received biological treatment. A questionnaire was performed to 373 patients who are at their reproductive ages. Patients were asked whether they or their wifes had got pregnant after the start of biological agent. If they had got, they were asked; 1. Was the pregnancy intented? 2. Did your physician know if you were going to get pregnant while taking a biological agent? Results There were total of 79 patients who or whose wifes had got pregnant after the start of biological agent. 34 (%43) out of 79 were female and 45 (%57) out of were male. Mean age of patients were 35.1 (5.3). Median disease duration was 10 (IQR=9) years. 24 (30.4%) pregnancies out of 79 were not planned [15 female (44.1%), 9 male (20%), p=0.021]. In addition, 43 (54.4%) out of 79 patients did not share their pregnancy plans with their physicians [15 (44,1%) female and 28 (62,2%) male (p=0.110)]. 28 (35.4%) of all patients stated that their disease was active during pre-conceptional period [20 (58,8%) female patients, 8 (17,8%) male patients, (p Conclusions In our study group, almost one third of the patients had unplanned pregnancies particularly with in female patients. One third of the patients were in active state before conception according to patients9 report. Almost half of the patients did not share their plan of pregnancy with their physicians. We suggest that in routine practise, physicians should ask plan of pregnancy in every clinical visit. Disclosure of Interest None declared

Background Abatacept (ABA), a T lymphocyte blocker, is one of the treatment options in rheumatoid arthritis (RA) patients who are resistant to initial therapy with non-biologic disease modifying drugs (DMARDs). Objectives Aim of this study was to evaluate the effectiveness, safety and drug survival rates of ABA in RA patients registered in HUR-BIO (Hacettepe University Rheumatology Biologic Registry) database. Methods HUR-BIO is a prospective,single center database of biological treatments including 1229 RA patients by August 2016. In total, 247 of patients had received ABA and in 175 (71%) of them ABA was the first biological agent. In this analysis, 158 patients were evaluated due to lack of first follow-up visit in 17 patients. Demographics, clinical and serological data were evaluated. DAS-28 and HAQ-DI scores before ABA and last follow-up visit were also assesed. Kaplan-Meier analysis was used to estimate drug survival rates. Results Among 175 (82% female) patients, mean age was 53.6±11.3 and mean disease duration was 10.3±7.3. Seropositivity for RF and/or ACPA was present in 74.8% of patients. Table 1 represents DAS-28 scores of patients before ABA and last follow-up visit. Mean duration from ABA initation to first and last follow-up visit was 4.5±3.1 and 15.1±10.2 months, respectively. Female gender was found as a risk factor in terms of remaining of DAS-28 above 3.2 at first control [OR 5,63 (%95 CI 1,72–18,41)]. In first follow-up visit, improvement in DAS-28 score of ≥1,2 from baseline was more frequent in ACPA positive patients (48/62 (77.4%) vs 17/33 (51.5%), p=0.01). HAQ score of >1.0 was observed in 57.8%, 36.3%and 26.1% of patients at baseline, first follow-up visit and last follow-up visit, respectively. ABA had been cessated in 60 (38.0%) patients during follow-up. The reason for ABA withdrawal was primary unresponsiveness in 22 (36.6%) patients and infection in 2 (3.3%) patients. Drug survival for ABA was shown in Figure 1. Log- rank between RF positive and negative patients was found as 0.067. Conclusions In our database, 70% of RA patients received ABA were biologic naive. Remission or low-disease activity was achieved approximately 50% of patients in first follow-up visit with an average of 4.5 months after begining of the treatment. Functional improvement was observed in two thirds of patients. Death or life threatening side effects were not seen during follow-up. ABA is an acceptable treatment option in DMARD resistant biologic naive RA patients. Disclosure of Interest None declared

Background Turkish Society of Rheumatology (TSR) proposed a guideline of recommendations for viral hepatitis screening before biological therapy in 2015. 1 Objectives The aim of this study was to assess results of viral hepatitis serological tests performed before biologics in RA patients. Methods HUR-BIO (Hacettepe University Rheumatology Biologic Registry) is a prospective,single center database of biological treatments including 1229 RA patients by August 2016. Results of serological tests for HBV of 468 RA patients were evaluated. The guideline includes 5 groups according to hepatitis B virus (HBV) serology; group 1 –HBV seronegative [HbsAg (-),anti HbS (-), anti-Hbc total (-)], group 2-vaccinated [HBsAg (-), anti-HBs (+), anti-HBc total (-)], group 3-previous HBV infection [HBsAg (-), anti-HBs (-), anti-HBc total (+)], group 4- Chronic HBV infection [HBsAg (+), anti-HBs (-), anti-HBc total (+)], group 5- secondary immunity [HBsAg (-), anti-HBs (+), anti-HBc total (+)]. Patients were also classified according to risk for HBV reactivation as very high risk (Group 4 patients receiving rituximab), high risk (group 3 patients receiving rituximab) and medium risk (Group3 or Group 4 patients receiving TNFi or T-cell blockers,IL12/23 pathway inhibitor). Screening results for hepatitis C virus (HCV) were also evaluated. Results Among 1229 patients (79.7% female), mean age was 54.9±11.7 and mean disease duration was 12.3±8.2 years. In total, 104 (22.2%) of patients had received rituximab. Serology for HbsAg, anti-Hbs and anti-HbC were avaliable in 468 patients. There were 273 (58.3%) patients in group 1, 81 (17.3%) in group 2, 19 (4.1%) in group 3, 9 (1.9%) in group 4 and 86 (18.3%) in group 5. Table 1 represents distribution of patients according to HBV reactivation risk. Anti-HCV was positive in 21 (2.5%) of patients. Conclusions Among patients registered in HUR-BIO, 1.9% had chronic HBV infection and 2.5% anti-HCV positivity. 5.0% of them were under high and/or very high risk for HBV reactivation. Medium risk group which includes 17.0% of patients, seems to deserve most of the attention. Screening for viral hepatitis must be performed before biologics and TSR guideline may be useful in this matter. References Karadag O, Kasifoglu T et al. Guideline of the viral hepatitis screening before biologic agents use in patients with rheumatic diseases. RAED Journal;7:28–32. Disclosure of Interest None declared