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Objective: Deep vein thrombosis (DVT) of the lower extremities is the most common form of vascular involvement in Behcet disease (BD), frequently leading to post-thrombotic syndrome (PTS) as a disabling complication. We have described the clinical characteristics and predictors of PTS presence among patients with BD and lower extremity DVT. We also used venous Doppler ultrasound (US) examinations in our assessment. Methods: Patients with BD (n = 205; 166 men, 39 women; age 39 6 9.5 years) and a history of DVT were investigated. The Villalta scale was used to assess the presence and severity of PTS. Doppler US examinations were performed within 1 week of the clinical evaluation. The total number of vessels with reflux, thrombi, recanalization, and collateral vessels were calculated. Results: Of the 205 patients with BD, 62% had had PTS and 18% had had severe PTS. Patients with PTS had had greater reflux (P = .054) and thrombosis (P = .02) scores compared with patients without PTS. Treatment with anticoagulation (AC), immunosuppressive (IS) therapy, or AC combined with IS drugs did not affect the occurrence of PTS. However, patients treated with IS therapy, with or without AC drugs, had a decreased incidence of severe PTS compared with the AC-only group (P = .017). Patients treated with AC plus IS agents also had increased collateral scores compared with patients treated with only IS drugs. Interferon-a use seemed to provide better recanalization scores compared with azathioprine only (1.0 [range, 0-14] vs 2.5 [range, 0-10]; P = .010). Conclusions: Patients with BD and DVT have a high risk of developing severe PTS. IS treatment decreases the development of severe PTS. AC therapy might influence the course of PTS by increasing the collateral scores, and the use of interferon-a also increased recanalization scores. Routine assessment with Doppler US examinations could be helpful in the prediction of severe PTS. (J Vasc Surg Venous Lymphat Disord 2021;9:1451-9.)

Objective: Inflammation markers increase during Familial Mediterranean Fever (FMF) attacks and often return to normal within a few days. It is known that a subclinical inflammation continues in FMF patients in the inter-episode period, which increases the risk of developing amyloidosis. In recent years, several works have been conducted showing the relationship between platelet indices and inflammation, such as platelet count (PLT), platelet distribution width (PDW), mean platelet volume (MPV), plateletrite (PCT). In this study, we aimed to investigate the relationship between platelet indices and disease severity in FMF patients without attacks. Method: The records of all FMF patients followed by us were reviewed retrospectively. Patients with a hematological or oncological disease, patients with data deficiency for the study, and patients under 18 years of age were excluded. While the laboratory data were recorded, the condition of patients to be at least 3 months without attack was required. As the control group, the automation records of all healthcare professionals working in the internal medicine clinic of our hospital were examined. Our clinical staff, who did not have chronic diseases and regular drug use, were taken as a healthy control group. 173 patients and 20 control groups who met the conditions mentioned above were included in the study. Platelet indices were compared between patient and control groups. Results: It was determined that the value of MPV, one of the platelet indices, in the patient group was statistically significantly higher than the value in the healthy control group (p = 0.029). MPV was also found to correlate with disease severity (p

Background/aim: Familial Mediterranean Fever (FMF) is the prototype of hereditary autoinflammatory disorders and caused by mutations on the MEFV gene located on the short arm of chromosome 16. Although some MEFV variants are clearly associated with disease phenotype, there are numerous variants with unknown clinical association which are termed as variants of uncertain significance (VUS). Here, we present clinical correlations of VUS in a large cohort of adult FMF patients from three tertiary centers located in Central Anatolia. Materials and methods: All patients were recruited from FMF in Central Anatolia (FiCA) cohort. Demographic (sex, age at disease onset) and clinical features (disease characteristics, attack frequency, mean colchicine dose, colchicine nonresponsiveness, amyloidosis, and persistent inflammation) of patients with VUS were compared with those harboring pathogenic variants. Disease severity and damage were also evaluated using international severity score for FMF (ISSF) and autoinflammatory disease damage index (ADDI), respectively. Results: Among 971 participants included, MEFV gene analysis results were available for 814 patients. Twenty-six (3.2%) patients had single heterozygous VUS and 54 (6.6%) had pathogenic/VUS complex heterozygous variants. Patients with single heterozygous VUS had similar demographic/clinical features, ISSF and ADDI scores compared to those with single heterozygous pathogenic variant (p > 0.05 for all). No difference was observed in the demographic and clinical features of patients with single heterozygous pathogenic mutation and pathogenic/VUS complex heterozygous variant (p > 0.05 for all). ISSF and ADDI scores were lower in pathogenic/VUS complex heterozygous patients than those harboring single pathogenic mutation (p = 0.006 and 0.004, respectively). Conclusion: Our findings suggest that patients with single heterozygous VUS has mild FMF phenotype similar to those with single pathogenic mutation. Pathogenic/VUS complex heterozygosity does not lead to a more severe clinical phenotype than having a single pathogenic variant.

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.

Background/aim Colchicine is the mainstay of treatment in FMF. However, in daily practice it is not easy to maintain effective colchicine doses in a substantial number of patients due to its side effects. In this study, we aimed to investigate prevalence and risk factors for colchicine side effects that limit optimal drug dosing and cause permanent discontinuation. Materials and methods All patients were recruited from “FMF in Central Anatolia” (FiCA) cohort, 915 adults with a minimum follow- up time of 6 months during which they had obeyed all treatment instructions. 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 five (0.5%), and allergic skin reaction in two. Colchicine had to be permanently ceased in 18 (2%) patients because of serious toxicity. Male sex and obesity were found to be associated with liver toxicity, and having a 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 is associated with complications.

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

AMAÇ: COVİD-19 hastalığı; güçlü bulaşıcılığa ve ağır patojeniteye sahip bir hastalıktır. COVİD-19 hastalığı için pek çok risk faktörü tanımlanmıştır. Sigara içiciliği bu risk faktörlerinden bir tanesidir. Bu çalışma COVİD-19 pandemisi boyunca enfekte hastalar ile yakın temas halinde olan sağlık çalışanlarının sigara içme alışkanlığının nasıl değiştiğini ve bu değişikliklere neden olan faktörlerin neler olduğunu ortaya koymak için yapılmıştır.GEREÇ VE YÖNTEM: Çalışma 3. basamak olarak hizmet veren bir pandemi hastanesinin sigara kullanan 242 sağlık personeline 15 - 30 Mart 2021 tarihleri arasında çevrimiçi bir anket uygulanarak gerçekleştirilmiştir. Anket yaş, cinsiyet, meslek gibi sorular ile başlamakta; COVİD-19 geçirip geçirmeme, COVİD-19 döneminde sigara içme alışkanlığının nasıl değiştiğine yönelik sorular ile devam etmekte ve Fagenstrom Nikotin Bağımlılık anketi ile sonlanmaktadır. İstatiksel analiz SPSS 26.0 paket programı ile yapılmış ve gruplar arasında kategorik değişken karşılaştırmasında ki-kare testi kullanılmıştır.BULGULAR: Katılımcıların 131’i erkek (%54.1), 111’i kadın (%45.9)dı. 90 kişi (%37.2) doktor,152 (%62.8) kişi yardımcı sağlık personeli idi. Katılımcılar içerisinden 58 kişi (%24) COVİD-19 geçirmişken, 184 kişi (%76) COVİD-19 geçirmemiş idi. COVİD-19 pandemisi seyri boyunca katılımcılar içerisinden 55 (%22.7) kişini sigarayı bıraktığı öğrenildi. Bu dönemde sigarayı bırakan 55 kişinin Fagerström nikotin bağımlılık testine göre 53 tanesinin çok az bağımlı-az bağımlı olduğu görülürken, 2 tanesinin yüksek ya da çok yüksek bağımlı olduğu görüldü.SONUÇ: COVİD-19 pandemisi sağlık çalışanlarının sigara içme alışkanlığı üzerinde istatiksel olarak anlamlı bir farklılık oluşturmamıştır. Sağlık çalışanlarının bu dönemde sigara içme alışkanlığında nikotin bağımlılıkları doğrudan bir rol oynamıştır., OBJECTIVE: COVID-19 disease; It is a highly contagious and highly pathogenic disease. Many risk factors have been identified for the COVID-19 disease. Smoking is one of these risk factors. This study was conducted to reveal how the smoking habits of healthcare workers who were in close contact with infected patients during the COVID-19 pandemic changed and what factors caused these changes.MATERIAL AND METHODS: The study was carried out by applying an online questionnaire to 242 smokers of a pandemic hospital serving as a third step between 15 - 30 March 2021. The survey starts with questions such as age, gender, occupation; Whether or not to have COVID-19 continues with questions about how smoking habits have changed during the COVID-19 period and ends with the Fagenstrom Nicotine Addiction questionnaire. Statistical analysis was done with SPSS 26.0 package program and chi-square test was used to compare categorical variables between groups.RESULTS: Of the participants, 131 (54.1%) were men and 111 (45.9%) were women. 90 people (37.2%) were doctors and 152 (62.8%) were assistant health personnel. Among the participants, 58 (24%) had COVID-19, while 184 (76%) had not had COVID-19. It was learned that 55 (22.7%) of the participants quit smoking during the course of the COVID-19 pandemic. According to the Fagerström nicotine addiction test of 55 people who quit smoking during this period, 53 of them were found to be very little addicted-low addicted, while 2 of them were highly or very highly addicted.CONCLUSIONS: The COVID-19 pandemic did not make a statistically significant difference on the smoking habits of health- care workers. Nicotine addictions played a direct role in the smoking habits of health workers in this period.

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

Objective: Pulmonary hypertension (PH) is a one of the major causes of death in patients with systemic sclerosis (SSc). In this study, we in- vestigated the impact of updated hemodynamic definition proposed by the 6th PH World Symposium (6th WSPH) on the frequency of PH and its subtypes in patients with SSc. Methods: Patients with SSc admitted between 2015 and 2019 and who underwent right heart catheterization (RHC) were included. The frequen- cy of PH and its subgroups based on the hemodynamic definitions of both 2015 European Society of Cardiology/European respiratory Society (ESC/ERS) PH guidelines and 6th WSPH was compared. Results: Of the 65 patients with SSc, 23 (35.4%) had normal mean pulmonary arterial pressure (mPAP), 20 (30.8%) had mildly elevated mPAP (21–24 mm Hg), and 22 (33.8%) had PH [pulmonary arterial hypertension (PAH) (n=16, 24.6%), group 2 PH (n=5, 7.7%), group 3 PH (n=1, 1.5%)] ac- cording to the 2015 ESC/ERS PH definition. Based on the updated criteria, 7 (10.8% of total cohort) additional patients were reclassified as having PH [PAH (n=3), group 2 PH (n=3), group 3 PH (n=1)]. Conclusion: The impact of the updated definition on the frequency of PH and PAH in our cohort was greater than previously reported, which may be caused by the difference in screening strategies for PAH.

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/aim: Peritonitis attacks of Familial Mediterranean Fever (FMF) usually requires emergency medical admissions and it's hard to distinguish a typical abdominal attack from surgical causes of acute abdomen. Therefore, history of abdominal surgery, particularly appendectomy, is very common in patients with FMF. However, history of appendectomy might also give some clues about the course of FMF in the adulthood. This study was to determine whether the history of appendectomy help to anticipate disease course of FMF in the adulthood. Materials and methods: All patients recruited from FMF in Central Anatolia (FiCA) cohort, comprising 971 adult subjects. All patients fulfilled the Tel Hashomer criteria. Demographic data, FMF disease characteristics, co-morbid conditions, past medical history, surgical history and disease complications were meticulously questioned and laboratory features and genotype data (if available) were recruited from patient files. Results: Appendectomy history was evident in 240 (24.7%) subjects. Disease onset was earlier and peritonitis is strikingly more prevalent (97.1% vs. 89.6%, p < 0.001) in appendectomized patients. These patients had reported almost two fold more frequent attacks in the last year compared to appendix intact patients (median 3.5 vs. 2 attacks, p = 0.001) without a difference in frequency of musculoskeletal and skin attacks. Severe disease was more common (10% vs. 5.9%, p = 0.038) due to involvement of more attack sites throughout the life and more frequent attacks. Appendectomy patients had used higher daily doses of colchicine to control disease (1.43 +/- 0.6 mg vs. 1.27 +/- 0.52 mg, p = 0.002) but colchicine resistance was also more common in these patients, 15% vs. 6.7% respectively, p < 0.001. Conclusion: Appendectomy history is common in FMF patients and associated with frequent serositis attacks in adulthood. These patients require higher colchicine doses with a lower rate of response and more need for Interleukin-1 antagonist therapies.

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.

The aim of the present study was to examine the effects of age on mucocutaneous activity by using moderation analysis in Behçet’s syndrome (BS). In this cross-sectional study, 887 BS patients (female : male, 481:406; mean age, 38.4 ± 10.9 years) followed in 13 tertiary centers in Turkey were included. Mucocutaneous activity was evaluated by using the Mucocutaneous Index (MI) according to sex and disease course. Moderation analysis was performed to test the effect of age on mucocutaneous activity. A moderator variable is a third variable and affects the relationship between independent and outcome variables. Age was chosen as a potential moderator variable (interaction effect), MI score as the outcome variable and sex as an independent variable in the analysis. The moderation analysis tested the effects of age in three steps: whole BS patient group, patients without systemic involvement and those with systemic involvement. The moderation model was only significant in BS patients with systemic involvement (P = 0.0351), and a significant relationship was observed between female sex and MI score (P = 0.0156). In addition, the interaction plot showed that female patients had increased MI scores compared with male patients, especially in the 28-year-old age group (P = 0.0067). Moreover, major organ involvement was newly diagnosed in the majority of these young female BS patients. Our results suggest that the relationship between sex and mucocutaneous activity was moderated by age in the systemic involvement group. Also, increased mucocutaneous activity may be associated with new major organ involvement in young female BS patients with systemic involvement. © 2020 Japanese Dermatological Association

Background/aim: Tumour necrosis factor inhibitors and anti-interleukin-6 (anti-IL-6) therapies are increasingly being used in Takayasu’s arteritis (TA) patients who are unresponsive to corticosteroids ± conventional immunosuppressive agents. The aim of this study is to assess the efficacy and safety of anti-IL-6 (tocilizumab) therapy in refractory TA patients in real life. Materials and methods: Fifteen TA patients (86.7% were female) who received at least 3 cycles of tocilizumab therapy were retrospectively assessed by clinical, laboratory, and radiological evaluations before and after tocilizumab therapy. Results: The median (min–max) age of the patients at evaluation was 35 (20–58) years and the median disease duration from diagnosis was 24 (12–168) months. The median (min.–max.) duration of follow-up after tocilizumab was 15 (3–42) months. There was a significant decrease in erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and patient global visual analogue scale (VAS) scores of patients after tocilizumab therapy. The median (min.–max.) ESR was 26 (5–119) vs. 3 (2–49) mm/h, P = 0.02; CRP was 39.8 (2.4–149.0) vs. 7.9 (0–92.9) mg/L, P = 0.017; and patient global VAS was 50 (0–90) vs. 30 (0–60), P = 0.027, respectively. In 8 patients, ESR and CRP levels were in the normal range in the last control. Imaging modality results after tocilizumab were available for 9 patients; 8 patients were radiologically stable and regression was seen in 1 patient. Comparable imaging modality results before and after tocilizumab were available for 5 patients; 4 patients were radiologically stable and regression was seen in 1 patient. Radiological findings were consistent with laboratory responses. Glucocorticoid dosages decreased from a mean dosage of 16.2 (9.1) mg/day at baseline to 7.1 (3.8) mg/day (P = 0.001) at the last follow-up visit. There was no increase in the steroid dosage in any of the patients. All patients tolerated tocilizumab well. Conclusion: Based on retrospective real life data, anti-IL-6 (tocilizumab) appears to be an effective and tolerable treatment option in refractory TA patients.

Background Free fascial flaps are widely used for the reconstruction of defects located on the distal extremities as they provide thin yet durable soft tissue coverage. The serratus anterior fascia flap stands out from other fascial flap alternatives as it has optimal anatomical properties that can provide ideal coverage in a wide range of reconstructive challenges. The purpose of this study was to present our experience with the serratus anterior fascia flap in a variety of complex soft tissue defects. Methods A retrospective review was conducted on patients who underwent reconstruction with the free serratus anterior fascia flap for complex soft tissue defects involving the distal extremities. Results Complex soft tissue defects of 13 patients were reconstructed using the free serratus fascia flap. No major postoperative complications such as flap loss, dehiscence, infection, or hematoma were encountered in any of the patients. All of the patients demonstrated durable and harmonious long-term flap coverage that facilitated movement without any signs of scar contractures and tendon adhesions at the site of reconstruction. Conclusions The serratus anterior fascia flap demonstrates versatility and stability for the closure of complex distal extremity defects. It creates thin yet durable coverage that facilitates underlying tendon gliding while providing harmony with the natural contours of these anatomic areas and has low donor site morbidity, making it a primary choice in the reconstruction complex soft tissue defects involving the hands and feet.

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: Relapsing polychondritis (RP) is a rare autoimmune disorder, and myelodysplastic syndrome (MDS) is accompanied by RP at variable rates. Herein, we report a case with RP and MDS who responded dramatically to 5-azacitidine for MDS. Case presentation: With conventional immunosuppressive treatment, our patient had several episodes of different side effects, including infections. With the diagnosis of MDS and initiation of azacitidine treatment, all the manifestations of RP disappeared, and remission was achieved for MDS. Although he had relapses of either RP or MDS after several years of azacitidine treatment, all relapses were controlled well with the initiation of azacitidine treatment every time. Conclusions: Azacitidine should be kept in mind as a treatment option for RP patients with MDS.

Oxaliplatin is a third-generation platinum anti-neoplastic agent, which is used in the treatment of colorectal, gastric, and biliary tract cancers with combination other chemotherapy drugs. In rare cases, thrombocytopenia may occur suddenly in approximately 24 hours due to immune-mediated reactions. This reaction is usually seen after long term usage of oxaliplatin-based chemotherapy. Here, we present our case of immune-mediated thrombocytopenia associated with oxaliplatin. A 56 years old man who had stage IV rectum cancer with liver metastasis, had palliative surgery due to bowel obstruction after initial diagnostic approach. After failure of first line treatment, mFOLFOX6 plus Cetuximab (Cetuximab: 500mg/m2 on day 1, Oxaliplatin: 85 mg/m2 day 1, Leucovorin (LV): 400 mg/m2 day 1, 5-FU 400 mg/m2 IV bolus on day 1 followed by 2400 mg/m2 on day 1 infused over 46 hours every two weeks) regimen was started. Before the 21st cycle of oxaliplatin plus cetuximab regimen, the patient had normal thrombocyte, haemoglobin and neutrophile count. Approximately 8 hours after the chemotherapy patient had been taken to the emergency service with petechia, vigorous gastrointestinal and nasal bleeding. The thrombocyte levels were 3000 at μl. The patient was given methylprednisolone for three days. The thrombocyte levels recover quickly in 24 hours. After this reaction, oxaliplatin stopped and the chemotherapy was changed to capesitabine plus cetuximab regimen. In conclusion, oxaliplatin induced immune-mediated thrombocytopenia is a rare side effect but a life threatening complication. A physician who uses oxaliplatin as a treatment option should keep in mind the possibility immune-mediated thrombocytopenia which may cause life-threatening bleeding. Especially the long term use of oxaliplatin (median >10 cycles) may alert the physician for immune-mediated adverse effects.

Background/aim: Since the majority of the IgG4-related disease (IgG4-RD) patients in the literature are from the Far East and the United States, there is a lack of large series from other parts of the world. We aimed to identify the clinical characteristics and outcome of Turkish IgG4-RD patients from a tertiary center. Materials and methods: Fifty-two patients classified as having definite IgG4-RD according to comprehensive diagnostic criteria were included in the study. Patients not fulfilling the definite criteria due to lack of pathologic specimen and/or serum IgG4 levels were excluded (n = 47). Clinical, laboratory, and histopathological features and treatment approaches were analyzed. Results: Median age at diagnosis was 51.1 years and sex predominance was not observed (male/female: 26/26). Median follow-up duration was 18 (IQR 25–75: 8–35) months. Retroperitoneal fibrosis was the most frequent presentation. Twenty-four (46.1%) patients had localized involvement. Corticosteroids were the mainstay of treatment (92.5%). Rituximab had been used for cases resistant to previous treatment or with relapses in 19 (47.5%) patients. A complete response was achieved in 52.5% and partial response (

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

Background/Aim Neurological involvement (Neuro-Behcet's Disease: NBD) is a rare manifestation of Behcet's Disease (BD) and it is related with significant mortality and morbidity. We aimed to evaluate disease course and outcome of NBD patients registered in Hacettepe University Vasculitis Center (HUVAC) prospective database starting from October 2014. Methods Totally, 419 patients (329 of the patients had fulfilled the International Study Group (ISG) criteria and 90 patients were considered as incomplete BD) were recorded as BD to March 2018. We retrospectively reviewed the charts of 123 patients with neurological complaints/ symptoms according International Consensus Recommendations (ICR) Criteria for Neuro-Behcet's disease. In final analysis, 77 NBD patients (Definite NBD = 61, possible NBD = 16) were included. Demographics, clinical features, treatment characteristics, disability status and survival status of the patients were evaluated. Results Forty-seven (61%) of the patients were male. Median time to neurological involvement from first diagnosis of BD is 6 (IQR = 8.8) years in patients who had diagnosis of BD before neurological involvement. Distribution of NBD: parenchymal (pNBD), non-parenchymal (npNBD), mixed (mNBD) and peripheral nervous system (pnsNBD) were 47 (61%), 22 (28.6%), 5 (6.5%), 3 (3.9%), respectively. Eye involvement was more frequent in pNBD compared to npNBD. Brainstem (72.9%) was the most frequently affected parenchymal area and followed by cerebellum (43.8%) and diencephalon (37.5%). Twelve patients had spinal cord involvement (n = 12, 24.5%). Among the patients with pNBD and mNBD (total n = 52), 48 patients were considered as acute onset parenchymal disease and 4 patients were evaluated as chronic progressive disease. Fifty-eight percent of the patients with acute onset parenchymal disease had only one attack. Totally 14 BD patients deceased during a median 9.4 (IQR = 13) years disease duration and 9 of these patients had NBD (pNBD = 6, mNBD = 2, pnsNBD = 1). Corticosteroids (IV pulse = 75.5% and oral medium-high dose = 90%), alpha-interferon (76.9%), cyclophosphamide (57.1%), and TNF inhibitors (23.5%) were the most frequently preferred treatment options for pNBD. Conclusions Neurological involvement is seen about 5 years after the diagnosis of BD, and ocular involvement more commonly seen in these patients than non-NBD patients. More than half of the patients with acute onset parenchymal NBD had only one attack. No death was observed in the patients with non-parenchymal NBD. Biologic agents (Interferon-alpha and anti-TNF agents) were used in most patients.

Background/aim: Abnormal capillaroscopic findings have been reported in vasculitic syndromes such as Behçet's disease, Henoch-Schönlein purpura, and Wegener's granulomatosis. This study was conducted to define the capillaroscopic changes in patients with Takayasu arteritis (TA). Materials and methods: We studied 28 TA patients (27 females). The nail folds from the 2nd to 5th fingers on both hands were examined with video capillaroscopy for all. A patient was defined as having an abnormal capillaroscopic examination if more than 1 morphologic abnormality was present in at least 2 nail folds. Results: The median capillary density of TA patients was 9 (range: 9-11). There were no patients with capillary disorganization or avascular areas. Tortuous capillaries were detected in all patients. The other common morphological capillary abnormalities included enlarged/dilated capillaries (39.3%), branching capillaries (35.7%), and hemorrhages (32.1%). Only 1 patient had giant capillaries with early scleroderma-like pattern. Overall, there were 11 (39.3%) patients with abnormal capillaroscopic findings. There were more patients with abnormal capillaroscopic findings in the subgroup of TA patients whose upper extremity blood pressure could not be measured as compared to those whose blood pressure could be measured (66.7% vs. 26.3% patients; P = 0.04). Conclusion: Capillaroscopic abnormalities are frequently seen in TA patients. We consider that abnormal capillaroscopic findings in TA patients reflect the impaired blood flow due to narrowed or occluded arteries rather than the primary capillary involvement of the disease process.

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: Familial Mediterranean Fever (FMF) is the most frequent auto-inflammatory disease characterized by recurrent, self-limiting attacks of fever, peritonitis, pleuritis, synovitis, myalgia, and erysipelas-like erythema (ELE). Attacks has a robust inflammatory response which completely normalize when attacks subside. However, substantial number of FMF patients possess chronic persistent inflammation even in between attacks. Clinical significance of persistent inflammation and its contribution to damage accrual are yet to be determined. Objectives: We aimed to determine the prevalence and underlying factors of chronic persistent inflammation and its association with the domains of Auto-inflammatory disease damage index (ADDI) in a large cross-sectional multicenter cohort. Methods: All patients recruited from FMF in Central Anatolia (FiCA) cohort, which is a duplication disabled, internal and externally controlled, cross-sectional, multicenter accessible web-based cohort. Demographic data, disease characteristic, attack features (ever presented) were meticulously questioned. Genotype data (if available) and laboratory features including inflammatory markers were recorded. FMF related damage scores was assessed by auto-inflammatory disease damage index (ADDI) which is recently validated. Patients were stratified according to their antecedent acute phase responses as having persistent inflammation (PI group) or not, both groups compared in terms of disease characteristics, attack features, inflammatory comorbidities and damage domains. Results: Study is comprised 970 adult patients (mean age 35.3±12.1, 61% female). 54 of them were excluded for their first inclusion. 15% of patients had persistent inflammation. PI group had significantly younger age of diagnosis, male dominance, homozygous M694V, more frequent attacks it the last year, more pleuritis, arthritis, myalgia and ELE and more severe disease according to ISSF than other patients (Table-1). Moreover, colchicine resistance and ADDI damage scores were remarkably higher in PI group. In multi-variate analyzes colchicine resistance OR=2.71 [95%CI 1.38-5.35], ISSF OR=11.06 [95%CI 5.23-23.39], male gender OR= 2.38 [95%CI 1.51-3.76], homozygous M694V mutation OR= 2.31 [95%CI 1.41-3.78] and ELE OR= 1.72 [95%CI 1.04-2.857] found as independent predictors of persistent inflammation. PI group had more damage in multiple domains like joint restriction, musculoskeletal pain, proteinuria, amyloidosis, renal insufficiency and developmental delay. Same variables and persistent inflammation further analyzed in in terms of predicting the damage. Persistent inflammation found to be an independent predictor of proteinuria OR=2.02 [95%CI 1.05-3.90], amyloidosis OR=2.71 [95%CI, 1.34-5.48]), and renal insufficiency OR=4.36 [95%CI 1.84-10.32]. Conclusion: Persistent inflammation is relatively common in FMF patients particularly in those harboring M694V homozygous mutation and is associated with major complications of disease indicating poor prognosis. Acknowledgement: None Disclosure of Interests: Hakan Babaoglu: None declared, Berkan Armagan: None declared, Erdal Bodakci: None declared, Timucin Kasifoglu: None declared, Hasan Satis: None declared, Nuh Atas: None declared, Alper Sari: None declared, Nazife Sule Yasar Bilge: None declared, Gozde Kubra Yardimci: None declared, reyhan bilici salman: None declared, Levent Kilic: None declared, mehmet akif ozturk: None declared, Berna Goker: None declared, seminur haznedaroglu: None declared, Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, abdurrahman tufan: None declared

Background: Arterial aneurysmatic lesions are one of the unique features of Behcet’s disease (BD). However, it is difficult to determine which BD patients will develop an aneurysm. Objectives: In this study, we aimed to determine the differences between BD patients with and without an arterial aneurysm. Methods: Data of the 23 BD patients with an arterial aneurysm was published as an abstract in 2013 (1). We retrospectively reviewed the medical records of 441 patients with BD according to International Study Group (ISG) criteria between January 2013 and June 2018. Totally, we determined 45 BD patients with an arterial aneurysm. Six patients with isolated carotid and/or cranial arterial aneurysms who were followed by other clinics excluded from the analysis. Overall, 39 BD patients with an extracranial and extra carotidal aneurysm included in the study. Data regarding demographic features, clinical, laboratory and vascular imaging findings were collected. Results: A total of 39 BD patients (Male: 76.9%) with an arterial aneurysm were analyzed in this study. Mean age, mean age of diagnosis and a median follow-up of patients were 40.9±11.0 years, 29.7±7.7 and 71.8 (2-186) months, respectively. The mean age at the onset of the arterial aneurysm was 36.1±11.5 years. The median time lag between the onset of BD and detection of an aneurysm was 63.6 (0-482) months. The prevalence of male patients in BD with aneurysms, without aneurysms and only mucocutaneous were 76.9%, 52.3%, and 34.3%, respectively. Comparison of BD patients with and without an aneurysm shown in table 1. In multivariate analysis, having a venous thrombosis was the sole risk factor for the development of arterial aneurysm (OR 10.53, 95% CI 1.53-72.71) (Table). Distribution of aneurysms was shown in the figure. At the first diagnosis, a median number of the aneurysm was 1 (1-4), the median diameter of an aneurysm was 39 mm (10-80) and 16 (41%) patients had more than one aneurysm. Type of the firstly detected aneurysm was as follows; 38.5% saccular, 17.9% fusiform, 15.4% pseudoaneurysm and 2.6% dissecting aneurysms. Aneurysm type was not known in 25.6% of patients. The first diagnostic finding was an aneurysmal symptom in the 25% of BD patients. Conclusion: Aneurysm was the diagnostic symptom in 25% of BD patients with an aneurysms. The median time lag between the onset of BD and detection of an aneurysm was almost 5 years. In aneurysmal BD, there is male sex dominance and having venous thrombosis is the most important risk factor. Although pulmonary arteries have an important role in the BD course, the involvement of the aorta and iliac arteries can be seen frequently. References [1] Kalyoncu U, et al. Ann Rheum Dis 2013;72 (abs) Disclosure of Interests: Berkan Armagan: None declared, Ertugrul Cagri Bolek: None declared, Alper Sari: None declared, Gozde Kubra Yardimci: None declared, Bayram Farisogullari: None declared, Emre Bilgin: None declared, Fatma Gonca Eldem: None declared, Levent Kilic: None declared, Omer Karadag: None declared, Bora Peynircioglu: None declared, Barbaros Erhan Cil: None declared, Metin Demircin: None declared, Ali Akdogan: None declared, Sule Apras Bilgen: None declared, Ali Ihsan Ertenli: None declared, Sedat Kiraz: None declared, Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim

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: Arterial aneurysms are one of the unique features of Behcet’s disease (BD). Although arterial aneurysms are relatively rare, they carry risk of rupture and have poor prognosis. Objectives: To evaluate the medical and interventional therapies and long-term outcomes of BD patients with extracranial arterial aneurysms. Methods: We retrospectively reviewed the medical records of 441 BD patients according to ISG criteria between 2013 and 2018 at the Hacettepe University Vasculitis Center (HUVAC). We determined 45 BD patients with an arterial aneurysm. Six patients with isolated carotid and/or cranial arterial aneurysms who were followed by other clinics excluded from the analysis. Overall, 39 BD patients with an extracranial and extra carotidal aneurysm included in the study. Data regarding demographic characteristics, clinical, laboratory and vascular imaging findings, history of medical treatments and outcomes were collected. Vascular intervention of patients were grouped as surgery or endovascular intervention. Radiological response after vascular intervention divided into 3 groups as regression, stable and progression according to radiological evaluation. Regression was defined as a decrease or disappearance of the aneurysm. Stable was defined as no change in the aneurysm diameter. Progression was defined as an increase in diameter of a previous aneurysm or occurrence of a new vascular event. We colligated the stable and regression groups and compared with the progression group. Relapse was defined the progression of the aneurysm which underwent an intervention. Results: A total of 39 BD patients (Male, 80%) with an arterial aneurysm were analyzed in this study. Mean age and mean age of diagnosis patients were 40.9±11.0 and 29.7±7.7 years, respectively. The clinical features of BD was as follows; oral ulcer 100%, genital ulcer 80.6%, acneiform lesion 47.2%, erythema nodosum 33.3%, pathergy positivity 34.5%, arthritis 27.8%, fever 24.3%, uveitis 34.5%, neurological involvement 30.6% and, gastrointestinal involvement 7.7%. Distribution of arterial aneurysms were 5.1% subclavian, 5.1% coronary artery & cardiac, 38.8% pulmonary, 10.3% thoracic aorta, 33.3% abdominal aorta, 5.1% renal, 25.6% iliac and 12.8% femoral. Induction and maintenance treatments are shown in figure 1. In our cohort, 25 (64%) patients needed a vascular intervention, including 9 (23%) open operations and 16 (41%) endovascular interventions. Of 7 (18%) patients had to perform an urgent open operation or endovascular intervention but we didn’t find any relation between the progression of an aneurysm and urgent intervention. First induction treatment options and prognosis of arterial aneurysms shown in figure 2. At median 71.8 months follow up, 7 (18%) patient died in the BD aneurysm group. The rate of progression of the aneurysm was 20% in BD patients with pulmonary artery involvement and 64% in BD patients with aortic and its branch involvement (p=0.038). In the absence of medical induction treatment, 5 patients had progression, 1 had regression and 1 had no follow-up. Conclusion: Arterial aneurysms in BD have poor prognosis and high mortality. Glucocorticoids, cyclophosphamide and interferon-α are the most preferred treatments for induction and maintenance therapy for aneurysm of the BD. Approximately 2/3 of the patients required a vascular intervention. Clinical and radiological progression was more prominent in the aorta and its branches. As expected, there is more progression in interventional procedures without medical treatment. Disclosure of Interests: Berkan-Armagan: None declared, Ertugrul Cagri Bolek: None declared, Alper Sari: None declared, Gozde Kubra Yardimci: None declared, Bayram Farisogullari: None declared, Emre Bilgin: None declared, Fatma Gonca Eldem: None declared, Levent Kilic: None declared, Omer Karadag: None declared, Bora Peynircioglu: None declared, Barbaros Erhan Cil: None declared, Metin Demircin: None declared, Ali Akdogan: None declared, Sule Apras Bilgen: None declared, Ali Ihsan Ertenli: None declared, Sedat Kiraz: None declared, Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim

Background Fever of unknown origin (FUO) is one of the most challenging clinical situations. Although several studies showed a relatively benign course of patients remained undiagnosed, long-term outcome of patients with a certain diagnosis remain non-established (1). Objectives To describe the follow-up results of patients investigated for FUO and had a certain diagnosis. Methods Data from patients who admitted to Hacettepe University Hospitals, inpatients sections of department of the internal medicine with the complaint of FUO collected prospectively from January 2015 to October 2017. Patients with an uncertain diagnosis after all diagnostic procedures excluded. Patients were divided into 3 main subgroups: rheumatologic, infectious and malignant groups. We compared Kaplan-Meier curves for all diagnosis-to-death time frames with the standart log-rank test. p Results Total 106 patients were included, 58(55%) of them were female. Median age was 48 (18-81) years. Patients were also divided into three subgroups: rheumatologic (RHE) (n=49, 46.2%), infectious (INF) (n=28, 26.4%) and malignant (MLG) (n=29, 27.4%) causes; adult-onset Still’s disease (n=20; 41% of), tuberculosis (n=9; 32%) and lymphoma (n=19; 66%) were the most common diagnosis among groups, respectively. Mortality rates in decreasing order were RHE, INF and MLG groups, respectively. As an interaction was found between gender and survival, results of survival analysis were given as stratified according to gender. While there was no survival difference between RHE and INF groups among men, both of these groups had better survival than MLG group (ROM-INF p=0.13; ROM-MLG p=0,001; INF-MLG p=0,022). Among women, only there was a significant survival difference between RHE and MLG groups (ROM-INF p=0.15; ROM-MLG p=0,007; INF-MLG p=0,42). Conclusion Among patients evaluated for FUO, survival rate was higher in patients who had a rheumatological diagnosis. Further diagnostic algorithms are needed to identify these subgroups, because of the higher mortality among INF and MLG groups. References [1] Knockaert DC, Dujardin KS, Bobbaers HJ. Long-term follow-up of patients with undiagnosed fever of unknown origin. Arch Intern Med. 1996;156(6):618. Disclosure of Interests Emre Bilgin: None declared, Berkan Armagan: None declared, Alper Sari: None declared, Ertugrul Cagri Bolek: None declared, Bayram Farisogullari: None declared, Gozde Kubra Yardimci: None declared, Levent Kilic: None declared, Ali Akdogan: None declared, Omer Karadag: None declared, Sule Apras Bilgen: None declared, Ali Ihsan Ertenli: None declared, Sedat Kiraz: None declared, Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim

Background Familial Mediterranean Fever (FMF) is the most frequent auto-inflammatory disease caused by MEFV gene mutations. Available reports investigated only specific components of damage such as amyloidosis. All possible organ targets of damage have not been entirely evaluated before. Such as Disease severity index which is emerged especially for FMF do not cover entire damage domains related to FMF. Recently, a new scoring system (Auto-inflammatory disease damage index) was developed and validated for autoinflammatory diseases. Objectives We aimed to investigate damage accrual caused by FMF and associated features with damage. Methods All patients recruited from FMF in Central Anatolia (FiCA) cohort, which is a duplication disabled, internal and externally controlled, cross-sectional, multicenter accessible web-based cohort. This study is comprising 970 adult patients (mean age 35.3 ±12.1 years, 61.5% female). Demographic data, FMF disease characteristics, co-morbid conditions, disease complications were meticulously questioned and laboratory features and genotype data (if available) were recruited from patient files. FMF caused damage was assessed by auto-inflammatory disease damage index (ADDI) which is recently validated. Association between damage and demographic, disease and treatment characteristics were analyzed. Results Proportions of FMF manifestations were fever 83.1%, peritonitis 91.5%, pleuritis 47.9%, arthritis 43.3% and skin rash 26.2%. Dominant attack types were fever in 6.2%, serositis in 65.7%, musculoskeletal in 16.8% and all types of attacks were common in rest of patients. MEFV mutations were available in 814 subjects and 75.9% of these subjects were harboring M694V mutation (42.5% homozygous for M694V). Among all 63.1% patients were well responded to colchicine and 8.8% were non-responders. Median ADDI score was 1 (min 0-max 11). Most common FMF related damages were observed in musculoskeletal, reproductive and kidney domains. Chronic musculoskeletal pain was present in 49%, joint deformity in 2.9%, infertility in 6.6%, amenorrhea in 3.9%, proteinuria in 6.9%, amyloidosis in 5.9% and renal failure in 3.7% of the patients. 411 (%42.3) of patients had no damage accrual. M694V homozygous mutation, male gender and colchicine nonresponse were found to be the independent predictors of damage. Conclusion M694V homozygous mutation, colchicine non-response and male gender are predictors of damage and effective therapeutic interventions must be undertaken to prevent from damage in these patients. Acknowledgement None Disclosure of Interests Hakan Babaoglu: None declared, Berkan Armagan: None declared, Erdal Bodakci: None declared, Timucin Kasifoglu: None declared, Hasan Satis: None declared, Nuh Atas: None declared, Alper Sari: None declared, Nazife Sule Yasar Bilge: None declared, Gozde Kubra Yardimci: None declared, reyhan bilici salman: None declared, Levent Kilic: None declared, mehmet akif ozturk: None declared, Berna Goker: None declared, seminur haznedaroglu: None declared, Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, abdurrahman tufan: 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

Background: Enteropathic spondyloarthritis (eSpA) is one of the diseases in the Spondyloarthritis (SpA) spectrum and occurs in patients with inflammatory bowel disease (IBD). Sacroiliitis is frequently found in patients with IBD and can be overlooked because of focusing on IBD. Objectives: Aim of this study is to evaluate the general features of eSpA and compare with psoriatic spondylitis (PsA), and ankylosing spondylitis (AS). Methods: HUR-BIO (Hacettepe University Rheumatology Biologic Registry) is a prospective, single center database of biological treatments since 2005. eSpA patients were enrolled from HUR-BIO registry. Sacroiliitis was defined as modified New York criteria or based on ASAS magnetic resonance imaging criteria. Age and disease duration matched 128 ankylosing spondylitis and 96 psoriatic spondylitis patients were selected as a control group from HUR-BIO database. Demographic, clinical, laboratory, therapeutic data and imaging features were collected from this database: age, gender, age at disease onset, disease duration, type of IBD. Baseline disease activity before the first biologic therapy use was assessed with BASDAI, BASFI, VAS-patient global assessment, ESR and CRP. Results: HUR-BIO SpA registry included 2576 SpA patients, and 90 (3.5%) patients had enteropathic arthritis (EA). Sixty four of 90 (71.1%) patients had sacroiliitis according to modified NY criteria, and these patients were included in the study. Of the 64 patients with eSpA, IBD type was ulcerative colitis (UC) in 34 (53%) patients, Crohn’s disease (CD) in 30 (47%) patients. For eSpA patients, initial biological DMARDs were infliximab in 26 (40.6%), adalimumab in 23 (35.9%), etanercept in 10 (15.6%), golimumab in 4 (6.3%), and certolizumab in 1 patient (1.6%). The proportion of bDMARDs were similar with control group. Baseline disease activity were similar between eSpA and control group. However, baseline ESR levels were higher in eSpA than AS (p=0.037) and psoriatic spondylitis (p=0.001), as well. Baseline demographic and clinical features were summarized in Table 1. Conclusion: Enteropathic spondyloarthrtis was present only a small part of all SpA patients. Sex, SpA family history, and uveitis were different from other SpA subgroups. Disease activities were similar with other spondyloarthritis, but particularly ESR level was higher in eSpA probably due to bowel disease activity. Sacroiliac and spine involvement seems to be the main reason for starting bDMARD in IBD patients, rather than peripheral arthritis. Data were given as mean (standard deviation) or median (min-max) HLAB27 were assessed in 27 eSpA, 52 AS and 33 PSA patients. Disclosure of Interests: Gozde Kubra Yardimci: None declared, Bayram Farisogullari: None declared, Alper Sari: None declared, Levent Kilic: None declared, Berkan Armagan: None declared, Emre Bilgin: None declared, Ertugrul Cagri Bolek: None declared, Omer Karadag: None declared, Ali Akdogan: None declared, Sule Apras Bilgen: None declared, Sedat Kiraz: None declared, Ali Ihsan Ertenli: None declared, Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim