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Objective: Cardiovascular diseases (CVD) are very common in the general population. Atherosclerosis is the main pathogenesis. Familial Mediterranean fever (FMF) is an autosomal recessive disease. The gene causing FMF, designated MEFV, encodes a protein called pyrin or marenostrin that is expressed mainly in myeloid bone marrow precursors, neutrophils and monocytes. We herein aimed to determine the prevalence of MEFV mutations (all exon 2, 10 mutations) in patients with early coronary heart disease (early CHD) and coronary heart disease (CHD) with multiple risk factors and among the healthy subjects as controls., Methods: A total of 197 patients and 119 healthy subjects were recruited and enrolled into three groups in terms of inclusion criteria. Ninety-one patients diagnosed with early CHD enrolled into group one (men < 45 years of age, women < 40 years of age), 106 patients with CHD (men > 50 years of age) to group two and 119 healthy controls enrolled into group three. None of patients was diagnosed with FMF. The diagnosis of CHD was established on electrocardiographic changes, echocardiography and coronary angiography., Results: Thirty-eight patients (41.8%) with early CHD, 17 patients (16%) with CHD and 24 healthy controls (20.2%) carried at least one mutated MEFV allele. Young patients with CHD have different risk factor profiles, clinical presentations and prognoses than older patients. Young patients with CHD usually have multiple risk factors., Conclusion: This study suggests that MEFV mutations in early CHD patients had significantly increased in contrast to CHD patients and healthy controls., (© 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.)

Objectives: Ultrasonography (US) has been demonstrated to improve assessment of synovitis and disease activity in rheumatoid arthritis (RA). However, the utility and feasibility of US in RA in clinical practice in real life is not known. We aimed to investigate: i) the indications for performing US in RA in daily practice; and ii) whether the number of scanned joints varies according to the purpose., Methods: Consecutive patients who had a US scan either for diagnosis or follow-up for RA from 5 centres were recruited. The sonographers were asked to mark the joints that had a US scan and grade their findings. Descriptive analysis was applied to find out the sites and the number of joints scanned and compared according to the indications of US., Results: Two hundred consecutive patients were recruited. The most common indication was assessing disease activity (48.5%) followed by diagnosis (45.5 %). Wrists (66%) and MCPs (63.5) were the most frequently scanned joints followed by knees (26%), PIPs (20%). The number of joints scanned by US was significantly higher when performed for diagnostic purposes as compared to assessing disease activity and guidance for injections (p=0.001)., Conclusions: The current data highlight differences between the numbers of joints for which that the clinician feels necessary to perform US in real life. This observation may be a guide when providing recommendations regarding which joints need to be scanned according to the indication.

Objectives: To estimate the annual cost of rheumatoid arthritis (RA) in Turkey by obtaining real-world data directly from patients., Methods: In this cross-sectional study, RA patients from the rheumatology outpatient clinics of 10 university hospitals were interviewed with a standardised questionnaire on RA-related healthcare care costs., Results: The study included 689 RA patients (565 females) with a mean age of 51.2±13.2 years and mean disease duration of 9.4±7.8 years. The mean scores of the Routine Assessment of Patient Index Data 3 and the Health Assessment Questionnaire-Disability Index (5.08±2.34 and 1.08±0.68, respectively) indicated moderate disease activity and severity for the whole group. One-third of the patients were on biologic agents and 12% had co-morbid conditions. The mean number of annual outpatient visits was 11.7±9.6 per patient. Of the patients, 15% required hospitalisation and 4% underwent surgery. The mean annual direct cost was € 4,954 (median, € 1,805), whereas the mean annual indirect cost was € 2,802 (median, € 608). Pharmacy costs accounted for the highest expenditure (mean, € 2,777; median, € 791), followed by the RA-related consultations and expenses (mean, € 1,600; median, € 696)., Conclusions: RA has a substantial economic burden in Turkey, direct costs being higher than indirect costs. Although both direct and indirect costs are lower in Turkey than in Europe with respect to nominal Euro terms, they are higher from the perspectives of purchasing power parity and gross domestic product. Early diagnosis and treatment of RA may positively affect the national economy considering the positive correlation between health care utilisations and increased cost with disease severity.

Purpose: The aim of this study was to examine the psychometric properties of the Turkish versionof the Compliance Questionnaire on Rheumatology (CQR-T) for patients with Behçet's disease (BD)., Method: A sample of 105 Turkish patients with BD participated in this study. The scale was cross-culturally adapted through a process including translation, comparison with versions in other languages, back translation, and pretesting. Construct validity was evaluated by factor analysis, and criterion validity was evaluated using the Morisky Medication Adherence Scale., Results: The CQR-T demonstrated acceptable internal consistency (Cronbach's α = .832), adequate test-retest reliability (intraclass correlation coefficient = .630), and correlations with Morisky Medication Adherence Scale scores (r = -.389, p< .001), indicating convergent validity., Conclusion: The CQR-T was found to be a valid and reliable instrument for evaluating the compliance of Turkish BD patients with prescribed medications., Implications for Practice: The CQR-T might be a helpful tool in two ways: for determining the level of compliance of patients with BD and for adjusting their management and follow-up based on the results., (© The Author(s) 2015.)

Background: Adult-onset Still's disease (AOSD) is a rare condition, and treatment choices are frequently dependent on expert opinions. The objectives of the present study were to assess treatment modalities, disease course, and the factors influencing the outcome of patients with AOSD., Methods: A multicenter study was used to reach sufficient patient numbers. The diagnosis of AOSD was based on the Yamaguchi criteria. The data collected included patient age, gender, age at the time of diagnosis, delay time for the diagnosis, typical AOSD rash, arthralgia, arthritis, myalgia, sore throat, lymphadenopathy, hepatomegaly, splenomegaly, pleuritis, pericarditis, and other rare findings. The laboratory findings of the patients were also recorded. The drugs initiated after the establishment of a diagnosis and the induction of remission with the first treatment was recorded. Disease patterns and related factors were also investigated. A multivariate analysis was performed to assess the factors related to remission., Results: The initial data of 356 patients (210 females; 59%) from 19 centers were evaluated. The median age at onset was 32 (16-88) years, and the median follow-up time was 22 months (0-180). Fever (95.8%), arthralgia (94.9%), typical AOSD rash (66.9%), arthritis (64.6%), sore throat (63.5%), and myalgia (52.8%) were the most frequent clinical features. It was found that 254 of the 306 patients (83.0%) displayed remission with the initial treatment, including corticosteroids plus methotrexate with or without other disease-modifying antirheumatic drugs. The multivariate analysis revealed that the male sex, delayed diagnosis of more than 6 months, failure to achieve remission with initial treatment, and arthritis involving wrist/elbow joints were related to the chronic disease course., Conclusion: Induction of remission with initial treatment was achieved in the majority of AOSD patients. Failure to achieve remission with initial treatment as well as a delayed diagnosis implicated a chronic disease course in AOSD., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Objective: Screening strategies for latent tuberculosis (TB) before starting tumor necrosis factor (TNF)-α inhibitors have decreased the prevalence of TB among patients who are treated with these agents. However, despite vigilant screening, TB continues to be an important problem, especially in parts of the world with a high background TB prevalence. The aim of this study was to determine the factors related to TB among a large multicenter cohort of patients who were treated with anti-TNF., Methods: Fifteen rheumatology centers participated in this study. Among the 10,434 patients who were treated with anti-TNF between September 2002 and September 2012, 73 (0.69%) had developed TB. We described the demographic features and disease characteristics of these 73 patients and compared them to 7695 patients who were treated with anti-TNF, did not develop TB, and had complete data available., Results: Among the 73 patients diagnosed with TB (39 men, 34 women, mean age 43.6 ± 13 yrs), the most frequent diagnoses were ankylosing spondylitis (n = 38) and rheumatoid arthritis (n = 25). More than half of the patients had extrapulmonary TB (39/73, 53%). Six patients died (8.2%). In the logistic regression model, types of anti-TNF drugs [infliximab (IFX), OR 3.4, 95% CI 1.88-6.10, p = 0.001] and insufficient and irregular isoniazid use (< 9 mos; OR 3.15, 95% CI 1.43-6.9, p = 0.004) were independent predictors of TB development., Conclusion: Our results suggest that TB is an important complication of anti-TNF therapies in Turkey. TB chemoprophylaxis less than 9 months and the use of IFX therapy were independent risk factors for TB development.

Spinal new bone formation is a major but incompletely understood manifestation of ankylosing spondylitis (AS). We explored the relationship between spinal new bone formation and ultrasound (US)-determined Achilles enthesophytes to test the hypothesis that spinal new bone formation is part of a generalized enthesis bone-forming phenotype. A multicenter, case control study of 225 consecutive AS patients and 95 age/body mass index (BMI) matched healthy controls (HC) was performed. US scans of Achilles tendons and cervical and lumbar spine radiographs were obtained. All images were centrally scored by one investigator for US and one for radiographs, blinded to medical data. The relation between syndesmophytes (by modified Stoke Ankylosing Spondylitis Spine Score (mSASSS) and the number of syndesmophytes) and enthesophytes (with a semi-quantitative scoring of the US findings) was investigated. AS patients had significantly higher US enthesophyte scores than HCs (2.1(1.6) vs. 1.6(1.6); p = 0.004). The difference was significant in males (p = 0.001) but not in females (p = 0.5). The enthesophyte scores significantly correlated with mSASSS scores (ρ = 0.274, p < 0.0001) with the association even stronger in males (enthesophyte scores vs. mSASSS ρ = 0.337, p < 0.0001). In multiple regression analysis, age, BMI, enthesophyte scores and disease duration were significantly associated with syndesmophytes in males, and keeping all other variables constant, increasing US enthesophyte scores increased the odds of having syndesmophytes by 67%. Male AS patients that have more severe US-determined Achilles enthesophyte also associated spinal syndesmophytes suggesting a bone-forming gender-specific phenotype that could be a useful marker predicting of new bone formation.

Background: Despite various quantitative studies reporting that pain is among the most serious problem in ankylosing spondylitis (AS), no detailed qualitative studies address how pain affects the life of patients with AS., Aim: To explore AS patients' experiences with pain and its effect on their lives., Design: Descriptive qualitative study., Methods: Data were collected by individual in-depth interviews. Colaizzi's phenomenological data analysis was performed., Results: During periods of pain, participants indicated that they experienced difficulty with performing their daily routine activities and meeting their personal needs. Pain also prevented them from fulfilling their responsibilities in their families, inhibited their social relations, and posed problems at their workplace. Due to the negative effects of pain in their lives, the participants felt helplessness, fear, stress, sadness, and unhappiness., Conclusions: Added to being asked about the quantity of pain, patients with AS should be questioned about how pain affects their lives.

A single questionnaire regarding to disease activity for all rheumatic diseases may present advantages to introduce quantitative measurement into routine care. The aim of this study was to evaluate the correlation of routine assessment of patient index data 3 (RAPID3) with Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and Ankylosing Spondylitis Disease Activity Score (ASDAS). A total of 341 consecutive AS patients who met the modified New York classification criteria were included. All patients completed BASDAI and RAPID3 at each visit, and their physicians completed physician global assessment. ASDASs were calculated using defined formulas. Proposed RAPID3 severity categories were compared to BASDAI and ASDAS categories. Spearman's rho correlation test and kappa statistics were used to analyze statistical significance. The median age of AS patients was 34.0 (21.0-69.0) years and the median disease duration 10.0 (2.0-35.0) years. Median scores for RAPID3, BASDAI, ASDAS-CRP, and ASDAS-ESR were 13.0 (0.0-27.3), 4.7 (0.0-9.7), 3.0 (0.4-5.8), and 2.5 (0.5-6.3), respectively. RAPID3 was strongly correlated with BASDAI and ASDAS-ESR (r = 0.842, r = 0.815; p < 0.001, respectively). Among the 209 patients with high disease activity according to BASDAI, 83.3 % had high or moderate severity according to RAPID3 (kappa 0.693; p < 0.001). Among the 133 patients with moderate, high, and very high disease activity on ASDAS-CRP, 91.7 % had high or moderate severity according to RAPID3 (kappa 0.548; p < 0.001). RAPID3 is as informative as BASDAI and ASDAS in our cohort of AS patients. We therefore suggest that RAPID3 may be used to assess the patient status quantitatively in AS patients, as part of routine care.

Objective: Gouty arthritis and familial Mediterranean fever (FMF) share some clinical and pathological features such as being classified as auto inflammatory disease, association with inflammasome, short-lived intermittent arthritis, and good response to colchicine and anti-interleukin-1 treatments. As Mediterranean fever (MEFV) gene is the causative factor of FMF, we aimed to investigate the prevalence of MEFV gene mutations and their effect on disease manifestations in Turkish gouty arthritis patients., Methods: Ninety-seven patients diagnosed with primary gouty arthritis (93M and 4 F, 54 [37-84] years) and 100 healthy controls (94M and 6 F, 57 [37-86] years) included in the study. All subjects were genotyped for the MEFV variations. Number of gout attacks, diuretic use, and history of nephrolithiasis and presence of tophus were also recorded., Results: The carriage rate of MEFV mutations for patients and controls were 22.7% (n=22) and 24% (n=24) respectively. The comparison of the patient and control groups yielded no significant difference in terms of the MEFV mutations carriage rate (p=0.87). The allelic frequencies of the MEFV mutations in patients were 11.9% (n=23) and 14% (n=28) in controls (p=0.55). The presence of MEFV variants did not show any association with clinical features of gouty arthritis. The subgroup analysis of patients revealed that gouty arthritis patients with mutations had similar frequencies of tophus, history of nephrolithiasis and podogra compared to the ones without mutations (p>0.05)., Conclusions: This study does not provide support for a major role of MEFV mutations in Turkish gouty arthritis patients., (Copyright © 2014 Elsevier Editora Ltda. All rights reserved.)

Retro-orbital granuloma is a rare and devastating component of granulomatosis with polyangiitis (GPA). Current medical treatment protocols are falling short, and outcomes are poor. The aim of the study was to investigate the frequency, clinical features, and treatment outcomes of retro-orbital granuloma in patients with GPA. This is a retrospective, multi-centre study, which involves GPA cohorts from five different clinics. Data were extracted from patient charts including history, physical examination, radiological-laboratory-histological findings, and treatment protocols. Major clinical outcome measures were changes in the volume of the granuloma on comparative MRI, and visual acuity on repeated ophthalmologic examinations. Among 141 GPA patients, nine (five females and four males) were diagnosed with a retro-orbital granuloma. Median duration of disease was 8 years. Proptosis and diplopia were the dominant presenting symptoms (77%), followed by orbital pain (55%). Three out of nine patients had isolated retro-orbital granulomas, without other organ involvement of GPA. Five patients received conventional pulse steroid and pulse cyclophosphamide (CYC) as the first-line remission induction therapy. Four of these patients had progressive disease, and a regression in granuloma size was observed in one patient using this regimen. Two patients were already receiving immunosuppressants when they were diagnosed with retro-orbital granuloma. Six patients had been treated with RTX as the second-line remission induction therapy. None of these patients had progression following RTX therapy. Three patients underwent orbital decompression surgery. The indication for the decision for surgery was either progressive loss of vision or intractable pain. Standard first-line chemotherapy (CYC and steroids) was ineffective against retro-orbital granuloma associated with GPA. RTX could be an alternative in these cases. Surgical intervention may help to decrease the morbidity. Further prospective studies with greater patient numbers are needed to test the clinical efficiency of RTX as a first-line remission induction chemotherapy.

Vascular involvement is one of the major causes of mortality and morbidity in Behçet disease (BD). There are no controlled studies for the management of vascular BD (VBD), and according to the EULAR recommendations, only immunosuppressive (IS) agents are recommended. In this study, we aimed to investigate the therapeutic approaches chosen by Turkish physicians during the initial event and relapses of VBD and the association of different treatment options with the relapses retrospectively.Patients with BD (n = 936, female/male: 347/589, mean age: 37.6 ± 10.8) classified according to ISG criteria from 15 rheumatology centers in Turkey were included. The demographic data, clinical characteristics of the first vascular event and relapses, treatment protocols, and data about complications were acquired.VBD was observed in 27.7% (n = 260) of the patients during follow-up. In 57.3% of the VBD patients, vascular involvement was the presenting sign of the disease. After the first vascular event, ISs were given to 88.8% and AC treatment to 59.8% of the patients. Median duration of AC treatment was 13 months (1-204) and ISs, 22 months (1-204). Minor hemorrhage related to AC treatment was observed in 7 (4.7%) patients. A second vascular event developed in 32.9% (n = 86) of the patients. The vascular relapse rate was similar between patients taking only ISs and AC plus IS treatments after the first vascular event (29.1% vs 22.4%, P = 0.28) and was significantly higher in group taking only ACs than taking only ISs (91.6% vs 29.1%, P < 0.001). During follow-up, a third vascular event developed in 17 (n = 6.5%) patients. The relapse rate was also similar between the patients taking only ISs and AC plus IS treatments after second vascular event (25.3% vs 20.8%, P = 0.93). When multivariate analysis was performed, development of vascular relapse negatively correlated with only IS treatments.We did not find any additional positive effect of AC treatment used in combination with ISs in the course of vascular involvement in patients with BD. Severe complications related to AC treatment were also not detected. Our results suggest that short duration of IS treatments and compliance issues of treatment are the major problems in VBD associated with vascular relapses during follow-up.

The risk of infections and malignancies is the major area of concern with anti-tumor necrosis factor (anti-TNF) agents. The aim of this study was to investigate patients' views about their treatments and the factors that influence patients' treatment decisions concerning the use of anti-TNF-α drugs. This descriptive study was conducted in a single rheumatology unit. Patients using anti-TNF-α drugs for at least 3 months were included. Patients' thoughts and perceptions about their treatment were evaluated using a questionnaire. A total of 101 (94.1% male) patients were recruited. The patients described their feelings as hopeful, worried, happy, scared, desperate, and hopeless, with the order decreasing beginning with the first. Hope for healing and an expectation of increased quality of life were the most significant determinants for acceptance of treatment. After the drug information was given, patients described their feelings as follows: increase in anxiety, psychologically wearisome, and worrying about their condition worsening in the future. After anti-TNF-α treatment, patients described their experience as follows: "the most effective medicine that I have ever used," "it saved my life," "control procedures that were carried out before the treatment and once every 3 months after the treatment were essential," "I feel myself safe with these controls," and "I advised other people." This study, to our knowledge, is the first to evaluate the attitudes of patients concerning anti-TNF-α drugs from the stage of informed consent to the post-experience stage. We found that standard consent forms caused an increase in the level of anxiety among new users of anti-TNF-α drugs, although the aim was the exact opposite. The reasons for acceptance were the hope for healing, reliance on physicians, and advice of other patients. Most patients accepted follow-up control procedures, which aimed to diagnose adverse effects early.

Objectives: Unmet needs of rheumatoid arthritis (RA) patients regarding physician/patient communication, treatment preferences and quality of life issues were investigated in a Turkish survey study., Methods: The study was conducted with the contribution of 33 rheumatologists, and included 519 RA patients. The study population included patients who had been on biologic therapy for >6 months and were still receiving biologic therapy (BT group), and those who were biologic naive, but found eligible for biologic treatment (NBT group). Of the RA patients, 35.5% initially had a visit to an internal disease specialist, 25.5% to a physical therapy and rehabilitation specialist, and 12.2% to a rheumatology specialist for their RA complaints. The diagnosis of RA was made by a rheumatologist in 48.2% of patients., Results: The majority of RA patients (86.3%) visit their doctor within 15-week intervals. Most of the physician-patient communication focused on disease symptoms (99.0%) and impact of the disease on quality of life (61.8%). The proportion of RA patients who perceived their health status as good/very good/excellent was higher in the BT group than in the NBT group (74.3% vs. 51.5%, p<0.001). However, of those RA patients in the NBT group, only 24.8% have been recommended to start a biologic treatment by their doctors. With respect to dose frequency options, once-monthly injections were preferred (80%) to a bi-weekly injection schedule (8%)., Conclusions: In conclusion, RA patients receiving biologic therapy reported higher rates of improved symptoms and better quality of life and seemed to be more satisfied with their treatment in our study.

Aims and Objectives: To determine the challenges experienced by women with systemic sclerosis, whose hands affected, while performing activities of daily living and their coping strategies., Background: Many of the patients with systemic sclerosis experience difficulties in performing daily activities. One of the most important reasons for that is the impaired hand function due to their diseases., Design: A descriptive cross-sectional design was conducted and questionnaire was used in this study., Methods: The study was performed in a Rheumatology Department at a tertiary-care hospital in Turkey between April 2010-December 2011. Nineteen patients with systemic sclerosis with hand involvement were enrolled in this study. The data were collected by using both a demographic data form and an Evaluation of Daily Activity Questionnaire., Results: According to Evaluation of Daily Activity Questionnaire, the most scored dimension that patients can do with much difficulty was 'eating' and the dimension that patients unable to do was 'washing/clothes care'. In 'eating' dimension, the most difficult activities were 'opening glass jar', 'opening juice bottle' and 'opening bottle' that requiring the movement of rotation. Their coping strategies for these activities were as follows: try to open with a towel, try to remove the edge of the palm with a knife, use the hand palm and help from someone else (spouse, neighbour, etc.). In 'washing/clothes care' dimension, the most difficult activities were 'turning up hem of a skirt', 'washing up in bowl' and 'cutting out material'. For these activities, they use some coping strategies such as getting help from tailor, washing in the machine instead of hand washing., Conclusion: This study demonstrates that impaired hand function affects the daily life activities of patients with systemic sclerosis, and patients have developed some coping strategies to overcome these difficulties., Relevance to Clinical Practice: The coping strategies used by patients can be helpful for the other patients with systemic sclerosis., (© 2013 John Wiley & Sons Ltd.)

Objective: To evaluate the autonomic activity of patients with acrocyanosis by using heart rate variability indices., Material and Methods: The study group consisted of 24 patients with acrocyanosis and the control group contained 22 sex- and age-matched healthy subjects. All subjects underwent 24-hour Holter monitoring. Among the heart rate variability (HRV) parameters, time-domain and frequency-domain indices were analysed., Results: The time-domain indices of HRV indicating global autonomic functions were found to be increased, and indices indicating parasympathetic activity showed a significant decrease in the patient group. Power-spectral analysis of HRV revealed that the low frequency and high frequency power were higher in the patient group than in controls. However, the ratio of Low Frequency/High Frequency was found to be lower in the patient group than in controls., Conclusion: In acrocyanosis, both sympathetic and parasympathetic systems seem to be disrupted. Therefore, we may conclude that acrocyanosis may be resulted of systemic autonomic imbalance rather than pure sympathetic over-activation. Also, these results suggest that acrocyanosis is not a localised disorder; on the contrary, it is associated with various abnormalities of the systemic autonomic nervous system.

The immunosuppressive drugs are widely used in systemic involvements of Behçet's disease. This study is aimed to investigate the extent of clinical involvement and preferred treatment approaches for type of involvements in Behçet's patients from the whole country. All patients with the diagnosis of Behçet's disease were enrolled to the study. These patients analyzed whether they fulfill the International Study Group Criteria, and only those were further evaluated. Demographic and clinical characteristics, laboratory results and treatments ever used were recorded. Further analysis is done regarding clinical manifestations and preferred therapeutic approaches. A total of 863 patients with the diagnosis of Behçet's disease were detected, but 682 of them (female/male: 113/569) found to be appropriate for analysis. The remaining patients were included to the analysis. The frequencies of articular, ophthalmic and vascular involvement were 49, 43 and 21 %, respectively. Colchicine and corticosteroids were the most preferred agents. The immunosuppressive agents frequently used for organ involvements were azathioprine, cyclosporine A, interferon-α, sulphasalazine and cyclophosphamide with decreasing order of frequency. In this relatively young population composed from all over the country, the frequency of ophthalmologic, venous and neurological involvement is less frequent than previous reported cohorts. Azathioprine and cyclosporine were the drugs of choice as a chronic immunosuppressive agent in patients with organ involvement. The previously reported increased frequencies in other cohorts could be a result of the reference of severe patients to dedicated centers.

We assessed the role played by the ERAP1 gene in Turkish patients with ankylosing spondylitis (AS) in terms of disease susceptibility, clinical manifestations, and disease severity. We included 150 consecutive AS patients who met the modified New York classification criteria and 150 healthy controls. We documented the presence of 10 ERAP1 single-nucleotide polymorphisms (SNPs) and HLA-B27 in these patients. ERAP1 SNPs were genotyped using competitive allele-specific polymerase chain reaction. Differences between genotype and allele frequencies were compared using the Pearson's Chi-square test. The associations between ERAP1 SNPs, on the one hand, and with disease severity and clinical findings, on the other, were determined. One SNP, rs26653, was significantly associated with AS susceptibility (OR 1.609, 95% CI 1.163-2.226; p = 0.004). The population-attributable risk of possession of the rs26653 SNP allele was 23.4%. No relationship was noted between HLA-B27 positivity and the distribution of rs26653 genotype frequency. No associations were seen between disease severity measures and clinical manifestations of AS. In summary, an ERAP1 polymorphism was associated with AS in a Turkish population. The contributions of HLA-B27 and the rs26653 SNP to AS pathogenesis appear to be independent.

Background/aims: Chronic arthritis of familial Mediterranean fever (FMF) involves weight-bearing joints and can occur in patients without a history of acute attack. Our aim was to investigate a possible causal relationship between FMF and osteoarthritis in a population in which FMF is quite common., Methods: Patients with late stage primary osteoarthritis were enrolled, and five MEFV gene mutations were investigated. The frequency of MEFV gene mutations was compared among patients with osteoarthritis and a previous healthy group from our center., Results: One hundred patients with primary osteoarthritis and 100 healthy controls were studied. The frequency of MEFV gene mutations was significantly lower in the osteoarthritis group (9% vs. 19%). M694V was the most frequent mutation (5%) in the osteoarthritis group, whereas in the control group, E148Q was the most common (16%). In subgroup analyses, the mutation frequency of patients with hip osteoarthritis was not different from that of patients with knee osteoarthritis and controls (7.1%, 9.7%, and 19%, respectively). There were no differences among the three groups with respect to MEFV gene mutations other than E148Q (8.1% vs. 3.6%). E148Q was significantly lower in the osteoarthritis group than in the controls (16% vs. 1%), although the mutations did not differ between patients with knee osteoarthritis and controls., Conclusions: In a population with a high prevalence of MEFV gene mutations, we did not find an increased mutation rate in patients with primary osteoarthritis. Furthermore, we found that some mutations were significantly less frequent in patients with osteoarthritis. Although the number of patients studied was insufficient to claim that E148Q gene mutation protects against osteoarthritis, the potential of this gene merits further investigation.

Aim: To compare apparent diffusion coefficients (ADCs) of sacroiliac joints (SIJs) in ankylosing spondylitis (AS) patients during advanced-active and advanced-nonactive stages., Materials-Methods: AS patients with chronic-active (n=19), chronic-nonactive (n=6), and controls with normal SIJs (n=8) were included. Mean ADCs through 43 subchondral bone marrow edema lesions (SBMELs) were calculated., Results: Mean ADCs were 1.60+/-0.32 × 10-3 mm(2)/s over SBMELs, 0.57+/-0.23 × 10-3 mm(2)/s at periphery of SBMELs, 0.57+/-0.24 × 10-3 mm(2)/s in chronic-nonactive group, and 0.61+/-0.19 × 10-3 mm(2)/s for controls., Conclusion: ADCs lower than 0.69 × 10-3 mm(2)/s, obtained at subchondral aspect of SIJs of established AS patients with chronic changes, which this number represents the receiver operating characteristic (ROC) best cutoff value, can be considered as normal without possible residual inflammation of concern., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Objectives: The Behçet's Syndrome Activity Score (BSAS) is the first patient reported outcome measure developed to assess the global disease activity in patients with Behçet's syndrome (BS). We aimed to evaluate the reliability and validity of the Turkish version of BSAS for measuring disease activity in BS. We further investigated the performance of Routine Assessment of Patient Index Data (RAPID)3, a patient-reported index originally developed for rheumatoid arthritis, in BS patients., Methods: Patients seen consecutively at a tertiary Rheumatology Centre were requested to complete BSAS and multidimensional health assessment questionnaire (MDHAQ). Besides, all attending physicians filled the Behçet's Disease Current Activity Form (BDCAF). Descriptive statistics and Pearson correlation coefficients were calculated accordingly for the reliability and validity assessments of BSAS., Results: A total of 104 patients completed all three assessments. The test-retest reliability of BSAS has a good level (ICC=0.84, 95% CI [0.69-0.94]). The mean scores for BSAS, BDCAF and RAPID3 were 39±20.8, 3.2±1.4 and 9.2±5.6, respectively. BSAS was correlated with BDCAF moderately (r=0.587), while it was moderately correlated with RAPID3 (r=0.648). The correlation between the RAPID3 and BDCAF was moderate (r=0.403), but lower as compared to the correlations between the other instruments., Conclusions: We found that the BSAS has modest correlation with BDCAF and is a reliable and valid patient reported measure of disease activity that can be used to assess patients with BS. An outcome score composed of only patient-derived observations may have the additional advantage of being easier to use in a routine care setting. Demonstration of a moderate level of correlation between RAPID3 and BDCAF (close to the level of weak relationship), suggests that RAPID3 likely needs more investigations before recommending its use in BS.

Objectives: CXCL16 is a member of CXC chemokine, which is synthesised in plasmacytoid dendritic cell as a transmembrane molecule. Transmembrane CXCL16 on plasmacytoid dendritic cell plays a role in binding, uptaking and accumulation of CpG D ODN in early endosomes rather then lysosomal vesicles, thereby causing a high level of interferon-alpha secretion. Previously, we disclosed pronounced interferon-alpha production from these cells in patients with Behçet's disease. The aim of this study was to investigate the relation between the secretion of IFN-α and the expression of CXCL16 on surface of plasmacytoid dendritic cell from patients with Behçet's disease, and compare it with patients with ankylosing spondylitis and healthy controls., Methods: The study population consisted of 73 cases (35 with Behçet's disease, 19 with ankylosing spondylitis and 19 controls). We investigated the expression of CXCL16 on surface of plasmacytoid dendritic cells by flow cytometry, and the serum levels of IFN-α and CXCL16 with ELISA., Results: Serum levels of IFN-α in patients with Behçet's disease were significantly higher than the controls (p=0.009), and than patients with ankylosing spondylitis, but not statistically significant (p=0.124). Serum levels of CXCL16 in patients with Behçet's disease and patients with ankylosing spondylitis were significantly higher than controls (p=0.009, p=0.003, respectively). We found no difference in the percentage and MFI of plasmacytoid dendritic cells and CD123+CXCL16+ cells determined by flow cytometry among the study and control groups. In patients with Behçet's disease, a positive correlation was found between the percentage of plasmacytoid dendritic cells and CD123+CXCL16+ cells (p<0.001). Furthermore, there was also a positive correlation between the percentage of plasmacytoid dendritic cells and serum levels of CXCL16 in patients with ankylosing spondylitis (p=0.001). In addition, there was a positive correlation between the percentage of CD123+CXCL16+ cells and serum levels of IFN-α in Behçet's disease group (p=0.034). We could not find any significant difference in other comparisons., Conclusions: We suggested that the expression of transmembrane CXCL16 on surface of plasmacytoid dendritic cell might contribute to high serum IFN-α levels seen in patients with BD.

Angiogenesis is controlled by a variety of angiogenesis stimulators and inhibitors. The increased power Doppler (PD) signals determined by ultrasonography is an indirect marker of synovial vascularity in arthritis. We aimed to investigate relationship between ultrasonographic findings and synovial angiogenesis modulators. Thirteen Behcet's disease (BD), 15 spondyloarthropathy, 21 rheumatoid arthritis (RA), and 15 osteoarthritis (OA) patients with knee arthritis were included. Cumulative effusion, synovial hypertrophy, and PD signal scores were calculated in arthritic joints. In synovial fluid samples, angiogenesis inhibitors (angiostatin, thrombospondin-1, and endostatin) and stimulators [bFGF (basic fibroblast growth factor), angiopoietin-1] were studied. The comparisons between groups were made by Kruskal-Wallis test, and correlation analysis was calculated with Pearson and Spearman tests. Effusion scores were significantly higher in inflammatory arthritis than in OA. Synovial hypertrophy scores were higher in RA and spondylarthritis than in OA and BD. PD scores were not different between the groups. Synovial angiostatin and bFGF levels were significantly higher in patients with inflammatory arthritis than in OA. Cumulative effusion scores were positively correlated with angiopoietin-1, angiostatin, and bFGF and negatively correlated with thrombospondin-1 levels. Synovial hypertrophy scores were positively correlated with angiostatin and bFGF levels and negatively correlated with thrombospondin-1. No correlation was found between PD scores and modulators of angiogenesis. In large joints like knee, detecting PD signals alone was not sufficient to assess the angiogenesis. However, cumulative activity scores were positively correlated with angiogenesis stimulators. Therefore, when investigating the angiogenesis, PD technique should be added to gray-scale examinations.

Although the inflammatory cascade of familial Mediterranean fever (FMF) is partially understood, triggering factors of those attacks has not been studied well. It is supposed that physical stresses such as cold exposure, tiredness and emotional stresses could provoke attacks. This study is aimed to survey the factors regarded as triggering the attacks in patients with FMF and their relationship with MEFV gene mutations. Clinical findings and genetic mutations (consist of M694V, M694I, M680I, V726A, E148Q) of patients were recorded. Patients were questioned about cold exposure, emotional stress, tiredness, long-lasting standing, long-duration travel, starvation, high intake of food, trauma, and infection as triggering factors for the attacks with both serositis and musculoskeletal pain. The study is comprised of 275 FMF patients (male/female: 177/98). The most common triggering factors for the attacks with serositis were cold exposure (59.3 %), emotional stress (49.8 %), tiredness (40.0 %) and menstruation (33.7 % in females). Long-lasting standing (78.8 %), long-duration travel (64.1 %) and tiredness (47.8 %) were the triggering factors for the attacks with musculoskeletal symptoms. The relationships between MEFV mutations and triggering factors were found as M694V allele with starvation, E148Q allele with high intake of food and V726A allele with long-duration travel. The attacks with serositis seem to be triggered by those factors to which whole body exposed, whereas the attacks with musculoskeletal complaints seem to be triggered by those factors to which regional or local part of body exposed. Since the number of alleles was small, a clear conclusion for a relationship between a particular gene variant and a specific trigger was not made.

Objective: To test contrast to noise ratios (CNRs) of both diffusion-weighted (DW) images and contrast enhanced images in terms of the visual assessment of activity in sacroiliitis of ankylosing spondylitis (AS) patients., Materials and Methods: The study included 21 patients with AS. All patients were examined with STIR, FST1/Gd and DWI (b = 0,600). A total of 54 hyperintense lesions on STIR were noted in their sacroiliac joints divided into four quadrants. CNRs were calculated for all of the sequences above. A second group of patients (n = 7) with normal sacroiliac joints (SIJs) served as controls. A total of 56 CNR measurements from apparently normal subchondral bone marrow in this control group were done as well. The differences between scores were tested for significance (SPSS version 17.0) using Wilcoxon's test in which p values lower than 0.01 were considered statistically significant., Results: In the first group with sacroiliitis, mean CNRs for STIR, FST1/Gd, DWI were 32.97, 30.16 and 24.47, respectively. Mean CNRs in the second group with normal SIJs were calculated as 3.52 , 2.99 and 3.96, respectively . There was a statistically significant difference between the CNR measurements of the first and the second group (p = 0.000). Hyperintense lesions on STIR were depicted as "active" in the first group. Except for four lesions that were not included into the study, all of these hyperintense lesions were enhanced after contrast media administration. All of the "active" lesions were observed on DWI as well, at b = 600. No statistically significant difference between CNRs of contrast enhanced images and DWI and of contrast enhanced images and fluid sensitive sequences were found in the first group with sacroiliitis (p > 0.01)., Conclusion: The CNRs are highest on STIR, followed by contrast enhanced images and DWIs. In terms of DWI and contrast enhanced images, there is no statistically significant difference between these two. Hence, contrast enhanced imaging can be replaced by DWI for visual analysis of active sacroiliitis, which is easy to apply without adverse affects of contrast media.

Objective: Various types of scientific abstracts are selected and presented at meetings and listed in abstract books. Recently, a systematic review has shown that 45% of 30,000 abstracts were published in a journal. The aim of this study was to determine the features of abstracts selected to be presented at a EULAR meeting (2008) and the corresponding publication rates., Methods: The EULAR 2008 Abstract Book was extracted, presented abstracts were assessed, their publication status was checked, and features related with publication rate were determined. The publication status of abstracts as of January 20, 2011, was verified using PubMed., Results: A total of 1,732 abstracts were assessed. Median publication duration was 13 (range: 0 to 31) months. Most of the abstracts (N=339) were of multi-national origin. Sixty seven percent of abstracts were clinical and 563 (33%) abstracts were preclinical. We found that 601 of all abstracts (34.7%) had been published in a journal, and most of were published in a rheumatology journal. Fifty-seven percent of published abstracts were in journals with an impact factor higher than 4. The publication rate was correlated with presentation type, number of centers involved, trial design, and number of patients enrolled., Conclusions: We found that the publication rate of EULAR 2008 abstracts at 30 months was approximately 35%. This is a high rate compared to a previously published systematic review that investigated the publication rate of studies initially presented as abstracts in medical meetings, which reported the publication rate at 24 and 36 months as 20.7% and 28.1%, respectively. More than half of the published abstracts were accepted by high-impact journals. Presentation type, number of centers, trial design, and enrolled patient numbers were all correlated with the rate of publication.

Adult onset Still's disease (AOSD) is a systemic inflammatory disease with unknown etiology and characterized by evanescent salmon pink rash, sore throat, liver dysfunction, lymphadenopathy, hepatosplenomegaly, arthritis, and leukocytosis. It is a diagnosis of exclusion; however, there are case reports in the literature about patients with malignancies and AOSD-like signs and symptoms. Here we report a patient with AOSD seems to be associated with sarcomatoid renal cell carcinoma. This phenomenon is not distinguishable from primary AOSD either in presentation or in treatment; except for the main purpose of the management should be targeted to the underlying malignancy.

In this study, it is aimed to determine the daily life experiences of patients on the basis their own way of statement. Sixteen patients with scleroderma were enrolled to this qualitative study. Data were collected using both a demographic data form and a semi-structured interview form. Study was made on individual patient interview by face-to-face manner. Data were evaluated using Colaizzi's phenomenological data analysis method. Data analysis revealed four categories and nine topics. These categories were (1) physical impact of disease, (2) emotional impact of disease, (3) social impact of disease and (4) patient behaviours for the cope with the disease. As emotional impact, patients imply that they have experienced disappointment, hope to get well and have fears about the future. In the physical impact category, tight skin, limitations of hand skills, swelling of the hands and feet, fatigue, swallowing difficulties and deformation of their bodies were the prominent features. In patients with recognizable disease, difficulty to join to social activities increases and eventually leads to isolation. There was also some evidence that patients who have been supported by their family and close relatives seem to be more optimistic about their disease. Most patients are not willing to communicate with other patients, particularly in an advanced stage. Scleroderma patients imply that they experience several difficulties regarding emotional, physical and social aspects. Individual abilities to cope with the disease were much more improved among patients who have a sustained social support. For advanced patients with apparent deformations, an effective social support system should be introduced.

Objectives: To determine the direct and indirect costs due to rheumatoid arthritis (RA) and ankylosing spondylitis (AS) patients in Turkey., Methods: An expert panel was convened to estimate the direct and indirect costs of care of patients with RA and AS in Turkey. The panel was composed of 22 experts chosen from all national tertiary care rheumatology units (n=53). To calculate direct costs, the medical management of RA and AS patients was estimated using 'cost-of-illness' methodology. To measure indirect costs, the number of days of sick leave, the extent of disability, and the levels of early retirement and early death were also evaluated. Lost productivity costs were calculated using the 'human capital approach', based on the minimum wage., Results: The total annual direct costs were 2,917.03 Euros per RA patient and 3,565.9 Euros for each AS patient. The direct costs were thus substantial, but the indirect costs were much higher because of extensive morbidity and mortality rates. The total annual indirect costs were 7,058.99 Euros per RA patient and 6,989.81 for each AS patient. Thus, the total cost for each RA patient was 9,976.01 Euros and that for an AS patient 10,555.72 Euros, in Turkey., Conclusions: From the societal perspective, both RA and AS have become burden in Turkey. The cost of lost productivity is higher than the medical cost. Another important conclusion is that indirect costs constitute 70% and 66% of total costs in patients with RA and AS, respectively.

In Behçet's disease, deep venous thrombosis occurs primarily in the lower extremities. Total recanalization rate is low, so thrombotic segment could be detected by imaging afterward. For disclosing vein involvement, leg swelling is commonly queried in the history taking in those patients. However, there are no data about the presence of "silent" thrombosis in patients with BD. We aimed at investigating the integrity of venous vessels in BD, without any known vascular event by using Doppler ultrasonography (DU). Patients having past events revealed in the vascular questionnaire or physical findings attributable to vascular disease were excluded. Various degree of venous insufficiency was detected in 74 patients in BD (74%), 24 out of 33 patients (72%) in AS and in 8 out of 34 (25%) in HC group. All were at the lower extremities, and there is no difference in the frequency between BD and AS, while both were significantly higher than in HC (P = 0.001, and 0.004, respectively). Six patients with BD (6%) have chronic venous thrombi at the lower extremities and none in either AS and HC. As a non-invasive method, DU of lower extremities may disclose "silent" thrombosis. Venous insufficiency in those patients should be considered cautiously as an indicator of vein involvement.

Pyogenic sacroiliitis (PS) is an acute form of sacroiliitis that mostly starts with very painful buttock pain. Here in this case, the followup magnetic resonance (MR) images of a 49-year-old male patient with PS is displayed. After his sacroiliitis was documented by MR images, he was treated with the combination of rifampicin plus streptomycin and moxifloxacin. Serial MR investigations were done to disclose acute and subsequent imaging changes concerning sacroiliac joint and surrounding bone structures. Although after treatment all the symptoms were completely resolved, 20 months later changes suggesting active sacroiliitis on MR images were continuing.

Muscular involvement in Behçet's disease is rare and there are only a few case reports in the literature. The causes of elevated muscle enzymes in a patient with Behcet's disease are many, including myositis, drug-induced myopathy, metabolic myopathy, and the disease itself. We herein have defined an algorithmic approach to a patient with Behcet's disease and elevated muscle enzymes and report a case of coexisting of lipid storage myopathy.

Although neurological involvement in Behçet's disease is not so uncommon, isolated spinal cord disease is quite rare and reported to be observed in about 2% of all cases with neurological involvement. Here we report a Behçet's patient with spinal cord disease presented with anterior spinal cord syndrome. This rare syndrome is caused by hypoperfusion of the anterior spinal artery and to our knowledge has not been previously reported in patients with Behçet's disease. This report defines the characteristic clinical features of this entity and emphasizes the importance of early immunosuppressive treatment and initiation of rehabilitation.

Objective: Behçet disease is a systemic disease with protean manifestations. Vasculitis is a hallmark of the disease and may involve arteries, veins, and capillaries. Varicocele is dilatation of the pampiniform plexus veins. We aimed to investigate the incidence of varicocele in patients with Behçet disease., Methods: The study included 47 male patients with Behçet disease and 31 healthy control participants. All underwent a clinical evaluation including a medical history and systemic and scrotal examinations. Subsequently, 2 investigators blinded to the clinical data performed sonographic examinations and measured pampiniform plexus vein diameters., Results: The mean age of the patients ± SD was 23.4 ± 3.2 years; disease durations ranged from 3 to 120 months (46 ± 31 months). Scrotal pain or a palpable mass was detected by clinical examination in 24 patients with Behçet disease (51.1%) and 5 healthy participants (16.1%; P = .002). By color Doppler examination, left varicocele was diagnosed in 26 patients with Behçet disease (55.3%) and 9 healthy participants (29%; P = .02). All patients with right varicocele also had left varicocele; that condition was detected in 10.6% (5 patients) of the Behçet disease group and 6.4% (2 patients) of the control group (P > .05). Eight patients with Behçet disease (17%) had epididymitis, whereas none of the healthy participants did (P = .019, Fisher exact test). Genital ulcers and erythema nodosum lesions were more common among patients with varicocele (P = .034 and .058, respectively). There were no differences in smoking, epididymitis, arthritis, uveitis, or other clinical parameters for distinguishing varicocele in patients with Behçet disease., Conclusions: The incidence of varicocele was increased in Behçet disease. Whether varicocele confers fertility problems in patients with Behçet disease and the underlying mechanism for a possible association are yet to be determined.

It is well established that there are people with higher risk of developing acute rheumatic fever (ARF) and rheumatic heart disease (RHD). Mediterranean fever (MEFV) gene mutations might be one of the genetic predisposition factors in the development of ARF/RHD since defect in familial Mediterranean fever (FMF) patients is proposed to be heightened inflammatory response to certain stimuli. Previous clinical observations suggested a relationship between FMF and ARF/RHD. The aim of this study was to investigate the role of the MEFV gene mutations in the susceptibility to RHD in Turkish patients. A total of 100 patients with RHD and 100 healthy controls were included in the study. Diagnosis of RHD was based on echocardiographic findings in which a predominant mitral stenosis was used as an inclusion criterion. Genetic analysis was carried out by sequence analysis investigating two hot spots (exons 2 and 10) for MEFV mutations. Mutation analysis showed that 22 RHD patients (22%) and 24 healthy controls (24%) carried at least one mutated allele. MEFV mutations were identified in 22 of 200 (11%) chromosomes in RHD patients while 26 of the 200 (13%) chromosomes of healthy controls were found to carry a mutated allele. No difference was found in allele frequencies and their distribution between the patients and healthy controls (p = 0.54). MEFV mutations are not associated with a predisposition to develop RHD in adult Turkish patients.

Behçet's disease is a systemic disease characterized by oral aphthosis, genital ulcers, ocular lesions, gastrointestinal, musculoskeletal, neurological and major vessel involvement. Arterial involvement, aneurysms and arterial thrombosis have been reported in 1.5-3% of patients. In this case report, we present a patient with ulnar arterial aneurysm associated with Behçet's disease.

The objective of the study was to investigate the response rate to non-steroidal anti-inflammatory drugs (NSAIDs) and the clinical parameters that might predict this response in patients with active ankylosing spondylitis. This is a prospective, observational, 3-month study that was conducted in a single center. Ninety-five consecutive patients with active ankylosing spondylitis were included in the study. Full dose NSAIDs (indometacin 150 mg daily or acemetacin [corrected] 180 mg daily) were given to patients. Relevant clinical data of all patients' were recorded at the beginning and on three consecutive monthly visits. At the end of the study period, patients who respond to NSAIDs were determined. Demographic, clinical, and laboratory parameters that might influence the response to the NSAIDs were investigated. The response rate to the full-dose NSAIDs according to the ASAS20 in patients with active ankylosing spondylitis was found as 29.5%. Similarly, 20.0% of the patients were responders according to the ASAS40 criteria, whereas 5.6% of the patients responded according to the 5-out-of-6 criteria at week 12. Patients who responded to the treatment were found to be younger at the study entry (P = 0.001) and had shorter disease duration (P < 0.001). Due to the markedly lower rate of response to the NSAIDs in patients with active ankylosing spondylitis, early identification of those patients who does not respond to NSAIDs and subsequent decision regarding the institution of second-line treatments (anti-TNF) may be of great value in the prevention of irreversible changes that might develop in most of the patients.

Reactive systemic (AA) amyloidosis leading to renal failure is the most severe complication of tumor necrosis factor receptor-associated periodic syndrome (TRAPS). There is now growing evidence to suggest that anti-tumor necrosis factor (anti-TNF) agents may be an attractive treatment option for amyloidosis not only in TRAPS but in several forms of secondary amyloidosis complicating inflammatory rheumatic diseases. In most of the reported cases, anti-TNF agents were deemed successful on the basis of regression of proteinuria and either improvement or stabilization of creatinine clearance, while objective proof of renal amyloid regression either by serum amyloid P scintigraphy or biopsy is limited. We herein report a case of TRAPS complicated with nephrotic syndrome due to AA amyloidosis in which treatment with etanercept was associated with remission of the nephrotic syndrome but no regression of amyloid mass on the follow-up renal biopsy. Indeed, amyloid deposition was noted to be more pronounced on the second renal biopsy, particularly at tubular basement membranes. Although the variable relation between reduction in amyloid load and changes in organ function is well-known, the basis for renal recovery in association with stable or even progressive amyloid deposition is challenging. We suggest that in patients with secondary AA amyloidosis, mechanisms other than the reduction of amyloid mass could have contributed to the observed improvement of renal function with anti-TNF agents.

Objective: The aim of the study was to evaluate the influence of dental and periodontal treatments to the course of oral ulcers in patients with Behcet's disease (BD)., Methods: Fifty-eight consecutive BD patients with oral ulcers were studied. Twenty-nine patients were in the intervention group (F/M: 15/14, mean age: 39.6 +/- 6.9 years) and 29 (F/M: 15/14, 39.4 +/- 10.6 years) were followed with a conventional treatment approach. In addition to oral hygiene education, dental and periodontal treatments were carried out in the intervention group, whereas the control group was only given oral hygiene education. Patients were evaluated in the pre-treatment observation period (1 month), treatment period (1 month) and 6 months after treatment., Results: An increase in the number of new oral ulcers (4.1 +/- 3.5) was observed within 2 days during the treatment compared with 3-30 days during treatment month (2.3 +/- 1.2) (P = 0.002). However, 6 months after the treatment, the number of oral ulcers (1.9 +/- 1.5) was significantly lower compared with the pre-treatment observation (4.8 +/- 3.2) (P = 0.000) and treatment periods (6.4 +/- 2.3) in the intervention group (P = 0.05), whereas a similar oral ulcer presence was observed in the control group (2.8 +/- 2.4, 3.7 +/- 2.3 and 4.8 +/- 4.3, respectively) (P > 0.05). Dental and periodontal indices were also better in the intervention group during the 6-month follow-up., Conclusion: Our results suggest that, in BD patients, dental and periodontal therapies could be associated with a flare-up of oral ulcers in the short term, but may decrease their number in longer follow-up. They also lead to a better oral health.

Osteopoikilosis (OPK) is a rare benign sclerosing disease of the skeleton and inherited as an autosomal dominant trait. OPK is associated with inflammatory rheumatic disorders, such as rheumatoid arthritis, scleroderma, reactive arthritis and familial Mediterranean fever (FMF). We report a rare case of OPK coexistent with ankylosing spondylitis and FMF. The patient presented multiple sclerotic lesions within and around the sacroiliac joints and a series of radiological diagnostic challenges.

In this study, our aim was to investigate the prevalence of Mediterranean fever (MEFV) gene mutations in patients with ankylosing spondylitis (AS) and assessing their clinical significance. Ninety-five consecutive patients (12 women, 83 men) with active AS were included to the study. All patient's relevant clinical data were recorded at the beginning and patient assessment measures were performed. The frequency of the eight most common MEFV mutations: M694V, V726A, E148Q, M680I, M694I, P369S, F479L, and the R761H were determined. Genetic analysis was carried out by the NanoChip Molecular Genetics Workstation. NSAIDs were given to patients for treatment. The rate of MEFV mutations and their clinical significance were assessed. With regard to the MEFV mutation analysis, 30.5% of AS patients were found to have at least one mutation. The response rate to the NSAIDs (P=0.825) or frequency of patients having active disease (P=0.066) after the treatment, were not found different between the patients those have MEFV mutations and the patients those were non-carriers. Furthermore, no clinical and laboratory difference between MEFV mutation carriers and non-carriers were found. We think that although prevalence of MEFV mutations is significantly high in AS patients without clinical features of familial Mediterranean fever, its influence to the prognosis is less likely. Further investigations are needed to define the impact of MEFV mutations on the disease course of ankylosing spondylitis.

The assessment of disease activity in Behçet's disease (BD), either by BD Current Activity Form (BDCAF) or Iranian BD Dynamic Measure (IBDDAM), depends largely on the history of the clinical features. Accuracy of recall of a retrospective questionnaire might influence its reliability to some extent. The aim of this study was to investigate whether patients with BD can recall the items included in both of the disease activity forms accurately. Twenty patients with BD completed a retrospective questionnaire that included BDCAF and IBDDAM, twice, 1 month apart. We made some modifications in the IBDDAM, while the BDCAF (revised 8.5.2002, GL) was applied in its original structure. In contrast to the original application of IBDDAM, in which the history obtained depends on the variable time period, all items of IBDDAM in the present study asked subjects to report their symptoms over the preceding 4 weeks. Furthermore, those IBDDAM items for which the operational definitions are not clearly provided by the developers, as well as the items that depend on the objective examination rather than the history, were not included. Concurrent daily data were collected for 2 months via telephone interview. The level of agreement between the retrospective responses and those extracted from daily recordings were quantified for each item using intraclass correlation coefficient (ICC). The agreement between the recalled and daily reports was good (ICCs>0.60) for the majority of items (oral and genital ulceration, skin lesions, arthralgia, arthritis, and eye involvement) included in BDCAF. Exceptions were gastrointestinal symptoms (ICCs<0.40) and headache (ICCs 0.40-0.50). With regard to the IBDDAM, all of the items examined had good agreement (ICCs>0.60), except for headache, for which the agreement was moderate (ICCs 0.40-0.50) for the first and poor (ICCs<0.40) for the second month. For most of the BD-related features questioned in the disease activity forms, patients provided reasonably accurate reports in comparison to information obtained from daily records. However, it should be kept in mind that the limitations of the study design need to be considered while interpreting the results of the present study. Notably, unlike its original application in which the assessment depends on the variable time period, IBDDAM used in the present study assessed the symptoms present during the month prior to the date of assessment. Therefore, our findings regarding IBDDAM cannot be applied to its original use. Furthermore, lack of patients in some of the symptom groups, as well as possible manipulation of recall with daily questioning, might have influenced our results to some extent.

Congenital afibrinogenemia is a rare disorder characterized by the absence in circulating fibrinogen, a hexamer composed of two sets of three polypeptides (Aalpha, Bbeta and gamma). Although predisposition to thrombosis is a well known feature of dysfibrinogenemia, the relatively frequent thrombotic manifestations seen in congenital afibrinogenemia are puzzling. We herein report a mutational analysis of a young afibrinogenemic man from Turkey with multiple thrombo-embolic events involving both arteries and veins. Purified DNAs of the propositus was used for amplification by polymerase chain reaction of all the exons of the A subunit gene with primers allowing the analysis of the intron-exon boundaries. Analysis of the genes coding for the three fibrinogen chains of the propositus found a homozygous G to A transition in the exon 5 of the A alpha chain gene (g.g4277a; access number gi458553). The TGG to TGA codon change predicts a homozygous W315X in the A alpha chain (p.W334X when referring to the translation initiation codon). Both parents and his brother were found to carry this heterozygous mutation. This is the first report of a patient homozygous for this rare mutation associated with afibrinogenemia. Our patient was free of known risk factors as well as diseases associated with thrombosis including atherosclerosis, vasculitis, Buerger's disease, and it seems therefore probable that afibrinogenemia itself might have contributed to both arterial and venous thrombosis.

Ever since the introduction of anti-tumor necrosis factor (TNF) agents, concerns have been raised regarding their potential for developing malignancy. We report the development of thymic atypical carcinoid tumor 9 months after the initiation of etanercept therapy in a patient having refractory spondylarthritis. This case indicates the need for following large cohorts of patients receiving anti-TNF agents to address the long-term effect of these agents on malignancies.

Objective: Various demyelinating disorders have been reported in association with anti-tumour necrosis factor alpha (TNFalpha) agents. The objective of this study was to review the occurrence, clinical features and outcome of optic neuritis (ON) during treatment with anti-TNFalpha agents., Methods: A PubMed search was conducted to identify literature addressing the potential association between anti-TNFalpha agents and ON, following our experience with a patient having rheumatoid arthritis in whom ON developed while being treated with infliximab., Results: 15 patients including the case presented here with ON in whom the symptoms developed following TNFalpha antagonist therapy were evaluated. Eight of these patients had received infliximab, five had received etanercept and two patients had received adalimumab. Among them, nine patients experienced complete resolution, and two patients had partial resolution, while four patients continued to have symptoms., Discussion: Patients being treated with a TNFalpha antagonist should be closely monitored for the development of ophthalmological or neurological signs and symptoms. Furthermore, consideration should be given to avoiding such therapies in patients with a history of demyelinating disease. If clinical evaluation leads to the diagnosis of ON, discontinuation of the medication and institution of steroid treatment should be a priority.