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Background Neonatal lupus erythematosus (NLE) which is caused by trans placental transmission of maternal anti SS-A/Ro and anti SS-B/La antibodies. Cutaneous, cardiac, hepatic and hematologic manifestations can be seen. Objectives The objective of this systematic review was to analyze demographic data, clinical manifestations, autoantibodies, treatment modalities and mortality status of NLE patients. Methods Systematic review has been done at August 2015 on Pubmed Medline. Publications are determined by using MeSH term: (“neonatal lupus, congenital hearth block” (MeSH)) and restricted to “in humans” and “in English”. Out of study design, abstracts of all publications has been assessed. 1377 article has been assessed in abstract format, after that; 277 original article/case report has been evaluated in full-text format. Finally, 199 article/ case report has been included to study after full-text evaluation. Totally, 755 patients enrolled. Demographic, clinical, laboratory features were assessed both maternal and infant level. Results Maternal features: Mean maternal age was 29.7 (4.8) years. Median gestational week was 23 (21–40) weeks. Accompanying diseases were known in 547 patients (SLE 176 (32.2%), Sjogren syndrome 99 (18.1%), undifferentiated connective tissue disease 31 (5.7%), rheumatoid arthritis 7, other autoimmune disease 53 (9.6%), no disease 179 (32.7%)). ANA results were known in 349 mothers with 287 (82.2%) positivity. ENA were known in 601 mothers (SS-A 506 (84.2%), SS-B 313 (52.1%), anti-RNP 31 (5.2%), negative 57 (9.1%)). In 327 mothers, diagnosis time of collagen tissue disease was known (212 (64.8%) mothers before pregnancy, 18 (5.5%) during pregnancy and 97 (29.7%) postpartum period). Maternal treatments before pregnancy were known in 137 of mothers (glucocorticoid 38 (27.7%), hydroxychloroquine 13 (9.5%), azathioprine 4 (2.9%), IVIG 2 (1.4%), and no treatment 94 (68.6%)). Infants9 features: Totally, 272/477 (57.0%) was female. Mean birth weight was 2680 (364–4370) gram, median delivery week was 37 (19–42) and 126 of 295 (42.7%) NLE was premature. Autoantibodies were known in 272 infants (225 (82.7%) SS-A, 126 SS-B (46.3%), 25 (9.2%) anti-RNP, all negative 20 (7.4%)). Mortality data were available for 485 infants with 412 (84.9%) were alive (85 with pacemaker), 37 (7.6%) were intrauterine exitus, and 36 (7.4%) were post-partum exitus. NLE features: Most common manifestations of NLE was; cardiac 467 (61.8%), cutaneous 249 (33.1%), hematologic 117 (15.5%), and hepatic 78 (10.5%). Cardiac involvement types were known in 284 infants (AV complete block 219 (77.1%), 2. block 27 (9.5%), 1. block 19 (6.7%), and sinus bradycardia 19 (6.7%). Totally, 62 of 195 (31.2%) AV complete block detected post-partum period. Conclusions NLE was cause of premature (almost 43%) delivery and had high mortality ratio (almost 15% of infants). SLE and Sjogren9s syndrome were leading maternal diagnosis. Although ANA and SS-A are main maternal autoantibodies, SS-B may also be found half of the cases. Congenital heart block, particularly AV block, is life-treating involvement, and unfortunately one of three infants have diagnosed at post-partum period. Disclosure of Interest None declared

Background Vasculitides are a heterogeneous group of diseases. The distribution of the type of vasculitis changes not only with age and sex but according to the geographic area as well. The prevalence of each vasculitis is important in guiding the local medical education and raising awareness of doctors and policy makers etc. Objectives This study is aimed to present the distribution of vasculitides in the Pediatric and Adult Vasculitis patient Groups. Methods Hacettepe University is one of the main referral centers in the country. Executive committee of our Vasculitis center consist of physicians from Pediatric and Adult Rheumatology, Nephrology, Radiology and Pathology Departments. Multidisciplinary approach improves management of our patients. All patients starting from October 2014 have been included and defined according to the 2012 revised Chapel Hill nomenclature criteria. Results A total of 882 patients had been newly diagnosed/followed in this period. 205 (23.2%) of them were pediatric patients. The leading vasculitis among adult patients was Behcet9s Disease whereas in pediatric patients it was HSP (Figure). More than half of the adult Behcet9s patients (58.4%) had uveitis/neurologic/vascular involvement. Takayasu arteritis was more frequent than giant cell arteritis among the adult patients. There was a female predominance in patients with large vessel vasculitis. Granulomatous polyangiitis was the most common small vessel vasculitis in adults. Finally 23 adult cases of Immunglobulin G4 related disease have been seen. Conclusions We describe the demographic characteristics and distribution of vasculitides in the eastern Mediterranean. Behcet9s disease has a high frequency as expected. Also Takayasu arteritis is more frequent than Giant cell arteritis. Microscopic polyangiitis is very rare. Among children Kawasaki was less and Behcet disease markedly more when compared to western Europe figures. The observed differences may be due to genetic modifiers or environmental factors. Disclosure of Interest None declared

Background Disease Activity index for Psoriatic Arthritis (DAPSA) originally developed from patients with reactive arthritis (1). Subsequently, validation of DAPSA was also shown on psoriatic arthritis (PsA) patients (2). DAPSA includes swollen joint count (SJC) (66 joint), tender joint count (TJC) (68 joint), CRP (mg/dl), patient global assessment, and pain. However, it is well known that assessment of 28 joint is feasible than 66/68 joints especially in the rheumatoid arthritis trials. Objectives Objective of this study was to assess construct validity and sensitivity to change of DAPSA-28 in a single center biological registry. Methods Hacettepe University biological registry (HUR-BIO) is a single center biological registry. Since 2012, consequently all PsA patients with new started anti-TNF treatments were recorded. HUR-BIO included sex, age, PsA disease duration, education, body mass index (BMI). Before anti-TNF treatments, baseline disease activity parameters were recorded: SJC and TJC (28 joints), health assessment questionnaire (HAQ), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Bath ankylosing spondylitis activity index (BASDAI), visual analog scale 0–100 mm (VAS) of patient9s global assessment of disease activity (PGA), pain, and fatigue. Original DAPSA calculated to sum of CRP (mg/dl), pain VAS (cm), PGA VAS (cm), SJC (66 joints), and TJC (68 joints). We used 28 TJC/SJC instead of 66/68 joints for calculation of DAPSA, which called DAPSA-28. At baseline, all DAPSA items completed in 78 patients. The subsequent clinical visit recorded in 53 patients (4.6±3.0 months after baseline). Statistical analysis: Internal consistency was evaluated using Cronbach9s α coefficient. Construct validity was assessed by Spearman9s correlation between the DAPSA-28 scores and other measures. Sensitivity to change assessed with standardised response mean (SRM). A SRM >0.8 is considered large. Results Overall, 58 of 78 (74.4%) were female, mean age was 43.1 (11.8) years, mean PsA duration was 6.4 (5.1) years. Mean DAPSA-28 score was 20.1 (8.1). Patients with low educational level (primary scholl 22.8 (9.6) vs high scholl 16.8 (5.7), p=0.009), and high BMI (BMI >30 kg/m 2 22.4 (8.4) vs BMI 2 17.8 (7.2), p=0.011) had higher DAPSA-28 score. Mean of all DAPSA items were followed; PGA 5.8 (1.7), pain 6.3 (2.3), CRP 2.0 (2.8), SJC 1.9 (2.2), TJC 4.0 (3.6). DAPSA-28 domains had moderate internal consistency (Cronbach9s α 0.61). Correlation of DAPSA-28 with other outcome measures followed; DAS-28 (r=0.86), TJC (r=0.78), SJC (r=0.73), PGA (r=0.48), pain VAS (r=0.47), CRP (r=0.44), fatigue VAS (r=0.38), HAQ (r=0.36), ESR (r=0.35), and BASDAI (r=0.31). DAPSA-28 had excellent SRM (1.14). Conclusions DAPSA domains are almost universal for all kind of inflammatory arthritis. Simple sum of those domains seem valid and feasible in PsA patients. Although assessment of 66/68 joints may be rational particularly patients with distal interphalangeal involvement, it has feasibility issue. Our results demonstrated that DAPSA-28 had excellent capacity at sensitivity to change for clinical trials. Unfortunately, we could not compare DAPSA with DAPSA-28, future studies are needed for these comparison. References Rheumatology 2000;39:148–55 Ann Rheum Dis 2010;69:1441–7 Disclosure of Interest None declared

WOS: 000304698800041, PubMed ID: 22309845, Introduction: HLA-B*51 and HLA-B*52 are two close human leukocyte antigen (HLA) allele groups with minor amino acid differences. However, they are associated with two different vasculitides (HLA-B*51 in Beh et's disease and HLA-B*52 in Takayasu's arteritis (TAK)) and with major clinical and immunological differences. In this study, we aimed to screen a large cohort of TAK patients from Turkey for the presence of HLA-B*51 and HLA-B* 52 as susceptibility and severity factors. Methods: TAK patients (n = 330) followed at a total of 15 centers were included in the study. The mean age of the patients was 37.8 years, and 86% were women. DNA samples from the patients and healthy controls (HC; n = 210) were isolated, and the presence of HLA-B* 51 or HLA-B* 52 was screened for by using PCR with sequence-specific primers. Results: We found a significant association of HLA-B* 52 with TAK (20.9% vs HC = 6.7%, P = 0.000, OR = 3.7, 95% CI = 2.02 to 6.77). The distribution of HLA-B* 51 did not differ between TAK patients and HCs (22.7% vs 24.8%, OR = 0.9, 95% CI = 0.60 to 1.34). The presence of HLA-B* 52 decreased in late-onset patients (> 40 years of age; 12.0%, P = 0.024, OR = 0.43, 95% CI = 0.20 to 0.91). Patients with angiographic type I disease with limited aortic involvement also had a lower presence of HLA-B* 52 compared to those with all other disease subtypes (13.1% vs 26%, P = 0.005, OR = 0.43, 95% CI = 0.23 to 0.78). Conclusions: In this study, the previously reported association of TAK with HLA-B* 52 in other populations was confirmed in patients from Turkey. The functional relevance of HLA-B* 52 in TAK pathogenesis needs to be explored further., Istanbul (BAP); Marmara University (BAPKO)Marmara University, This study is funded by Istanbul (BAP) and Marmara University (BAPKO) Research Funds. The authors thank G Mumcu for her assistance with statistical analysis.

Effectiveness of anti-tumor necrosis factor (anti-TNF) agents in colchicine-resistant familial Mediterranean fever (FMF) patients has attracted attention in recent years.We analyzed the effect of anti-TNF agents on clinical findings of colchicine-resistant FMF patients with chronic arthritis and/or sacroiliitis.Data from 10 FMF patients (5 male and 5 female patients: mean age, 30.1 [SD, 8.5] years) with chronic arthritis and/or sacroiliitis who were on anti-TNF agents are reviewed. Frequency of FMF attacks before and after treatment with anti-TNF agents was recorded from hospital files. The effects of the anti-TNF treatment were determined by using the number of tender and/or swollen joints, serum acute phase reactant levels, and Bath Ankylosing Spondylitis Disease Activity Index scores. Change in urine protein loss was also evaluated in patients with amyloidosis. In 6 patients, FMF attacks had been considered to be unresponsive to colchicine, and 4 patients were partial responders before treatment with anti-TNF agents.Mean attack frequency of the patients in the 3 months' period before anti-TNF agent treatment was 3.8 (SD, 3.1). After anti-TNF treatment, in 3 patients, FMF attack frequency decreased, and in the remaining 7 patients, no attack occurred. Serum acute phase reactant levels were decreased significantly at 3 and 6 months after anti-TNF treatment (P0.05 for all). After anti-TNF treatment Bath Ankylosing Spondylitis Disease Activity Index scores were also decreased significantly (6.2 [SD], 1.7 vs. 2.1 [SD], 1.7; P = 0.012). In all 3 patients with amyloidosis, urine protein loss decreased without any increase in serum creatinine levels.Anti-TNF treatment can have beneficial effects for controlling FMF attacks in FMF patients with chronic arthritis and/or sacroiliitis.

Sleep disturbances and problems are increased in ankylosing spondylitis (AS). But much is not known in a quantitative way about sleep problems and effect of treatments on AS. This study is aimed first, to investigate sleep disturbances in AS and secondly, to evaluate the effects of anti-TNF treatment on SD in AS. One hundred seventy-one (Female/male: 90/81) AS patients fulfilling modified New York criteria and 86 (F/M: 56/30) age- and gender-matched controls without inflammatory diseases were included into the study. Demographic data and disease activity and treatments were recorded using The Bath Ankylosing Spondylitis Functional Index (BASFI) and The Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). The Medical Outcomes Study (MOS) Sleep Questionnaire was used for evaluating sleep and problems of sleep. AS patients had higher sleep disturbance scale (SDS) and sleep problem index (SPI) II scores. Group A (patients using NSAID and/or DMARD, 53.2% of patients) had higher BASDAI and BASFI compared with Group B (Patients using anti-TNF treatments) (4.29 ± 2.38 vs. 2.46 ± 2.32, p < 0.001; 1.95 ± 2.15 vs. 0.93 ± 1.31, p < 0.001, respectively). Whereas Group A had higher scores of SDS, awaken short of breath or headache, somnolence, and SPI-II than controls, none of the sleep parameters were statistically different between patients on anti-TNF treatments and controls. BASDAI was positively correlated with SPI-I, SPI-II, SDS, and somnolence scale. AS patients had increased sleep problems and disturbances compared with controls. Anti-TNF agents improve significantly these problems. Sleep problems are significantly correlated with the disease activity.

WOS: 000281954700013, PubMed ID: 20542893, Objective. Disease Extent Index-Takayasu (DEI.Tak) is a new index developed for the follow-up of Takayasu's arteritis ( TA), assessing only clinical findings without the requirement of imaging. We investigated the effectiveness of DEI.Tak in assessing disease activity and progression by comparing with physician's global assessment ( PGA) and active disease criteria defined by Kerr et al. Methods. The initial DEI.Tak forms were filled in for 145 TA patients cross-sectionally to detect the baseline damage and after 29.8 (31) months (n = 105, 144 visits) only by including the new/worsening symptoms within the past 6 months. Results. At baseline, all patients had a DEI.Tak > 0 [mean (s.d.): 7.6 (4.2)]. At this evaluation, 62% of the patients had active, 16.2% had persistent and 21.8% had inactive disease according to the PGA. At follow-up, in 69% of the patients the DEI.Tak score was 0. However, 14% of them were accepted as having active and 17% persistent disease according to PGA. In contrast, 18% with a DEI.Tak epsilon 1 were inactive according to PGA. Patients with active or persistent disease with PGA had higher DEI.Tak compared with inactives [1.3 (1.9), 1 (1.3) vs 0.2 (0.6), respectively; P < 0.001]. According to Kerr's criteria 27% were active. The total agreement between DEI.Tak and Kerr's criteria was 94% (kappa = 0.85). Compared with PGA, Kerr's criteria had a total agreement of 74% and DEI.Tak 68%. Conclusion. During follow-up, most TA patients showed no clinical activity with DEI-Tak. Although the agreement between Kerr's criteria and DEI.Tak seemed very good, using Kerr's criteria instead of DEI.Tak increased the consistency with PGA, which could be explained by the influence of imaging data and acute-phase reactant levels on the physician's decisions.

A possible relationship between Takayasu arteritis (TA) and tuberculosis (TB) has been suggested. An increased frequency of tuberculin skin test (TST) was observed in TA patients. Quantiferon-TB Gold test (QFT) is a new in vitro assay measuring interferon-gamma response to M. tuberculosis antigens and helpful in diagnosing latent TB infection. The aim of this study was to investigate latent TB infection among TA patients by the use of both TST and QFT Gold test. Ninety-four (male/female: 7/87) TA patients fulfilling ACR 1990 TA criteria from three different university hospitals in Turkey and 107 control subjects without inflammatory diseases were included in the study. Data about medical history (TA and TB) were collected for both groups. TST and QFT were performed. TST valuesor =5 mm for TA patients andor =15 mm for controls was accepted as TST positivity. Even though TA group was older (40 +/- 12 vs. 32 +/- 8, P0.001), there was no significant difference between TA patients and controls regarding demographic characteristics. Six TA patients and one control had a history of previous TB infection (P = 0.054). Although TST positivity was higher in TA group [55 patients (62.5%) vs. 24 controls (41.4%), P = 0.008], QFT positivity was similar between two groups [21 patients (22.3%) vs. 24 controls (22.4%), P0.05]. QFT was negative in two of six TA patients with previous TB history. Rate of latent TB infection in TA patients measured with QFT is no more than controls. QFT seems to be a good and favorable test compared with TST in detecting LTBI in TA.

Although nerve conduction study (NCS) is the method most frequently used in daily clinical practice to confirm clinical diagnosis of Carpal tunnel syndrome (CTS), ultrasonographic (US) measurement of the median nerve cross-sectional area is both sensitive and specific for the diagnosis of CTS. Moreover, an algorithm evaluating CTS severity based on CSA of median nerve was suggested. This study is aimed to investigate the clinical usefulness of this algorithm in assessing CTS severity. The patients underwent a full clinical examination, including Tinel and Phalen test, and questioned about symptoms and the secondary causes of CTS. All of the patients refilled a Turkish version Levine Boston Carpal tunnel syndrome questionnaire (BQ) and the visual analog scale for pain (VAS 0-100 mm) A MyLab 70 US system (Esaote Biomedica, Genoa, Italy) equipped with a broadband 6-18 MHz linear transducer was used for US examination. The cross-sectional area of the median nerve was measured at the proximal inlet of the carpal tunnel (US cut-off points that discriminate between different grades of CTS severity as 10.0-13.0 mm(2) for mild symptoms, 13.0-15.0 mm(2) moderate symptoms and15.0 mm(2) for severe patients). Nerve conduction studies were carried out, and severity of electrophysiological CTS impairment was reported as normal, mild, moderate, severe and extreme. The agreement between NCS and US in showing CTS severity (normal, mild, moderate and severe) was calculated with Cohen's kappa coefficient. Ninety-nine wrists of 54 patients (male/female: 4/50) were included in the study. Mean ages of patients were (+/-SD) 43.3 +/- 11 years. Forty-nine patients had idiopathic CTS, whereas five had secondary CTS (4 had diabetes mellitus and 1 had hypothyroidism). Symptoms were bilateral in 45 patients (83.3%). There were statistical differences between the groups according to electrophysiologic severity scale in terms of age (P0.001), body-mass index (P = 0.034), VAS (P = 0.014), Boston symptom severity (P = 0.013) and CSA of median nerve (P0.001). The identification of CTS severity showed substantial agreement (Cohen's kappa coefficient = 0.619) between the US and NCS. Also the four groups based on US CTS severity classification were significantly different in VAS (P = 0.017) and Boston symptom severity (P = 0.021). The median nerve swelling detected by calculation of the CSA reflects in itself the degree of nerve damage as expressed by the clinical picture. In addition to CTS diagnosis, sonographic measurement of CSA could also give additional information about severity of median nerve involvement. Using of US may cost-effectively reduce the number of NCS in patients with suspected CTS.

Cardiac involvement is one of the major problems in systemic sclerosis (SSc). Subclinical cardiac involvement has a higher frequency than thought previously. In this study we investigated whether subclinical cardiac involvement can be detected by using echocardiographic strain imaging in SSc patients without pulmonary hypertension.Echocardiographic examinations were performed to 27 SSc patients and 26 healthy controls. Left ventricular strain parameters were obtained from apical views and average strain value was calculated from these measurements.There were no significant differences between patients and controls regarding two-dimensional (2D), conventional Doppler and tissue Doppler velocity measurements. Strain was reduced in 6 of 12 segments of the left ventricle (LV) and in 1 of 2 segments of the right ventricle (RV). Strain rate (SR) was reduced in 2 of 12 segments of the LV and 1 of 2 segments of the RV in SSc patients as compared to controls (P0.05 for all). These involvements did not match any particular coronary artery distribution. More important differences were detected by average strain and SR values of the LV between patients and controls (19.78 +/- 3.00% vs 23.41 +/- 2.73%, P0.001; 2.01 +/- 0.41 vs 2.23 +/- 0.27/sec, P = 0.026, respectively). Furthermore, carbon monoxide diffusion capacity (DLCO) in scleroderma patients significantly correlated with LV average strain (r = 0.59; P = 0.001).Evaluation of ventricular function by using echocardiographic strain imaging appears to be useful to detect subclinical cardiac involvement in SSc patients with normal standard echocardiographic and tissue Doppler velocity findings.

PubMed ID: 18375974, Objective. Takayasu's arteritis (TA) is a chronic, rare granulomatous panarteritis of unknown aetiology involving mainly the aorta and its major branches. In this study, genetic susceptibility to TA has been investigated by screening the functional single nucleotide polymorphism (SNP) of PTPN22 gene encoding the lymphoid-specific protein tyrosine phosphatase. Methods. Totally, 181 patients with TA and 177 healthy controls are genotyped by PCR-RFLP method for the SNP rs2476601 (A/G) of PTPN22 gene. Polymorphic region was amplified by PCR and digested with Xcm I enzyme. Results. Detected frequencies of heterozygous genotype (AG) were 5.1% (9/177) in control group and 3.8% (7/181) in TA group (P = 0.61, odds ratio: 0.75, 95% CI: 0.3, 2.0). No association with angiographic type, vascular involvement or prognosis of TA was observed either. Conclusion. The distribution of PTPN22 polymorphism did not reveal any association with TA in Turkey. © The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved., SAG-YYP/0007-15/01/ 2007 British Association for Psychopharmacology, Funding: The study is supported by Marmara University Scientific Research Fund (BAPKO) (project no: SAG-YYP/0007-15/01/ 2007) and Istanbul University Research Fund (BAP).

We report here the case of a 18-year-old young man with Behcet's disease who had suffered deep venous thrombosis of the right femoral and popliteal veins. Consequently, right sciatic nerve injury, drop foot and tightness of the achilles tendon also ensued. The clinical scenario was further challenged by demonstration of the agenetic inferior vena cava and epidural vein dilatations compressing the lumbar nerve roots. To the best notice of the authors, this is the first patient encompassing all these complications in the literature concerning Behcet's disease.

Colchicine is a mainstay of treatment in familial Mediterranean fever (FMF); however, 5%-10% of patients do not respond to colchicine. Adenosine triphosphate-binding cassette subfamily B member 1 (ABCB1 or MDR1) is a drug transporter that extrudes colchicine out of cells. ABCB1 gene 3435C to T polymorphism has been demonstrated to alter MDR1 expression in mononuclear cells. Thus, the amount of MDR1 in mononuclear cells may alter response to colchicine. We investigated the association between MDR1 3435C to T polymorphism and colchicine response in patients with FMF.Patients (n = 120) were examined for colchicine responses. ABCB1 gene 3435C to T genotypes were determined to analyze associations with colchicine resistance.Ninety-eight patients were evaluated as responders and 22 as nonresponders. The distributions of ABCB1 CC, CT, and TT genotypes were significantly different between responsive and nonresponsive groups (chi-square = 6.86, p = 0.032). Colchicine resistance was significantly higher in patients harboring the C allele than in patients with TT genotype (odds ratio 9.71, 95% CI 1.58-58.76). Similarly, the mean colchicine dose to prevent remission was significantly lower in the TT group compared with subjects with the C allele (p = 0.014).Our study revealed an association between 3435C to T polymorphism and colchicine response in patients with FMF. Patients with the TT genotype for the ABCB1 3435C to T variant responded better to colchicine in terms of treatment efficacy and colchicine dose requirements.

This study was conducted to define the value of procalcitonin (PCT) levels in the differential diagnosis of abdominal familial Mediterranean fever (FMF) attacks from acute appendicitis. From October 2006 to January 2007, 28 FMF (12 males, 16 females) patients with acute abdominal attacks and 34 patients (18 males) with acute abdomen who underwent operation with the clinical diagnosis of acute appendicitis were consecutively enrolled in this study. FMF patients with concurrent infectious diseases were excluded. PCT values were measured by an immunofluorescent method using the B.R.A.H.M.S. PCT kit (B.R.A.H.M.S. Diagnostica, Berlin, Germany). Erythrocyte sedimentation rate (ESR), C-reactive proteins (CRP) and leucocyte levels were also noted. Mean disease duration in FMF patients was 9.6 +/- 8.1 years (range 2-33 years) and all were on colchicine therapy with a mean colchicine dosage of 1.2 +/- 0.4 mg/day. Among the operated patients, 5 were excluded: 3 patients had normal findings and 2 had intestinal perforation (PCT levels were 2.69 and 4.93 ng/ml, respectively) at operative and pathologic evaluation. There were no significant differences between the two groups with respect to gender and age (p was not significant (NS) for all). Acute phase reactants and PCT levels were increased in patients with FMF compared to patients with acute appendicitis (0.529[0.12 +/- 0.96] vs 0.095 [0.01-0.80] p < 0.001, respectively). PCT levels higher than 0.5 ng/ml were found in 11% (3/28) of FMF patients compared to 62% (18/29) of acute appendicitis patients (p < 0.001). Our results suggest that PCT could be a useful test in the differentiation of abdominal FMF attacks from acute appendicitis, though it should not supplant more conventional investigations.

Familial Mediterranean Fever (FMF) is characterized by recurrent acute attacks of fever and serositis, and colchicine is the primary treatment. The pathogenesis of the disease has not been fully understood. Resistance to colchicine remains to be a problem in up to 30% of the patients and yet there seems to be no alternative treatment. In this study our objective was to investigate whether a selective serotonin re-uptake inhibitor (SSRI) could affect the attack frequency and acute phase response in FMF patients who were unresponsive to colchicine. We retrospectively evaluated the hospital files of 11 colchicine-unresponsive FMF patients who had been treated with SSRIs. According to the records and re-evaluation of the patients, the total number of the FMF attacks was calculated before and after the SSRI, adjunct to colchicine. The laboratory values including erythrocyte sedimentation rate, C-reactive protein, fibrinogen and white blood cell counts were also noted before and after the SSRI treatment from their hospital files. The mean attack frequency before adding SSRI to colchicine was 8.09 +/- 3.53 per 6 months, and at the end of this period there was a great decline in the number of mean attack frequency (0.36 +/- 0.50 attacks per 6 months) (p < 0.001). Acute phase reactants were significantly decreased after SSRI treatment (p < 0.001). All of the colchicine-unresponsive patients had depression and 3 of those patients also had fibromyalgia. SSRIs appear to be useful adjuncts in the management of FMF patients who continue to have attacks despite regular colchicine treatment.

Familial Mediterranean fever disease (FMF) is an autosomal recessively inherited disease characterized by recurrent, self-limited febrile episodes (attacks) with serositis, synovitis, and occasionally skin involvement. The disease primarily affects people of eastern Mediterranean descent, typically presenting at age

Background Conventional angiography is the diagnostic imaging criteria in 1990 ACR Takayasu Arteritis (TA) Classification Criteria. In recent years, non-invasive imaging methods such as Doppler Ultrasonography (Doppler US), Computerized Tomography Angiography (CT-Ang) and Magnetic Resonance Angiography (MR-Ang) are frequently used in diagnosis and/or follow up in TA. However, studies investigating optimal timing of follow up imaging are rare.1 Objectives This study is aimed to investigate the radiologic changes in vessel involvements of TA patients approximately 1-year after diagnosis. Methods Database of our Vasculitis Center is retrospectively analyzed and 97 (Female/Male: 90/7) TA patients were included into the study. Demographic, clinical, radiological and therapeutic findings of patients were recorded. Patients with the same imaging method after one year of diagnosis were recruited into the further analysis. Radiological changes in the involved vessels were investigated (Doppler US, CT-Ang, MR-Ang). The effect of different immunosuppressive agents on vessel involvement changes were also studied. Results Mean ages and disease duration of patients were 43.0 (14.4) and 9.0 (5.1) years, respectively. Subclavian arteries and common carotid arteries were the most frequent involved vessels. All patients were on corticosteroid therapy and additional immunosuppressive agent. Most frequent initial immunosuppressive agents were methotrexate (n=35), cyclophosphamide (n=33), and azathioprine (n=20). Totally 13 (13.4%) patients had been treated with biologic therapies during all follow up. Fifty patients had a follow-up imaging with the same modality at 12±3 months. Sixty-eight percent of patients had stabile vessel involvements whereas 28% had progression and 4% had regression. No difference was found in radiological changes regarding patients with usage of different immunosuppressive agents (p=0.634). [Table 1][1] shows the comparison of the diagnostic accuracy, coefficient of agreement and AUC results of 4 criteria. View this table: Table 1. Vessel involvements of TA patients in first imaging modality Conclusions Follow-up imaging modalities were investigated in a larger TA patient group than previous studies.1 Almost 30% of TA patients in our Vasculitis center had progression in follow up imaging. This finding was concordant with previous literature. A follow up imaging at around 1-year of treatment, seems feasible in management of TA patients. References 1. Mavrogeni S et al. The role of multimodalityimaging in the Evaluation of TakayasuArteritis. Sem ArthRheum 2013;42:401-412. Disclosure of Interest None declared [1]: #T1

Background Ocular involvement is one of the major reason of disability for Behcet disease (BD). BD may be a cause of acquiered blindness particularly among young adult population. Objectives The objective of this study was to assess prevalence of severe visual loss with BD among blind population. Methods Demographic data of blind people was recorded and followed by Ankara metropolitan municipality education and technology center. There were 675 blind people at these center. Telephone numbers were not found at 131 peoples and we also did not reach 250 peoples by phone. At the end, medical history was took from 294 peoples. Participants were searched by standard questionnaire for BD. We also searched to cause of blindness either acquired or congenital. If patients had BD or suspicious BD, they were recalled for future investigation for BD by a rheumatology and ophtalmology physician. Patients with BD were assessed for disease characteristics, time of visual impairment. Results Overall, 213 of 294 (72.4%) peoples were male. One hundred nine of 294 (37.1%) peoples had acquired blindness. Six of those 109 (5.5%) patients had BD. Five of 6 patients already had BD, however, 1 of 6 patients did not known their diagnosis. All of BD were male sex, median age was 38.6 years old (range 24-64 years).The median age for diagnosis was 20 years old (range 14-35 years) and the median duration from diagnosis to visual loss was 2.5 years (range 1- 11 years). All patients with BD had recurrent oral aphtous ulcer and ocular involvement. Other diseease characteristics were genital ulcers (83%), skin involvement (67%), arthritis (50%), arthralgia (66%), neurological involvement (17%). None of the patients had deep venous thrombosis. Uveitis location were known at 3 patients, one had both anterior and posterior uveitis, 2 patients had only posterior uveitis. Conclusions BD is still one of the cause of acquired visual impairment in Turkey. In a Japan study at 1965, on the other hand before immunosuppressive treatment period, BD was the 12% of cause of acquired blindness (1). Today, immunosuppressive treatments use in routine practice, and frequency of BD among visual loss population looks tendency of decrease. However BD still seems to be important cause of acquired visual loss, particularly prevalant country such as Turkey. References Shimizu T, Tanaka I, Ogino K. Current status of Behcet9s disease in Japan. Igaku no Ayumt 75:332-341, 1970 Disclosure of Interest None declared

Background Anti-TNF alpha (TNFi) drug survival is one of the relevant outcome measure in rheumatological diseases. Objectives Objective of this study was to compare TNFi drug survival in ankylosing spondylitis (AS) and axial spondyloarthritis (AxSpA). Methods HUR-BIO (Hacettepe University Rheumatology Biologic Registry) is a single center biological registry since 2005. HUR-BIO biological dataset included demographic data, education level, co-morbidities, smoking, BMI, HLA-B27, switch ratio, baseline and follow-up disease activity parameters (such as BASDAI, BASFI, CRP, ESR, global VAS). Patients with lost of follow-up searched regarding to last TNFi prescription date with either local computer system or national social security institution database. Inition and last date of TNFi were noted from those systems. First TNFi drug switch date (either advers event or inefficacy) was accepted as main variable for drug survival. Kaplan-Meier plots and log rank tests were used to assess drug survival. HUR-BIO is not sponsored by any pharmaceutical company. Missing data ratio of baseline disease activity measures were 38.5% at BASDAI, 22.1% at ESR, 23.9% at CRP and 60.6% at BASFI. Results There were 874 patients (747 AS and 127 AxSpA). Mean age was 40.7±11.4 years old. Overall, 533 (61%) of patients were male, mean disease duration was 8.3±7.1 years and mean symptom duration was 12.8±8.9 years. Initial biological drugs were etanercept 346 (39.6%), infliximab 270 (30.9%) and adalimumab 258 (29.5%). Overall, initial biological drugs were continued at 497 (56.9%) patients. Of 245 (29.7%) patients had at least one biological switch. First biological drug survival was more frequent at male sex (61.4% vs 49.8%, p=0.001) and patients with highest ESR level (31.6 (23.7) vs 27.8 (22.3) mm/hour, p=0.041). In logistic regression analysis, male sex had tendency of better TNFi drugs survival [OR 1.65 (95%CI 0.96-2.83), p=0.070]. Patients with use usage of etanercept had tendency of more frequent drugs survival than monoclonal antibodies (log-rank p=0.057) (Table and Figure 1). Retention rate was similar at both AS and AxSpA patients. Conclusions In this single center observational registry, etanercept had tendency of better drug retention rate than monoclonal antibodies. Overall, anti-TNF drugs retention rate was well both AS and AxSpA than rheumatoid arthritis. On the other hand, certain confounder factors such as baseline disease activity, functional status were not known in whole patients, thus our results should evaluate in this limitation Disclosure of Interest None declared

Background Five Factor Score (FFS) has been used in evaluating prognosis in systemic vasculitis including GPA. A revised FFS was published in 2011.1 Objectives This study is aimed to investigate usefullness of revised FFS and its relationship with mortality in GPA. Methods Totally 77 (F/M: 41/36) GPA patients from database of Vasculitis center was retrospectively analyzed. Demographic and clinical characteristics were recorded. Five factor score of patients were calculated. Patients who had been lost from follow up was evaluated by phone call (n=14). Survival analysis was done by Kaplan-Meier method. Results Mean ages of patients were 50±15 years. Distribution of organ involvements were as; ear-nose –throat (ENT): 81.7%, pulmonary: 83.3%, renal: 59.5%, skin: 32.4%, musculoskeletal: 68.6%, eye: 23.3%. ANCA was positive in 74% of patients (cANCA/ pANCA:65%/14.7%). Most frequent treatment protocol in remission induction was corticosteroid + cyclophosphamide (75.5%). Rituximab was used in 12 (15.6%) patients. Seven of patients with renal involvement (n=23) were died, whereas three patients had renal transplantation and four had been on hemodialysis therapy. Totally 14 patients had died. Causes of mortality were co-infections in chronic renal failure (n=2), pulmonary hemorrhage (n=3), pulmonary hemorrhage and infection (n=2), gastrointestinal perforation (n=2), infective endocarditis (n=1), not known (n=4). Survival analysis was done with 63 patients. Median survival time was 36 (1-264) months. One-year and three-year survival rate was 92% and 81%, respectively. Twenty-three (36.5%) of patients had follow-up ≥5 years. Survival rate was statistically different between groups regarding to FFS scores (Table, p=0.003, Figure). 3-year survival rate was higher in patients with ENT involvement compared to without (87% vs 58%, p=0.052). Conclusions Three-year survival rate of GPA patients in our Vasculitis center is 81%. Survival rate was lower in patients with higher FFS scores concordant with literature. According to our results, FFS was found useful in evaluating prognosis in GPA. References Guillevin L ve ark. The Five Factor Score revisited. Medicine 2011; 90:19-27. Disclosure of Interest None declared

Background ASAS partial remission is relevant treatment target for ankylosing spondylitis (AS) and axial spondyloarthritis (AxSpA) patients. Objectives Objective of this study was to assess ASAS partial remission ratio during anti-TNF treatment in real life experience. Second objective was to assess related factors of ASAS partial remission at both AS and AxSpA. Methods HUR-BIO (Hacettepe University Rheumatology Biologic Registry) is a single center biological registry since 2005. HUR-BIO includes patients with AS and AxSpA under anti-TNF drugs. HUR-BIO biological dataset included demographic data, education level, co-morbidities, smoking status. After september 2012, outcome measures of ASAS were recorded regularly every six months or during switched of anti-TNF. Outcome measures of HUR-BIO include BASDAI, BASDAI 2, 5 and 6. questions, BASFI, VAS global, VAS pain, VAS fatique, ESR and CRP. ASAS partial remission was defined as less than 20/100 mm of BASFI, back pain, morning stiffness and VAS global. ASAS partial remission was calculated according to last control visit of patients. Results There were 874 patients (747 AS and 127 AxSpA) in HUR-BIO dataset. Among 716 of 874 (81.9%) patients [602 (84.1%) and AxSPA 115 (15.9%)] had at least one control visit during last 2 years. Overall, 452 of those 716 (63.1%) patients were male. Mean age (41.3±11.3 vs 35.5±9.7, p Conclusions At real life experience, ASAS partial remission was reached only almost 29% of AS and 23% of AxSpA patients. We should keep in mind that, almost 20% of patients were not followed in HUR-BIO and analysis was performed only patients with continue anti-TNF treatments. Patients with HLA B27 positive, high baseline CRP and disease duration less than 5 years were candidate for remission. Disclosure of Interest None declared

Background Low back pain (LBP) and chronic LBP are one of the most frequent medical conditions in the population. Objectives The present population-based epidemiological study aimed to determine the frequency of LBP and CLBP and associated factors. Methods In 2013, a cohort for chronic diseases was initiated by the Turkish Society of Internal Medicine. CLBP was defined as a LBP lasting for longer than3 months. Demographic characteristics and habits of the participants, self-reported joint swelling, and presence of recurrent aphthous stomatitis were questioned. Results Data of 11,049 participants (mean age, 44.6±16.4 years; 56.1% males) were evaluated. Of the participants, 44.3% had LBP, 22.7% had CLBP, 19.8% had self-reported joint swelling, and 13.2% had oral aphthous lesions. In multivariate analysis, CLBP was positively associated with female gender, advanced age, low education level, oral aphthous lesion, self-reported joint swelling, smoking, and high body mass index, whereas a negative correlation was determined with alcohol consumption (Table 1). Conclusions In the present study, the frequency of LBP and CLBP were about 44% and 23%, respectively. In addition to previously reported classical risk factors, recurrent aphthous lesions were associated with both LBP and CLBP. Financial Interests The present study was unconditionally funded by the Turkish Society of Internal Medicine, which provided financial support for data collection and analysis and manuscript preparation. The Turkish Society of Internal Medicine had no contribution to the study design and interpretation of the results. The authors declare no conflict of interests. Disclosure of Interest None declared

Background There are still controversial data regarding the development of malignancy after TNF inhibitor (TNFi) therapy. Objectives The objective of this study was to assess whether TNFi increases the risk of cancer development in Turkish Rheumatoid Arthritis (RA) patients in a single center real life experience Methods HUR-BIO (Hacettepe University Rheumatology Biologic Registry) is a single center biological registry since 2005 that include 815 RA patients under biological treatments. Overall, 679 of 815 patients were treated with TNFi drugs. Collected data includes demographic data, co-morbidities, smoking, switch ratio, baseline and follow-up disease activity parameters. Malignancy history before TNFi drugs were recorded from local database. After TNFi exposure, malignancy data were obtained from local database, social security administration data and Republic of Turkey Ministry of Health data. Development of cancer was assessed by calculating 100 patient–year exposure. These results are compared with Turkey 2005 Cancer Data and standardized incidence ratio (SIR) were identified (1). SIR was calculated by multiplying the ratio between expected number of cancers and observed number of cancer by 100. Results The mean age of 679 RA (77.8% women) patients was 50.4 (13.1) years and total duration of TNFi was 1623 patient-years. Initial TNFi were adalimumab 223 (32.8%), etanercept 321 (47.3%), infliximab 115 (16.9%) and golimumab 20 (3.0%). Before TNFi drugs, 2 patients had malignancy history (one breast cancer and one prostate cancer). During follow-up period, 5 additional cancer were obtained (Table 1). SIR was calculated as 1.26 in patients with advanced malignancy after anti-TNF drugs (confidence interval could not be obtained because of the small sample size). Conclusions In this single center real life experience, the incidence of malignancy after TNFi drugs at RA patients probably remains within the confidence interval according to the healthy population. We did not have RA control groups with DMARDs, and this is the limitation of our study. References Haydaroglu A et al. Turk Onkoloji Dergisi 2007;22:22-28. Disclosure of Interest None declared

Background Hemophagocytic syndrome (HS) is a severe, potentially life-threatening, hyperinflammatory disorder. It is difficult to distinguish reactive HS (RHS) form other diseases such as sepsis, rheumatic diseases, and malignancies. Recently, Fardet et al have developed a score called HScore to help clinicians in the differential diagnosis of RHS. Objectives Our aim was to analyze the performance of HScore (with and without bone marrow criteria) for RHS diagnosis in rheumatic diseases and to determine the best cut-off values for this subgroup of patients. Methods We have retrospectively reviewed the medical records of patients with the diagnosis of RHS and rheumatic disorders who were evaluated in Departments of Rheumatology and Pediatric Rheumatology at Hacettepe University, Ankara, Turkey between 2002-2014. The first group (n=32) consisted of patients who had RHS and the control group (n=64) included patients with rheumatic diseases but not RHS. We have applied the HScore and examined the performances of different cut-off values. SPSS 15.0 for Windows is used for statistical analysis. Results We have included a total of 32 patients in the RHS group. Of these patients, 14 (43.75%) had AOSD, 10 (31.25%) SJIA, six (18.75%) SLE, and two (6.25%) had vasculopathies. The control group (n=64) was consisted of 32 AOSD, 13 SJIA and 19 SLE patients who did not have RHS. Adult/pediatric patient ratios were 20/12 for RHS and 47/17 for control group. The HScore parameters and numbers of patients according to the categories of each parameter were summarized in Table 1. The best cut-off value was 190.5 with a sensitivity of 96.9% and specificity of 98.4%. In adult and pediatric subgroups, it had a sensitivity of 95% and 100%, and specificity of 97.9% and 100%, respectively. A lower cut-off value of 155.5 seemed to perform better with a sensitivity of 96.9% and specificity of 84.4% when we excluded the bone marrow item of the HScore. This cut-off value had a sensitivity of 95% and 100%, and specificity of 80.9% and 94.1% in adult and pediatric groups respectively. Of note, in the pediatric subgroup, a higher cut-off value of 169.5 performed better with a sensitivity and specificity of 100%. Conclusions According to the original study, the best cut-off value for the HScore was 169; however, in our study group, 190.5 performed better. Our results indicate that different cut-off values should be determined for RHS according to the different underlying disorders. We do not always perform bone marrow aspiration to determine hemophagocytosis for RHS diagnosis, especially in patients with rheumatic diseases. Thus, it may be more proper to use the HScore set without the bone marrow item especially in this subset of patients. Further prospective studies are needed to evaluate the strength and potential practical usefulness of the HScore in different patient group. References Fardet L, Coppo P, Kettaneh A, Dehoux M, Cabane J, Lambotte O. Low glycosylated ferritin, a good marker for the diagnosis of hemophagocytic syndrome. Arthritis Rheum 2008;58(5):1521-7 Disclosure of Interest None declared

Background IgG4-Related Disease (IgG4-RD) is a recently recognized condition with a wide range of organ involvements.Few data exist on the global perspective of IgG4-RD regarding prevalence and the frequencies of the different clinical presentations. Objectives This study is aimed to describe the clinical and laboratory characteristics of a series of patients diagnosed with IgG4-RD in Turkey. Methods A retrospective study was performed. Demographic, clinical characteristics and imaging studies including PET-CT, in addition to serum IgG4 levels were recorded. Totally 48 pathologic specimens of the patients were re-evaluated regarding IgG4-RD morphologic findings and IgG4/total Ig G ratio. Patients were diagnosed according to diagnostic criteria for IgG4-RD. 1 Results Totally 27 patients fulfilling the diagnostic criteria were included in the study, 18 of whom (66.7%) were male. Mean age at diagnosis was 55.9±11.8 years. Retroperitoneal fibrosis is the most common finding. Fourteen (51.8%) of the patients had more than one clinical involvement. Thirty percent of patients had lymphadenopathy. Nine (33.3%) of patients had any additional cardiovascular involvement (thoracic aortitis/inflammatory abdominal aneurisms/coronary periarteritis). The global distribution by specific organ involvement was shown in Table. Three of the patients had normal serum IgG4 levels. Six patients had PET-CT. Patients had a wide range of inflammation assessed by PET-CT (SUV max levels were between 2.2-11.4). Twenty of the patients were pathologically proven IgG4-RD -16 of them had morphologic features and all had a ratio of IgG4/IgG ≥40%. Conclusions The most usual presentation of IgG4 related disease in our study is middle aged males and as retroperitoneal fibrosis. Cardiovascular involvement, lymphadenopathy, and orbital/periorbital involvements were the other frequent findings. Not also the organ involvement but also inflammation level could be in a very wide range in IgG4-RD. More collaborative studies are required in order to assess the real prevalence of the disease and the frequencies of the different presentations. References Guma M, Firestein GS. IgG4 relateddiseases. Best PractClinRheum 2012;26:425-438. Disclosure of Interest None declared

Cardiovascular disease is a major cause of death in patients with systemic lupus erythematosus (SLE) especially during the late phase of the disease. This study was conducted to evaluate B-type natriuretic peptide (BNP) levels in female SLE patients without cardiac symptoms and to investigate whether BNP levels correlated with echocardiographic findings. We studied 59 women with SLE and 33 healthy women. SLE patients with history of cardiac disease, diabetes mellitus, hypertension, and other inflammatory diseases were excluded from the study. All subjects had a complete history and physical examination. Overall disease activity assessment in SLE patients at the time of the study were derived by calculation of SLE disease activity index (SLEDAI). BNP levels were determined, and transthoracic echocardiography were performed in all subjects. There was no difference between SLE patients and controls in terms of age, blood pressure, smoking status, plasma glucose, creatinine levels, and lipid profiles. Nine patients had SLEDAI score greater than 5. All subjects had an EF greater than 55%. Diastolic dysfunction was more frequent in lupus patients than in controls (15 [25.4%] vs. 2 [6%]; p = 0.022). BNP levels of SLE patients were significantly higher than controls (median 17.9 range [5-211] pg/ml vs. median 14.7 range [5-39.7] pg/ml; p = 0.033). Twenty-seven of the SLE patients (46%) and seven of the controls (21%) had BNP levels greater than or equal to 20 pg/ml (p = 0.019). There were no differences in BNP levels of SLE patients with and without diastolic dysfunction (median 17.8 range [5-117] pg/ml vs. median 18.5 range [5-211] pg/mL; p = NS). BNP levels were positively correlated with left atrium diameter (r (2) = 0.39, p = 0.001). BNP levels did not correlate with erythrocyte sedimentation rate/C-reactive protein levels, SLEDAI scores, total steroid dosage used, or other echocardigraphic parameters. BNP levels were increased in female SLE patients without cardiac symptoms as compared to healthy controls. Although none of the SLE patients in our study had clinical signs of ischemic heart disease, increased levels of BNP in SLE patients might be a reflection of a ischemic myocardial tissue.

The objective of this study is to evaluate the efficacy, toxicity, and long-term outcome of low-dose IV cyclophosphamid therapy with repeated frequent intervals in combination with oral and IV methylprednisolone in patients with SLE nephritis. In this study, 113 patients diagnosed as having SLE and glomerulonephritis were assessed in between 1993 and 2002, with a median follow-up of 44.1 +/- 41.2 months. The patients were treated with 500 mg IV cyclophosphamide and 1 g IV methylprednisolone together with 60 mg/alternate-day oral methylprednisolone in a given schedule. The clinical and laboratory data were evaluated. There were significant improvements in the clinical and the laboratory parameters. Six patients died shortly after being hospitalized due to the disease activity itself. Eight patients were excluded from the study because of low compliance. The renal functions of the patients remained stable throughout the therapy; only 16/99 patients needed one or two additional pulses. Temporary leukopenia developed in 18/99 patients and diminished with the suspension or prolongation of the IV cyclophosphamide administration. Gastrointestinal side effects, which needed extra medication, developed in 20 patients. Hematuria was observed in 6/99 patients. Menstrual abnormalities were seen in 7/99 patients. No serious infections due to immunosuppression were observed with the given regimen. Hypertension was observed in 13 patients (minimum of 140/90 mmHg, maximum of 190/110 mmHg) and controlled with angiotensine-converting enzyme inhibitors. Mild central obesity was observed in 15 of the patients. Leimyosarcoma was observed in one patient who died during the follow-up period. Therapy starting with the weekly low-dose IV cyclophosphamide to induce remission together with IV and oral steroids, followed by prolonged intervals with the same doses for 2 years, appears to be useful in preserving renal function without major side effects in patients with lupus nephritis, in comparison to other studies.

Subclinical cardiac involvement may occur in patients with Behçet's disease (BD). The purpose of our study was to assess the noninvasive parameters of biventricular function derived from Doppler tissue imaging (DTI) of the tricuspid and mitral annular motion in BD.Twenty-one patients with BD and 20 control subjects were enrolled in this study. All subjects were selected to exclude those with cardiovascular risk factors. Standard echocardiography and pulsed DTI were obtained in every patient.Peak systolic (13.71 +/- 2.09 vs 20.01 +/- 1.57, P0.001), peak early diastolic (11.26 +/- 2.52 vs 15.35 +/- 2.06, P0.001) tricuspid annular velocities were significantly lower in patients than controls. Peak systolic (8.68 +/- 1.4 vs 12.25 +/- 1.7, P0.001), peak early diastolic (7.89 +/- 1.07 vs 9.94 +/- 1.12, P0.001), and peak end diastolic (8.30 +/- 1.32 vs 9.23 +/- 0.91, P = 0.013) lateral mitral annular velocities were significantly lower in patients than controls.We demonstrated that myocardial velocities, were affected in patients with BD. Therefore, we conclude that right and left ventricular function is impaired in patients with BD.

Behçet disease (BD) is a chronic relapsing systemic vasculitic disorder affecting the arteries, veins, and vessels of any size. Vascular lesions in BD usually represent an occlusive nature suggesting a hypercoagulable/prothrombotic state. Coronary arteries are rarely involved in BD. In this report, a 27-year-old male patient in whom myocardial infarction developed secondary to coronary arterial thrombosis together with deep venous thrombosis was presented. This is a review of the pathologic hemostasis and the prothrombotic state of BD.

Systemic lupus erythematosus (SLE) is associated with severe and premature cardiovascular disease, which cannot be explained by traditional risk factors alone. This study aims to investigate novel cardiovascular risk factors and cardiac event predictors in inactive SLE female patients who do not have any major cardiovascular risk factors. Twenty-five inactive (SLE disease activity index score4) SLE female patients and 22 healthy control women were studied. SLE patients with a history of diabetes mellitus, hypertension, hyperlipidemia, smoking, or coronary artery disease (CAD) were excluded. Venous blood samples were analyzed for lipid subfractions and novel cardiovascular risk factors such as lipoprotein (a), homocysteine, fibrinogen, high-sensitivity C-reactive protein (hs-CRP), and serum amyloid A (SAA) levels. Endothelial dysfunction was assessed by flow-mediated dilatation (FMD) from the brachial artery at baseline and during reactive hyperemia. SLE patients and controls were similar in terms of age (40+/-10 years vs 38+/-10 years, p = NS). No significant difference was found between the groups regarding family history of premature CAD, blood pressure, body mass index, lipoprotein (a), homocysteine, fibrinogen, SAA, apoprotein A-1 and B levels. Compared with the controls, SLE patients had higher levels of hs-CRP [median (range): 1.82 (0.02-0.98) vs 0.68 (0.02-0.35), p=0.04]. FMD was lower in SLE patients than controls (7.1+/-2.1 vs 11.4+/-1.2%, p0.001). Increased levels of hs-CRP and decreased FMD were found in inactive SLE patients. Increased hs-CRP levels may reflect ongoing low-grade inflammation that could be a cause of impaired FMD in SLE patients. These findings suggest that SLE patients without traditional major cardiovascular risk factors may have increased risk of cardiovascular disease and future cardiac events.

Although autopsy studies have documented that heart is affected in most of systemic lupus erythematosus (SLE) patients, clinical manifestations occur in less than 10%. QT dispersion, a new parameter that can be used to assess homogeneity of cardiac repolarization and autonomic function, has not been studied in SLE patients. The aim of our study was to evaluate the QT dispersion (QTd) in SLE patients without overt cardiac involvement. Eighty-three patients with a diagnosis of SLE (mean age 41+/-13) and age- and sex-matched 77 healthy control subjects (mean age 43+/-10) were enrolled in the study. All subjects had their complete history taken, laboratory examination, and transthoracic echocardiography (ECG). Patients with cardiac disease, hypertension, diabetes, or taking medications that may effect QTd or any ECG abnormalities were excluded. Resting 12-lead ECG were recorded for measurement of QTd. None of the patients and control subjects had overt cardiac involvement. The mean SLE duration was 86.5+/-15.4 months. QT dispersion was significantly greater in SLE patients than incontrol subjects (55.2+/-24.7 vs 20.7+/-5.3 ms, respectively; p0.001). There was no correlation between QTd and duration of SLE, SLEDAI-K score, corticosteroid usage, and presence of anti SS-A antibody. QT dispersion is significantly increased in SLE patients without overt cardiac involvement. Our result suggests that prolonged QT dispersion can be a useful noninvasive and simple method for early detection of cardiac involvement in SLE patients.

Adult-onset Still's disease (AOSD) has often been regarded as the adult spectrum of systemic juvenile idiopathic arthritis (sJIA). The present study aims to compare the clinical and laboratory features, the disease course and the response to treatment in patients having AOSD with those having sJIA. Retrospective review of all available data that were filled out by adult and paediatric rheumatologists from six centers using a standard data extraction form was performed. A total of 95 patients with AOSD and 25 patients with sJIA were recruited for the study. The frequency of fever, rash, myalgia, weight loss and sore throat was higher in patients with AOSD. The pattern of joint involvement differed slightly. Laboratory findings were similar in both groups, except that liver dysfunction and neutrophilia were more common among adults. A multiphasic pattern dominated the childhood cases, whereas the most frequent course was a chronic one in adults. Corticosteroids and methotrexate were the most commonly employed therapy; however, chloroquine was another popular therapy in the adult group. We showed a difference in the rate of clinical and laboratory features between patients with AOSD and those with sJIA. AOSD and sJIA may still be the same disease, and children may simply be reacting differently as the result of the first encounter of the putative antigens with the immune system.

Hyperfunction of neutrophils is a characteristic finding in Behçet's disease (BD). Microbial agents have been proposed as causative agents in the disease flares. Fc gamma receptor 1 (CD64) is not normally expressed by neutrophils of healthy individuals, but is upregulated by these cells in response to microbial wall components and proinflammatory cytokines. The degree of polymorphonuclear leukocyte (PMN) CD64 expression is different in autoimmune diseases and systemic infectious diseases. We investigated PMN CD64 expression in patients with BD.Thirty-seven patients with active BD (M/F: 18/19, mean age: 34.4 +/- 9.7 yrs), 35 patients with inactive BD (M/F: 11/24, mean age: 35.9 +/- 11.6 yrs), 27 patients with culture proven infections (M/F: 19/8, mean age: 54.4 +/- 15.2 yrs), 31 healthy controls (M/F: 14/17, mean age: 37.7 +/- 8.7 yrs), and 42 patients with active inflammatory disease (M/F: 13/29, mean age: 39.3 +/- 14.9 yrs) were enrolled in this study. Flow cytometry was used to assess the prevalence of CD64-bearing PMN in whole blood samples.The prevalence of CD64-bearing PMN was significantly higher in patients with infectious disease (77.1 +/- 18.4), inflammatory disease (37.1 +/- 27.5), and active BD (48.9 +/- 22.5) than in healthy controls (9.5 +/- 7.8) or patients with inactive BD (12.9 +/- 9.5). CD64 expression was similar in controls and patients with inactive BD. In the infectious disease group, expression of CD64 was significantly higher than in the active BD and active inflammatory disease groups, while there was no significant difference between the groups of patients with active BD and inflammatory disorders.Neutrophil CD64 expression increases during exacerbation of BD. This increase appears to be a non-specific inflammatory response and does not reflect PMN activation triggered by a living microorganism.

The aim of this study was to evaluate the clinical and laboratory features, the treatment approaches, and the long-term outcome of patients with Wegener's granulomatosis (WG) who were followed up in our hospital. The hospital files of the patients with the diagnosis of WG who were followed up between the years 1985 and 2003 in Hacettepe University Hospital were retrospectively evaluated. Male/female ratio was 12:8. The mean age was 39 years (range 20-65 years). Constitutional symptoms and upper and lower airway involvement were seen in 95% of all patients. Renal and musculoskeletal symptoms were seen in 90 and 80% of the patients, respectively. Five patients were treated with oral monotherapy (three with methylprednisolone and two with cyclophosphamide). Three patients were given a combination of orally administered cyclophosphamide and methylprednisolone. Ten patients were treated with pulse cyclophosphamide and methylprednisolone combination together with oral alternate-day methylprednisolone therapy. The remaining two resistant patients were treated with pulse cyclophosphamide, methylprednisolone, and intravenous immunoglobulin combination together with oral alternate-day methylprednisolone. Four patients died because of the disease activity. Intravenous pulse therapies with oral, alternate-day methylprednisolone were well tolerated. Sixteen patients experienced long-term remission after immunosuppressive treatment. Eleven patients have been asymptomatic for more than 12 months. In five patients, residual symptoms persisted: constitutional symptoms and renal and respiratory tract symptoms in varying combinations. The demographic and laboratory findings in this trial were similar with those of the previous results. Alternate-day glucocorticoids plus cyctotoxic drugs may be beneficial in patients with WG.

PubMedID: 15880831 Objective. Scleroderma (SSc) is an autoimmune disease of unknown etiology. The disease is 3-8 times more frequent in women than in men. The role of X chromosome inactivation (XCI) in the predisposition of women to autoimmunity has been questioned. Until now this has not been illustrated experimentally. This study was undertaken to test the hypothesis that disturbances in XCI mosaicism may be involved in the pathogenesis of the disease in female patients with SSc. Methods. Seventy female SSc patients and 160 female controls were analyzed for the androgen receptor locus by the Hpa II/polymerase chain reaction assay to assess XCI patterns in DNA extracted from peripheral blood cells. Furthermore, skin biopsy samples were obtained from 5 patients whose blood revealed an extremely skewed pattern of XCI, and the analysis repeated. Since microchimerism in SSc was reported, Y chromosome sequences were investigated in all samples. Results. Skewed XCI was observed in DNA from peripheral blood cells in 35 of 55 informative patients (64%), as compared with 10 of 124 informative controls (8%) (P < 0.0001). Extreme skewing was present in 27 of the patient group (49%), as compared with only 3 of the controls (2.4%) (P < 0.0001). However, XCI was random in all skin biopsy samples. The potential contribution of microchimerism to the random XCI pattern is highly unlikely based on the medical histories of the patients. Conclusion. Skewed XCI mosaicism may play a significant role in the pathogenesis of SSc. © 2005, American College of Rheumatology.

To evaluate the levels of endogenous interferon-gamma (IFN-gamma) in patients with familial Mediterranean fever (FMF).Plasma levels of IFN-gamma were assayed in 29 FMF patients in attack-free period (mean age: 32, min-max: 17-48; male/female: 10/19), 18 FMF patients with acute FMF attack (mean age: 32, min-max: 19-50; male/female: 8/10), and 19 healthy controls (mean age: 31.94 +/- 1.50, min-max: 23-42; male/female: 11/8). IFN-gamma levels were also compared among colchicine treated and untreated groups.Median plasma IFN-gamma levels were significantly higher in patients both with and without FMF attack than the control group (P0.05). Moreover, plasma IFN-gamma levels were higher in patients with acute FMF attack compared to patients in attack-free periods (P0.05). Plasma levels of IFN-gamma were comparable in colchicine treated and untreated groups.Our results suggest that IFN-gamma may contribute to the inflammatory cascade of FMF.

Local functional renin-angiotensin systems (RAS) have been demonstrated in many organ and tissue systems. Angiotensins, the effector growth factors of the RAS, are essentially cytokines and growth factors which actively contribute to many inflammatory reactions. Among the components of RAS, angiotensin-converting enzyme (ACE) and renin have been previously investigated separately in RA. In this study, ACE levels and renin concentrations were measured in the sera of 16 patients with RA (median age: 45 (26-69), male/female: 3/13), 13 patients with osteoarthritis (OA) (median age: 55 (28-72), male/female: 5/8), and 11 healthy adults (median age: 44 (35-70), male/female: 6/5). Synovial ACE levels and renin concentrations were also measured concurrently in patients with RA and OA. Serum ACE levels were comparable between the groups. However, synovial fluid ACE levels were significantly higher in the patients with RA than in patients with OA. Likewise, synovial fluid renin concentrations were higher in RA patients than in OA patients, while serum renin concentrations were similar in patients with RA and OA and in healthy controls. Moreover, there was a significant negative correlation between the duration of the disease and synovial renin concentrations in RA patients. In conclusion, locally-generated active renin and ACE could contribute to joint destruction in rheumatoid arthritis. © Springer-Verlag 2005.

Buerger's disease is a recurrent inflammatory, nonatherosclerotic vasoocclusive disease, which typically affects small and medium-sized arteries, veins, and nerves of the upper and lower extremities. Systemic manifestations involving cerebral, mesenteric, and coronary arteries are exceptional. Moreover, multisystem involvement of 2 or more organs is extremely rare. The authors present a case of Buerger's disease in a patient who subsequently developed cerebral and bowel infarcts as well as cavernomatous transformation of the portal vein. Therefore, Buerger's disease, although rare, does have a chronic aggressive nature in some patients.

Endothelial cell activation and/or injury is a characteristic feature of Behcet's disease (BD). The local renin-angiotensin system (RAS) in the vessel wall plays a prominent role in the endothelial control of vascular tonus and contributes to inflammatory processes. Angiotensin-converting enzyme (ACE) is the regulatory component of the RAS. In this study, we investigated the distribution of different alleles of the ACE gene in patients with BD, and the influence of the I/D polymorphism on different clinical manifestations of the disease. A cohort of 90 patients with BD were evaluated for their ACE genotype (male/female: 49/41, mean age: 36.9 +/- 10.6 years, min/max: 16-66 years). The mean duration of symptoms was 9.5 +/- 6.9 years (min/max: 1-35 years). The control population was composed of 30 healthy subjects (male/female: 15/15, mean age: 31.2 +/- 7.1 years, min/max: 20-45). The distribution of DD, ID and 11 genotypes of the ACE gene was 22 (24.5%), 56 (62.2%) and 12 (13.3%) for patients with BD, and 9 (30%), 16 (53.3%) and 5 (16.7%) for healthy controls, respectively. There was no significant difference between the groups (p > 0.05). Similarly, there was no significant association between the ACE gene polymorphism and ocular, neurologic or vascular involvement of BD. The ACE gene polymorphism does not seem to play a role in the pathogenesis of BD. Moreover, possession of either the D or the I allele does not have an impact on the development of ocular, neurologic or vascular manifestations of the disease.

We reported here on three patients in whom the diagnosis of familial Mediterranean fever was established after avascular necrosis of the femoral head had been detected. The pathogenesis and the management of this rare concomitance are discussed in the light of the relevant literature.

Study Design. Validation of a translated, culturally adapted questionnaire. Objectives. To translate and culturally adapt the Turkish version of the Oswestry Disability index (ODI) (2.0), and to validate its use for assessing disability in Turkish patients with low back pain. Summary of Background Data. The ODI is a reliable evaluation instrument for disability, but no validated Turkish version is available. Methods. A total of 95 outpatients with low back pain were assessed by the ODI. Sixty-five of these patents were observed on a second occasion. Translation/retranslation of the English version of the ODI was done blindly and independently by four different individuals, and adaptus by a team. Individuals were given the ODI and other scales (Visual Analog Scale, Schober Test, and the Roland Morris Disability Questionnaire) on their first visit and a week later. Results. Scores of the two ODIs were 27 10 (SD 16.22) on day 1 and 22.88 (SD 13.94) on day 7, with an intraclass correlation coefficient of r = 0.938 (P < 0.001). Cronbach's alpha was 0.918 (day 1) and 0.895 (day 7) in the validation. Concurrent vaildity, measured by comparing ODI responses with the results of Visual Analog Scale and Schober rest, was r = 0.367 (P < 0.01), r = -0.068 (P = 0.591) for day 1, and t = 0.392 (P < 0.01), r = -0.041 (P = 0.745) for day 7, respectively. Construct validity, tested by determining the correlation between the Turkish ODI and the Turkish adaptation of the Roland-Morris Disability Questionnaire, yielded r = 0.815 (P < 0.001) on day 1 and r = 0.708 (P < 0.001) on day 7. Conclusion. The Turkish version of ODI has good comprehensibility, internal consistency, and validity and is an adequate and useful instrument for the assessment of disability in patients with low back pain.

The aim of this study was to investigate the changes in serum levels of endothelial cell injury markers, soluble (s) E-selectin and thrombomodulin (TM), in patients with rheumatoid arthritis (RA) before and after antirheumatic drug treatment and to assess the relationship between these changes and clinical responses to the drug treatment. Eleven patients with RA having active arthritis and 12 healthy volunteers were enrolled in the study. They were monitored by clinical and laboratory parameters while receiving a combination of methotrexate, hydroxychloroquine and sulphasalazine. Pre- and post-treatment clinical and laboratory parameters, including sE-selectin and sTM levels, were measured. The ages of the patients were comparable with those of the control groups. Significant improvements were detected in erythrocyte sedimentation rate, C-reactive protein, hemoglobin, morning stiffness, patients' global assessment, physicians' global assessment, number of tender joints and number of swollen joints improved at the end of the therapy (for each parameter p < 0.05). Significant improvements were detected in clinical and laboratory parameters. In the patient group there were significant decreases in the levels of sTM and sE-selectin after treatment (p < 0.05). The patient group had significantly higher sTM and sE-selectin levels than the control group at the beginning of the study (p < 0.01), but the difference returned to normal after the treatment (p > 0.05). The sE-selectin and sTM levels significantly correlated with each other, and also with clinical and laboratory findings. Combination treatment successfully treated RA patients. sE-selectin and sTM levels probably reflect disease activity and can be helpful in monitoring disease status and response to therapy. © Clinical Rheumatology 2004.

The endothelial damage of microvascular structures in systemic sclerosis (SSc; scleroderma) is associated with increased levels of endothelial adhesion molecules and endothelium-associated cytokines, including E-selectin and thrombomodulin. Although there is still no ideal specific pharmacologic therapy for SSc, cyclophosphamide has resulted in clinical improvement in patients with SSc-related active alveolitis. This study was designed to assess the expression of E-selectin and thrombomodulin in patients with early diffuse SSc, and to investigate the effects of oral cyclophosphamide combined with prednisolone therapy on the levels of these endothelium-associated cytokines and on the patients' clinical outcomes.Thirteen patients with early diffuse SSc were treated with oral cyclophosphamide (2-2.5 mg/kg/day) and methylprednisolone (30 mg/every other day) for 1 year. The outcomes were determined as clinical (skin score) and laboratory parameters (including the erythrocyte sedimentation rate, complete blood cell count, levels of C-reactive protein, antinuclear antibody, anti-double-stranded DNA, rate of creatinine clearance, and findings on pulmonary function tests, esophageal manometry, and echocardiography). The concentrations of E-selectin and thrombomodulin were measured in the pretreatment and posttreatment serum samples from the SSc patients and from 12 healthy adults as controls.In the patients with early diffuse SSc, pretreatment and posttreatment mean levels of E-selectin were 51 ng/ml (range 34.2-135.5) and 33.4 ng/ml (range 23-62.5), respectively (P = 0.01), and those of thrombomodulin were 82 ng/ml (range 35.8-120.5) and 74.6 ng/ml (range 23.3-91.3), respectively (P = 0.016). Clinical and laboratory parameters (the skin score and measures of pulmonary function [forced vital capacity and diffusing capacity for carbon monoxide]) were also improved (P0.05 for each) at the end of the followup period.Combination therapy with cylophosphamide plus prednisolone is effective in the treatment of early diffuse SSc. Circulating levels of E-selectin and thrombomodulin not only demonstrate the extent of endothelial injury and/or activation, but also could be a useful marker to monitor the disease activity in SSc.

Interactions of the FAS with FAS ligand have been proposed as a major regulatory mechanism of immune homeostasis. Soluble FAS (sFAS) acts as a competitive antagonist to FAS, thereby inhibiting FAS mediated apoptosis. sFAS concentrations have been studied in various autoimmune diseases, with controversial results. In this cross sectional study, we investigated the role of sFAS protein in attack-free patients with familial Mediterranean fever (FMF) with and without amyloidosis.Twelve FMF patients without amyloidosis (male/female: 7/5; median age 23.5 yrs, range 17-38), 10 FMF patients with amyloidosis (male/female: 5/5; median age 41.5 yrs, range 33-51), and 14 controls (male/female: 6/8; median age 46 yrs, range 38-57) were enrolled in the study. Serum sFAS concentrations were studied by ELISA.Median serum sFAS concentrations were 4630 (2580-12,270), 1338 (453-3240), and 3430 (2110-5960) pg/ml in FMF patients without amyloidosis, FMF patients with amyloidosis, and controls, respectively. Intergroup differences were all statistically significant (p0.05).Elevated serum sFAS concentrations in attack-free FMF patients might be due to dysregulated apoptosis of polymorphonuclear leukocytes together with the ongoing subclinical inflammatory activity. On the other hand, decreased sFAS concentrations could contribute to the augmented apoptosis together with the alterations in immune response leading to the amyloidosis.

Giant cell arteritis (GCA) is a common systemic vasculitis with an unknown etiology. It mainly affects people older than 50 years of age and often presents with symptoms such as headache, jaw claudication, visual loss, polymyalgia rheumatica and increased erythrocyte sedimentation rate (ESR). Established blindness is irreversible if the steroid treatment is not administered within a few days. Here, we report a case of GCA in a patient with a normal ESR whose left eye perceived just light at the initiation of treatment. Immediately prior to the combined treatment with high dose oral steroids and cyclophosphamide, the ESR level had increased to 80 mm/h and the vision improved after the combined treatment four months later.

Interferon α (IFNα) has recently been introduced in the treatment of uveitis, mucocutaneous lesions, and arthritis of Behcet’s disease (BD).1–6 To our knowledge, there is currently no clinical trial which has evaluated the efficacy of IFNα treatment in the vascular or neurological involvement in BD. In this open study we evaluated the efficacy, toxicity, and tolerability of IFNα in the management of BD with ocular, articular, vascular, or neurological manifestations which had previously been unsuccessfully treated conventionally. A total of 29 patients (17 men, 12 women; mean age 33.2 months, range 16–51) who were resistant to conventional treatments were treated with systemic IFNα. Previous conventional treatments had been colchicine, aspirin, and penicillin plus sulfasalazine for patients with arthritis; or colchicine, aspirin, and penicillin plus steroids and/or immunosuppressive agents, azathioprine, cyclosporin A, or cyclophosphamide for ocular, vascular, and/or neurological involvement. The mean duration of the disease was 8.86 years (range 1–30). Four patients were excluded from the statistical analysis because of the short duration of treatment (

Thrombopoietin (TPO) is the major regulator of growth and differentiation of megakaryocytes. Recent studies have shown that TPO may also act as an acute-phase reactant, and it has been suggested as a component of inflammatory reactions. In this study our objective was to investigate serum TPO levels in patients with rheumatoid arthritis, a complex chronic inflammatory disorder not uncommonly associated with thrombocytosis. Bloodstream TPO concentrations were assessed in 13 RA patients with platelet counts between 450 and 650 x 10(9)/l, 10 RA patients with platelet counts >650 x 10(9)/l, 15 RA patients with normal platelet counts and 12 healthy controls. RA patients with normal platelet counts had TPO levels comparable with healthy controls. TPO concentrations in patients with mild thrombocytosis were significantly elevated, whereas patients with markedly increased thrombocyte counts had prominently decreased TPO levels. These results indicate that TPO seems to be associated with reactive thrombocytosis in RA patients with active disease. In patients with extremely increased thrombocytosis serum TPO levels might be regulated by increased platelet mass via receptor-mediated uptake and metabolism.