Objective: To assess the efficacy and safety of the IL-1b inhibitor canakinumab in all adults with refractory Still's disease identified from the National Organization For Medicines for off-label drug use. Methods: : In a retrospective longitudinal multicenter cohort of 50 patients (median age 39 years) with active Still's disease despite treatment with corticosteroids (n = 11), conventional and synthetic (n = 34) and/or biologic disease modifying anti-rheumatic drugs (n = 30), we assessed the efficacy of canakinumab 150-300 mg administered every 4 (n = 47) or 8 weeks (n = 3) as combination therapy or monotherapy (n = 7) during a median follow-up of 27 (3-84) months. Results: A complete response was initially observed in 78% of patients within 3 months (median), irrespective of age at disease onset. A partial response was evident in 20%. One patient had resistant disease. Treatment de-escalation was attempted in 15 of 39 complete responders and a complete drug discontinuation in 21 patients for 8 months (median). Eleven patients (22%) relapsed during treatment, one during de-escalation process, and 11 after treatment discontinuation. Overall, 9 of 11 relapses were successfully treated with canakinumab treatment intensification or re-introduction. At last visit, 18% of patients were off treatment due to remission and 26% due to disease activity. Canakinumab had a significant corticosteroid sparing effect allowing weaning in 21 of 41 cases. Infections (20%, severe 4%) and leucopenia (6%) led to treatment cessation in one patient. Conclusion: High rates of sustained remission were observed in this, largest so far, real-life cohort of adult patients with refractory Still's disease treated with canakinumab. (C) 2020 Elsevier Inc. All rights reserved.

Background: Interleukin-1 (IL-1) is a major mediator of the inflammatory cascade in Still’s disease and an established therapeutic target. Objectives: To assess the efficacy and safety of the IL-1b inhibitor canakinumab in adolescent and adult patients with refractory Still’s disease. Methods: We conducted a retrospective longitudinal outcome study of 50 consecutive patients aged 39 years (median, range 14-72), fulfilling the Yamaguchi disease classification criteria, with active disease despite treatment with corticosteroids (CS) (n=11) and/or methotrexate (n=9) and/or biologics (n=30) [tumor necrosis factor inhibitors (n=13), IL-6 blockade (n=7), abatacept (n=2), anakinra (n=24); ≥1 biologics (n=13)]. Canakinumab 150-300 mg was administered sc, starting every 4 (n=48) or 8 weeks (n=2), for a median of 24 months (range 3-84). Concomitant treatment included CS (n=41), methotrexate (n=12) and leflunomide (n=3). Results: Complete remission was initially achieved in 78% of patients within a median time of 3 months, irrespective of age at disease onset. Partial clinical and laboratory response was evident in 20%. Canakinumab was discontinued in one patient with resistant disease (primary failure) and in 6 out of 10 initial responders, who relapsed during treatment (secondary failure). Of 39 patients in complete remission, increase in drug administration interval and/or drug dose reduction was attempted in 7, of which only 1 relapsed, whereas drug discontinuation was attempted in 19 patients for a median time of 8 months (range 3-68), of which 8 relapsed. Overall, in half of all disease flares, canakinumab re-introduction or intensification was successful. Canakinumab had a significant CS sparing effect permitting weaning in 21 of 41 cases. Infections (20%, severe 4%) and leucopenia (6%) led to treatment cessation in one patient. Conclusion: In this largest so far real-life patient cohort with refractory Still’s disease, high rates of sustained remission were induced by canakinumab both in adolescent and adult patients. Disclosure of Interests: Katerina Laskari: None declared, Panagiotis Athanassiou Grant/research support from: MSD, Genesis pharma, Janssen, Consultant of: Roche, Genesis pharma, Janssen, Speakers bureau: MSD, Janssen, Roche, Genesis pharma, Athanasios Georgiadis: None declared, Charalampos Gerodimos: None declared, Georgia Gkoni: None declared, Dimitrios Daoussis: None declared, Theodoros Dimitroulas: None declared, Despoina Dimopoulou: None declared, Chrysoula Iliou: None declared, Ioannis Kallitsakis Grant/research support from: MSD, Speakers bureau: Genesis pharma, Bristol-Myers Squibb, Dimitrios Karamitsos: None declared, Christina Katsiari: None declared, Stamatis-Nick Liossis: None declared, Clio Mavragani: None declared, CHARALAMPOS PAPAGORAS: None declared, Dimitrios Pikazis: None declared, Ioannis Raftakis: None declared, Theodosios Sarikoudis: None declared, Loukas Settas: None declared, Prodromos Sidiropoulos: None declared, Despoina Soukera: None declared, Evangelos Theodorou: None declared, Panagiota Tsatsani: None declared, Eleni Tsiakou: None declared, Dimitrios Vassilopoulos: None declared, PANAYIOTIS VLACHOYIANNOPOULOS: None declared, Georgios Vosvotekas: None declared, Paraskevi V. Voulgari: None declared, Marina Zakalka: None declared, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer, Petros Sfikakis Grant/research support from: Grant/research support from Abvie, Novartis, MSD, Actelion, Amgen, Pfizer, Janssen Pharmaceutical, UCB

To assess the impact of golimumab on quality-of-life (QoL) and other patient-reported outcomes in patients with rheumatoid arthritis (RA) in real-world settings. GO-Q was an observational, prospective, 12-month study conducted in Greece, in naïve to golimumab patients with moderate-to-severe active RA; patients previously treated with >1 biologic (b)DMARDs or switching anti-TNFs were excluded. Primary endpoint: the impact of golimumab on QoL at 3/6/12 months, by the EQ-5Q-3L questionnaire. Other endpoints assessed the impact of golimumab on disease activity (Disease Activity Score for 28 joints with erythrocyte sedimentation rate [DAS28-ESR]), physical function (Health Assessment Questionnaire–Disability Index [HAQ-DI]), and work productivity/activity impairment (by Work Productivity and Activity Impairment questionnaire [WPAI] for RA), at 3/6/12 months respectively. Non-parametric tests were used for all comparisons. A total of 145 patients were enrolled (age and disease duration [mean±sd]: 54.6±12.2 and 4.6±5.1 years, respectively). At baseline, 78.6% of patients were bDMARD-naïve, 87.6% had received conventional synthetic (cs)DMARDs and 77.2% corticosteroids. During follow-up, golimumab was mostly (99.4%) administered in the 50mg once-monthly dose, plus background csDMARDs. Improvements in EQ-5D-3L UK index scores from baseline to 3/6/12 months were: 0.21±0.24, 0.28±0.25, 0.38±0.30, respectively (p<0.001, all comparisons). DAS28-ESR scores improved from baseline to 3/6/12 months; mean±sd scores were 5.4±0.9, 4.1±1.2, 3.7±1.2 and 3.2±1.1, respectively (p<0.001, all comparisons). Of patients attending the 12-month visit, 27.3% and 54.6% achieved DAS28-ESR<2.6 (remission) and DAS28-ESR≤3.2 (low disease activity). Improvements were observed in HAQ-DI and WPAI scores from baseline to 3/6/12 months (p<0.001, all comparisons). Mean adherence rate to golimumab was 90.3%; non-significant difference in adherence rates for bDMARD-naïve vs. -experienced (p=0.152). Healthcare resources for RA-related reasons were utilised by >93.2% of pts. In real-world settings, patients with RA receiving golimumab over 12 months experienced significant improvements in QoL, physical function and work productivity/activity impairment.

Background The different antirheumatic drugs reduce inflammation in RA patients, causing alterations in cholesterol levels (mainly decreasing LDL and increasing HDL cholesterol levels), but HDL and LDL structure and function predict cardiovascular disease better than LDL and HDL cholesterol levels. Objectives The investigation of the qualitative changes of LDL and HDL lipoprotein subclasses in RA patients who are good responders, depending on the anti-inflammatory treatment (sDMARDs vs anti-TNFa+sDMARDs) and their in between associations. Methods 85 patients ((89%) 76females) with established RA (mean disease duration ≥5 yrs), mean age 57 yrs (SD: 12yrs), without known cardiovascular disease, D/M and thyroid disorders, on sDMARD (MTX, LEF, SSZ, low dose prednisolone or combination) and naïve to biologic treatment, were divided into two groups: the 1st one of 43 patients who had DAS28>3.2 and were given in addition a TNFα inhibitor (23 patients had golimumab and 20 patients had certolizumab-pegol) for at least 54weeks (mean treatment duration 18 months) with good clinical response according to Eular and the 2nd group of 42 patients (disease control group) with DAS28≤3.2,who continued on sDMARDs and followed closely the same time period so as to ensure, as well, good clinical response without biologic therapy. Plasma electrophoresis was performed in two time points, before and after anti-TNFα administration and on the same time for the control group, with non-denaturing polyacrylamide gel (ND-PAGE) for size-based separation of lipoprotein subclasses, a standard laboratory technique that indentify various HDL subspecies separable on the basis of average diameter/size into 6 distinct subclasses (HDL-1, HDL2a, HDL2b, HDL3a, HDL3b, HDL3c), as well as LDL lipoprotein into 2 subfractions (LDL-B, LDL-A.) Results The RA patients on DMARDs had a percent reduction of LDL-B subfraction by 11.9% (p=0.014), but a significant increase of HDL-3c subclass by 2.38% (p=0.049). In a multivariate model of stepwise logistic regression the after (treatment)-LDL-B subfraction in these patients was found to have significant positive association only with pro-LDL-B subfraction (odds ratio: 10.95, %CI (1.59–62.79)). In the RA patients who took TNFα inhibitors was observed a prominent percent decrease of the more lipoprotein subclasses: LDL-B by 2.33% (p=0.005), LDL-A by 2.33% (p=0.0001), HDL3a by 11.63% (p=0.072) and HDLc by 4.65% (p=0.035). Accordingly, in stepwise logistic regression the after-(treatment) LDL-B subfraction had a significant positive association only with pro-LDL-B subfraction (odds ratio: 12.91,95% CI (2.2–75.83)), as well as, after-LDL-A with pro-LDL-A subfraction (odds ratio:36.16, 95% CI (5.05–258.89)), whereas the reduced after (treatment)-HDL3c subclass was significantly positively associated with pro-HDL3a subclass (odds ratio:8.15, 95% CI (1.04–63.65)) and after-HDLa subclass (odds ratio:22.09, 95% CI (3.56–137.18)). Conclusions The discrepancy of qualitative modification of lipoprotein subclasses (LDL and HDL) after treatment with different antirheumatic drugs (sDMARDs, anti-TNFa+sDMARDs) demonstrate their different effect on RA dyslipidemia and their subsequent antiatherogenic prospective. Disclosure of Interest None declared

OBJECTIVESTo evaluate the long-term safety of rituximab (RTX) in rheumatoid arthritis (RA) patients in daily clinical practice.METHODSThis was a multicentre (17 Greek Rheumatology sites), prospective, long-term, pharmacovigilance study of patients with moderate to severe RA and an inadequate response or intolerance to ≥1 anti-tumour necrosis factor (TNF) agents. Adverse events (AEs) were recorded and collected prospectively every 2-6 months.RESULTS234 patients (mean age: 59±12.5, 79.5% women, mean DAS28: 5.35±1.32) were included and followed for 27.7 months (median). The overall AEs, serious AE (SAEs) and serious infection (SIEs) rate were 48.36, 6.68 and 2.53/100 patient-years, respectively. Three cases of hepatitis B virus (HBV) reactivation were recorded (two in chronic and one in past HBV infection). Withdrawals due to AEs (5.6%) occurred more frequently during the first cycles of RTX therapy while repeated RTX cycles were not associated with an increased risk of AEs. There were 3 deaths with an incidence rate of 0.69/100 patient-years. Age ≥65 years was associated with a higher incidence rate ratio of AEs and SAEs as compared to <65 years (1.53, p=0.002 and 2.88, p=0.005, respectively). Drug retention rate during 434.28 patient-years of follow-up was 57.3%. Factors associated with drug discontinuation by multivariate analysis included age, baseline swollen joint count and no use of concomitant methotrexate therapy.CONCLUSIONSLong-term RTX therapy in a real-life RA cohort, did not reveal any new safety issues. Advanced age was associated with increased risk of AEs and premature drug discontinuation.

OBJECTIVES To assess in daily practice in patients with rheumatoid arthritis (RA) the effect of treatment with first tumour necrosis factor-α inhibitor (TNFi) in quality of life (Qol), disease activity and depict possible baseline predictors for gains in Qol. METHODS Patients followed prospectively by the Hellenic Registry of Biologic Therapies were analysed. Demographics were recorded at baseline, while RA-related characteristics at baseline and every 6 months. Paired t-tests were used to detect divergences between patient-reported (Health Assessment Questionnaire (HAQ), EuroQol (EQ-5D)) and clinical tools (Disease Activity Score-28 joints (DAS28)). Clinical versus self-reported outcomes were examined via cross-tabulation analysis. Multiple regression analysis was performed for identifying baseline predictors of improvements in QALYs. RESULTS We analysed 255 patients (age (mean±SD) 57.1±13.0, disease duration 9.2±9.1 years, prior non-biologic disease-modifying anti-rheumatic drugs 2.3±1.2). Baseline EQ-5D, HAQ and DAS28 were 0.36 (0.28), 1.01 (0.72) and 5.9 (1.3), respectively, and were all significantly improved after 12 months (0.77 (0.35), 0.50 (0.66), 3.9 (1.5), respectively, p<0.05 for all). 90% of patients who improved from high to a lower DAS28 status (low-remission or moderate) had clinically important improvement in Qol (phi-coefficient=0.531,p<0.05). Independent predictors of gains in Qol were lower baseline HAQ, VAS global and younger age (adjusted R2=0.27). CONCLUSIONS In daily practice TNFi improve both disease activity and Qol for the first 12 months of therapy. 90% of patients who improved from high to a lower DAS28 status had clinically important improvement in Qol. Younger patients starting with lower HAQ and VAS global are more likely to benefit.

Background Interleukin-1 (IL-1) is a major mediator of the inflammatory cascade in Familial Mediterranean Fever (FMF) and an established therapeutic target (1). Objectives To assess the efficacy and safety of the IL-1 inhibitor Canakinumab in adult and adolescent patients with refractory FMF. Methods Fourteen patients (7 men) with genetically confirmed FMF, fulfilling the Tel Hashomer criteria, aged 38.5 years (median, range 13–70), with median disease duration of 14 years and active disease despite colchicine (n=9) or both colchicine and anakinra (n=5), received Canakinumab 150mg subcutaneously (sc) every 4 (n=7) or 6 (n=2) or 8 weeks (n=5) for a median of 18 months (range 7–53). Canakinumab was given as monotherapy in 8; 6 patients received concomitant treatment with colchicine and/or corticosteroids. Results Eleven out of 14 patients (79%) achieved complete clinical remission (median time 2 months), while normalization of all laboratory parameters associated with inflammation occurred in 92% of patients (median time 3 months). The remaining patients achieved partial responses. Response was maintained until the last visit in all but 4 patients. Reducing the Canakimumab administration interval led to suppression of disease activity in three cases, whereas in another two patients drug administration intervals could be increased without disease exacerbation. The corticosteroid dose was significantly reduced during follow up. The FMF50 score (2) was achieved by 50% and 86% of patients at 1 and 12 months, respectively. Canakinumab was well tolerated; one patient experienced an urinary tract infection and another one a viral gastroenteritis. Conclusions The rapid and sustained response to Canakinumab in the majority of our patients, together with the favorable safety profile, encourages its further use in FMF. References Ter Haar N et al. Ann Rheum Dis. 2013;72(5):678–85. Ozen S et al. Ann Rheum Dis. 2014;73(5):897–901. Disclosure of Interest None declared

Background Interleukin-1 (IL-1) is a major mediator of the inflammatory cascade in Familial Mediterranean Fever (FMF) and an established therapeutic target (1). Objectives To assess the efficacy and safety of the IL-1 inhibitor Canakinumab in adult and adolescent patients with refractory FMF. Methods Fourteen patients (7 men) with genetically confirmed FMF, fulfilling the Tel Hashomer criteria, aged 38.5 years (median, range 13–70), with median disease duration of 14 years and active disease despite colchicine (n=9) or both colchicine and anakinra (n=5), received Canakinumab 150mg subcutaneously (sc) every 4 (n=7) or 6 (n=2) or 8 weeks (n=5) for a median of 18 months (range 7–53). Canakinumab was given as monotherapy in 8; 6 patients received concomitant treatment with colchicine and/or corticosteroids. Results Eleven out of 14 patients (79%) achieved complete clinical remission (median time 2 months), while normalization of all laboratory parameters associated with inflammation occurred in 92% of patients (median time 3 months). The remaining patients achieved partial responses. Response was maintained until the last visit in all but 4 patients. Reducing the Canakimumab administration interval led to suppression of disease activity in three cases, whereas in another two patients drug administration intervals could be increased without disease exacerbation. The corticosteroid dose was significantly reduced during follow up. The FMF50 score (2) was achieved by 50% and 86% of patients at 1 and 12 months, respectively. Canakinumab was well tolerated; one patient experienced an urinary tract infection and another one a viral gastroenteritis. Conclusions The rapid and sustained response to Canakinumab in the majority of our patients, together with the favorable safety profile, encourages its further use in FMF. References Ter Haar N et al. Ann Rheum Dis. 2013;72(5):678–85. Ozen S et al. Ann Rheum Dis. 2014;73(5):897–901. Disclosure of Interest None declared

Methods Twelve patients (7 men) with genetically confirmed FMF, fulfilling the Tel Hashomer criteria, aged 32.5 years (median, range 13-70), with median disease duration of 168 months and active disease refractory to colchicine (n=8) and/or anakinra (n=4), received Canakinumab 150mg subcutaneously every 4 (n=7) or 6 (n=3) or 8 weeks (n=2) for a median of 12 months (range 4-46). Canakinumab was given as monotherapy in 9; 3 patients received concomitant treatment with colchicine and/or corticosteroids. Clinical and laboratory parameters during follow-up were recorded.

OBJECTIVESThe aim of this study was to examine the distribution of lectin-like oxidised LDL receptor-1 (LOX-1) levels in patients with active BD, possible association of LOX-1 with the oxidised LDL (oxLDL), endothelial nitric oxide synthase (eNOS), nitric oxide (NO), endothelin-1 (ET-1) levels, and to characterise the differences between patients with active BD and those with systemic lupus erythematosus( SLE) in terms of these parameters compared with healthy controls.METHODSA total of 30 patients with active BD, 22 patients with SLE as patients controls, and 30 healthy subjects were enrolled in this study.RESULTSSignificantly lower eNOS ve NO levels were observed in patients with BD and SLE compared with healthy controls. oxLDL, LOX-1 ve ET-1 levels were significantly increased in active periods of patients with BD and SLE compared with healthy control. There was no significant difference in oxLDL levels between subjects with BD and SLE. LOX-1 levels were significantly higher in active periods of patients with BD than in SLE , ET-1 levels were significantly lower.CONCLUSIONSEndothelial dysfunction parameters are elevated in patients with BD having active disease. The necessary measures should be considered in terms of risk of atherosclerosis in BD, especially for the early identification of endothelial damage by looking at LOX-1 levels.

Background Autoinflammatory diseases are characterized by recurrent episodes of systemic inflammation with polyarthritis and constitutional symptoms that lead to severe morbidity and disability, as there are no structured therapeutic recommendations for their management. The recognition of the culpable genetic mutations confirms the diagnosis of autoinflammatory syndromes, mainly when the patients do not have pathognomonic clinical characteristics and directs their treatment schedule. Part of the broad spectrum of autoinflammatory syndromes is the periodic fever syndromes, in which inherited or de novo mutations in several genes lead to the increase of IL-1 due to inflammasome activation. Objectives Investigation of efficacy and safety of Canakinumab in patients with cryopyrin-associated periodic syndrome(CAPS) in adults resistant to treatment with non steroidal anti-inflammatory drugs, systemic corticosteroids, disease modifying anti-rheumatic drugs(DMARDs) and biologic modifiers such as TNF blockers and IL-1 inhibitors12. Methods Retrospective research in the medical records of two University Hospitals in Northern Greece indentified three suitable patients. All 3 presented with recurrent episodes of polyarthritis accompanied by non-specific symptoms as fatigue, myalgia, arthralgia, fever and rash for many years, alongside with increased inflammatory serological markers. Furthermore, they showed substantial lack of response in many therapeutic combinations of different disease modifying anti-rheumatic drugs(DMARDs). One of these patients, due to characteristic rash, recurrent conjuctivitis and childhood onset of symptoms was considered having Familial cold autoinflammatory syndrome(FACS) and therefore no genetic testing was pursued. In the other 2 patients due to the clinical suspicion of autoinflammatory syndrome, molecular genetic analysis for the following 4 genes was performed: MEFV, MVK, TNFRSF1A and NALP3. The same silent mutations pA242A/c.726G>A and pR260R/c.780G>A were found in both patients in homozygosity in exon 3 of the NALP3 gene. Moreover in one of them, the polymorphisms pL411L/c.1231C>T and pS434S/c.1302C>T in heterozygosity in exon 3 of the same gene were also found (all these mutations are not registered in INFEVERS database). All patients received Canakinumab (sc) 150mg every 8 weeks for 6 months. All of them showed clinical remission 2 weeks after the first injection, as well as, reduction of the abnormal values of inflammatory serological markers (decrease of ESR:1st45%, 2nd37%, 3rd80% and decrease of CRP:1st40%, 2nd37%, 3rd 60% respectively). There were no relapses or adverse effects. Conclusions The immediate and continuous clinical and laboratory remission of these 3 patients showed the favorable therapeutic effect of this monoclonal antibody against IL-1β in patients with CAPS resistant to treatment. It is known that a response to the administration of an IL-1 inhibitor is considered to be a diagnostic criterion of CAPS. References Lachmann HJ.et al N. Engl J. Med 2009;360(23):2416-2425 Kuemmerle-Deschner JB. et al. Ann. Rheum. Dis.2011;70 (12):209 Disclosure of Interest None Declared

Background About one third of RA patients do not initially respond to treatment with an anti-TNF agent whereas a similar rate demonstrates lack of efficacy over time. Rituximab/Mabthera administration (temporary B-lymphocyte depletion) is one of the therapeutic options for them. Objectives The LAUNCH prospective study aimed at the evaluation of long-term efficacy and safety data following rituximab administration in standard clinical practice Methods 17 Rheumatology sites in Greece enrolled 234 adult patients (63.0±12.4 years, 79.5% women) with severe RA and an inadequate response or non-tolerance to anti-TNF treatment. Rituximab (1gr) was administered IV on days 1 and 14 of each cycle, repeated every 6-12 months, for up to 7 cycles. Of these patients 41.2% and 56.2% had received one, or more, anti-TNF agent(s), respectively. Adverse events, DAS28, and the quality of life evaluation indices (Euroqol) were collected every 2 to 6 months for 5 years according to each site’s standard clinical practice Results During 496 patient/years, 28 adverse events /100 pt-yrs (including9.9 serious adverse events and 7.7 serious infectious per 100pt-yrs, respectively) were observed. Of the total number of adverse events a 46.7% was not related to rituximab. The mean number of adverse events per patient remained stable during repeated treatment cycles. Disease activity at baseline (mean±SD DAS28 of 5.36±1.40) was significantly reduced in cycles 1,2, 3, 4, 5, and 6 by 1.34, 2.12, 2.25, 2,56, 2.42 and 2.79, respectively (p<0.01). Compared to baseline, significant improvement in quality of life was also observed in all cycles (p<0.01) Conclusions Rituximab administration in clinical practice for up to 5 years demonstrated an acceptable safety profile which was maintained over time. Likewise, maintenance and/or improvement of efficacy with repeated treatment cycles in patients with severe RA not responding to anti-TNF were evident Disclosure of Interest L. Settas: None Declared, A. Andrianakos: None Declared, S. Aslanidis: None Declared, P. Boura: None Declared, M. Katsounaros: None Declared, D. Vassilopoulos: None Declared, P. Athanassiou: None Declared, K. Tempos: None Declared, G. Skarantavos: None Declared, C. Antoniadis : None Declared, L. Sakkas: None Declared, A. Andonopoulos: None Declared, V. Galanopoulou: None Declared, F. Solioti: None Declared, K. Boki: None Declared, E. Vritzali Employee of: Roche Hellas SA, P. Sfikakis: None Declared

BACKGROUND:To analyze the pattern of clinical expression and the 5-year disease course in Caucasian patients with late onset of systemic lupus erythematosus (SLE) and to compare the findings with an early onset SLE group.METHODS:Medical records of 551 patients who presented with SLE at hospitals of the region of Thessaloniki between 1989 and 2007 were studied. Patients who developed SLE at or after the age of 50 years were classified as the late onset group, while younger patients served as the early onset group. Data on clinical manifestations and damage accrual at disease onset and at 5 years was obtained and compared between the two groups.RESULTS:In 121 patients, the disease started after the age of 50 years. Elderly patients showed less pronounced female predominance and less often presented with malar rash, nephropathy, fever and lymphadenopathy, while lung involvement, pericarditis and sicca syndrome were more frequent. Damage accrual was similar in both groups. The main causes of damage at 5 years differed, with the elderly exhibiting more cardiovascular damage. They also had a higher incidence of hypertension and osteoporosis at 5 years.CONCLUSIONS:Caucasian SLE patients with late onset of the disease present with different clinical manifestations, suggesting that age affects the expression of SLE. Damage accrual at 5 years is similar in the elderly and the younger patients. However, the causes of this damage and the occurrence of other comorbidities follow a different pattern, possibly reflecting the disease process and the effects of aging.

Background: The aim of this study was to determine the alterations in serum copper (Cu), zinc (Zn) and selenium (Se) levels in Familial Mediterranean Fever (FMF) patients as compared to healthy controls. Methods: This study was conducted on 33 patients with FMF during an attack-free period and 30 healthy volunteers. Serum levels of Cu, Zn and Se were assessed by the atomic absorption spectrophotometry method. Results: Serum Cu and Zn levels were similar between the FMF patient and healthy control groups (p>0.05). However, Se levels in the FMF attacks-free group were significantly higher than in the control groups (p< 0.05). Conclusions: Our study shows that serum trace elements are variable in attack free patients with FMF. Serum Se concentrations may at least in part contribute to the subclinical inflammation in FMF patients during attack-free periods. However, further studies are necessary to support this result.

BACKGROUND AND AIMRheumatoid arthritis (RA) is a chronic polyarthritic syndrome in which actively inflamed joints coexist with others being in remission. Compatible bone scan (BS) reveals joints with increased activity due to degenerative alterations, whilst scanning with human polyclonal immunoglobulin (HIG) is capable to show which of the joints present active inflammation of the synovial membrane. The aim of the study is to investigate the utility of molecular imaging with HIG in patients suffering from RA.PATIENTS AND METHODSForty patients (9 males plus 31 females), suffering from painful polyarthritic syndrome, with a mean age 45.3±7 years and a duration of disease 18.3±4.2 months were enrolled in the study. Twenty-six of the patients were serum positive to RA factor, considered as suffering from RA, whilst fourteen of them were RA factor negatives and they were considered as patients with serum-negative polyarthritis. All patients were submitted to x-rays and ultrasound examination (US) in joints of interest, plus whole body BS with (99m)Tc-MDP and finally scan with (99m)Tc-HIG.RESULTSA total of 1680 joints have been evaluated. In 6 of the patients-two with serum negative RA (252 joints), radionuclide imaging with HIG was within normal limits, despite the fact that in compatible bone scan degenerative alterations have been mentioned in 30 joints. In all these patients disease was evaluated as inactive ("arthrotic changes"). In the remaining 34 patients-12 with serum negative RA (1428 joints), increased accumulation of HIG, concerning serum positive patients, has been mentioned to 163 joints ("arthritic changes"), whilst in the same group, BS revealed degenerative changes to 265 joints. Concerning serum negative patients, the respective results were 64 versus 190 joints. Increased uptake of HIG has been found in 189/226 swollen and painful joints (overall sensitivity according to clinical criteria 83.3%) and in 38 joints without any clinical evidence of inflammation, with clinical active inflammation presented after follow-up to 35 of them, yielding thus specificity at the level of 92%. Matched findings between these two methods have been mentioned to 185 out of 227 joints with an abnormal scan with HIG. Abnormal x-rays and US findings have been mentioned in 67 of the joints.CONCLUSIONSAccording to the above mentioned, BS in RA reveals joints being actively inflamed or not, whilst radionuclide study with HIG is capable to distinguish actively inflamed joints, even in patients with serum negative RA, in a greater extent than anatomical imaging modalities.

Objective. To investigate the possible influence of tumour necrosis factor-alpha (TNF), TNF receptor I (TNFRI) and TNF receptor II (TNFRII) gene polymorphisms on anti-TNF treatment responsiveness, stratified by autoantibody status.Methods. A Greek multi-centre collaboration was established to recruit a cohort of patients (n=100) with active RA treated with anti-TNF drugs. TNF g.-238G>A (rs361525), g.-308G>A (rs1800629), g.-857C>T (rsl 799724), TNFRI c.36A>G (rs4149584) and TNFRII c.676T>G (rs1061622) polymorphisms were genotyped by PCR-RFLP assays. Serum RF and anti-CCP antibody status were determined using commercially available kits. Single-SNP, haplotype and stratification autoantibody status analyses were performed in predicting response to treatment by 6 months, defined as the absolute change in DAS28.Results. 31 patients (31%) were defined as non-responders due to failure to fulfill the DAS28 criteria. 79% and 66% were RF and anti-CCP positive, respectively. None of the genotyped SNPs was alone associated with responsiveness to drug treatment. However, after stratification by autoantibody status, carriage of TNFRII c.676G allele was associated with poorer response to drug treatment in anti-CCP positive patients (p=0.03), after 6 months of anti-TNF therapy.Conclusion. In concordance with previous studies, genetic polymorphisms alone cannot be used to safely predict clinical response to anti-TNF therapy however the combination of genetic factors and autoantibody status warrants further investigation in larger independent cohorts.

The aim of this study was to analyse the prevalence of the most relevant clinical features of the diagnosis of systemic lupus erythematosus (SLE) in a sample of male patients with lupus as well as the incidence of the main causes of morbidity in a 5-year period after the diagnosis. A further aim of this study was to investigate the impact of gender on expression and morbidity of SLE. Data were collected from the medical records of 59 male and 535 female patients with SLE who were diagnosed at the hospitals in the region of Thessaloniki. Several differences in the expression and morbidity of the disease were found in relation to the gender of the patient. Male patients had a higher prevalence of thromboses, nephropathy, strokes, gastrointestinal tract symptoms and antiphospholipid syndrome when compared with female patients, but tended to present less often with arthralgia, hair loss, Raynaud’s phenomenon and photosensitivity as the initial clinical manifestations. During the 5-year follow-up, positive associations have been found between male gender and the incidence of tendonitis, myositis, nephropathy and infections, particularly of the respiratory tract. In conclusion, this study has provided information regarding the features of clinical expression and morbidity in male patients, and has shown that gender is a possible factor that can influence the clinical expression of SLE.