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Objective: Non-radiographic axial spondyloarthritis (SpA) is characterised by a lack of definitive radiographic sacroiliitis and is considered an early stage of ankylosing spondylitis. The objective of this study was to develop candidate classification criteria for axial SpA that include patients with but also without radiographic sacroiliitis. Methods: Seventy-one patients with possible axial SpA, most of whom were lacking definite radiographic sacroiliitis, were reviewed as "paper patients" by 20 experts from the Assessment of SpondyloArthritis international Society (ASAS). Unequivocally classifiable patients were identified based on the aggregate expert opinion in conjunction with the expert-reported level of certainty of their judgement. Draft criteria for axial SpA were formulated and tested using classifiable patients. Results: Active sacroiliitis on magnetic resonance imaging (MRI) (odds ratio 45, 95% CI 5.3 to 383; p

Objectives: To present and analyse the literature sources regarding the management of Behçet disease (BD) identified during the systematic literature research, which formed the basis for the European League Against Rheumatism (EULAR) evidence-based recommendations for the management of BD. Methods: Problem areas and related keywords regarding the management of BD were determined by the multidisciplinary expert committee commissioned by EULAR for developing the recommendations. A systematic literature research was performed using MedLine and Cochrane Library resources through to December 2006. Meta-analyses, systematic reviews, randomised controlled trials (RCTs), open studies, observational studies, case control studies and case series' involving ≥5 patients were included. For each intervention the effect size and number needed to treat were calculated for efficacy. Odds ratios and numbers needed to harm were calculated for safety issues of different treatment modalities where possible. Results: The literature research yielded 137 articles that met the inclusion criteria; 20 of these were RCTs. There was good evidence supporting the use of azathioprine and ciclosporin A in eye involvement and interferon (IFN) in mucocutaneous involvement. There were no RCTs with IFNa or tumour necrosis factor (TNF)a antagonists in eye involvement. Similarly controlled data for the management of vascular, gastrointestinal and neurological involvement is lacking. Conclusion: Properly designed, controlled studies (new and confirmatory) are still needed to guide us in managing BD.

Objective: Inflammatory back pain (IBP) is an important clinical symptom in patients with axial spondyloarthritis (SpA), and relevant for classification and diagnosis. In the present report, a new approach for the development of IBP classification criteria is discussed. Methods: Rheumatologists (n = 13) who are experts in SpA took part in a 2-day international workshop to investigate 20 patients with back pain and possible SpA. Each expert documented the presence/absence of clinical parameters typical for IBP, and judged whether IBP was considered present or absent based on the received information. This expert judgement was used as the dependent variable in a logistic regression analysis in order to identify those individual IBP parameters that contributed best to a diagnosis of IBP. The new set of IBP criteria was validated in a separate cohort of patients (n = 648). Results: Five parameters best explained IBP according to the experts. These were: (1) improvement with exercise (odds ratio (OR) 23.1); (2) pain at night (OR 20.4); (3) insidious onset (OR 12.7); (4) age at onset

Patients with ankylosing spondylitis (AS) may develop cardiovascular manifestations ranging from asymptomatic forms to life threatening conditions. The most important cardiovascular manifestation of AS is aortitis, which frequently involves the aortic root and the ascending aorta leading to valvular insufficiency. The extension of the subaortic fibrotic process into the interventricular septum may cause conduction abnormalities that represent the second common cardiovascular manifestations occurring in AS patients. More rarely, an involvement of coronary arteries and of thoracic and abdominal aorta could be present. Rheumatologists managing AS patients should carefully consider, both in late and in early phases of the disease, the occurrence of an aortic involvement in order to promptly administer adequate treatment.

BACKGROUND: Ten ASAS/EULAR recommendations for the management of ankylosing spondylitis (AS) were published in 2006. OBJECTIVES: (a) To disseminate and (b) to evaluate conceptual agreement with, and (c) application of, these recommendations as well as (d) potential barriers to the application. METHODS: A questionnaire was sent to rheumatologists in 10 countries. It included (a) the text of the recommendations; (b) rheumatologists' demographic variables; (c) two numerical rating scales from 1 to 10 for each recommendation: conceptual agreement with, and application of, the recommendation (10 indicates maximal agreement and maximal application); and (d) a list of potential barriers to the application of the recommendation. Statistical analysis included descriptive and multivariate analyses. RESULTS: 7206 questionnaires were sent out; 1507 (21%) were returned. Of the 1507 answering rheumatologists, 62% were men, mean (SD) age 49 (9) years, and 34% had an academic position. Conceptual agreement with the recommendations was high (mean (SD) for all recommendations 8.9 (0.9)). Self-reported application was also high (8.2 (1.0)). The difference between agreement and application varied across recommendations and countries. The most pronounced discrepancies were reported for use of anti-tumour necrosis factor drugs in a few countries, with funding as the most commonly reported barrier for application of this recommendation. CONCLUSION: This large project has helped the dissemination of the ASAS/EULAR recommendations for the management of AS and shows that conceptual agreement with the recommendations is very high. The project also highlights inequalities in access to healthcare for European citizens with AS. researcherid-numbers: Pavelka, Karel/E-6578-2017 orcid-numbers: Pavelka, Karel/0000-0003-1952-8422 Collantes Estevez, Eduardo/0000-0002-7647-6289 unique-id: ISI:000255774400009 ispartof: ANNALS OF THE RHEUMATIC DISEASES vol:67 issue:6 pages:782-788 ispartof: location:England status: published

To evaluate the frequency and duration of clinical remission in patients with PsA.All consecutive new outpatients with peripheral PsA requiring second-line drugs and RA observed between January 2000 and December 2005 were included in a prospective, case-control study. Primary end point was to assess the frequency of remission in peripheral PsA compared with RA. Secondary end points were to compare the duration of clinical remission during treatment and after therapy interruption, ACR 20, 50, 70 response rates and to detect any remission predictor at diagnosis. Treatment regimen was standardized in both groups. From January 2003 to December 2005, therapy was suspended in PsA patients and controls if achieving remission.One or more episodes of remission occurred in 57/236 (24.1%) PsA patients and in 20/268 (7.5%) controls (P0.001). The mean duration of remission was of 13 +/- 9.4 months in PsA patients and 4 +/- 3.7 in controls (P0.001). Remission episodes were more frequent in PsA patients treated with anti-TNF compared with those receiving traditional DMARDs (P0.001), with no differences regarding the duration. After therapy interruption, the remission duration was 12 +/- 2.4 months in PsA and 3 +/- 1.5 in RA (P0.001). No remission predictor at diagnosis resulted by multivariate analysis.Remission is possible in up to 24% of patients with peripheral PsA. It is significantly more frequent, but not longer, in patients receiving anti-TNF drugs compared with those treated with traditional DMARDs. Patients remain in remission for a long period after therapy interruption, thus suggesting an intermittent therapeutic strategy.

Objective. Recommendations and/or guidelines represent a popular way of integrating evidence-based medicine into clinical practice. The 3E Initiatives is a multi-national effort to develop recommendations for the management of rheumatic diseases, which involves a large number of experts combined with practising rheumatologists addressing specific questions relevant to clinical practice. Methods. Ten countries participated in three rounds of discussions and votes concerning the management of AS. A set of nine questions was formulated in the domains of diagnosis, monitoring and treatment, after a Delphi procedure. A literature search in MedLine was conducted. Predefined outcome parameters for the domains of diagnosis, monitoring and treatment were assessed. The evidence to support each proposition was evaluated and scored. After discussion and votes, the final recommendations were presented using brief statements by each national group, following which the final international recommendations were formulated. Results. A total of 2699 papers were found and 467 were selected for analysis. Twelve key recommendations were developed: three in the domain of diagnosis addressing general diagnostic considerations, early AS diagnosis and general practitioners' referral recommendations; three concerning monitoring of AS disease activity, severity and prognosis; six concerning pharmacological treatment (except biologics): non-steroidal anti-inflammatory drugs/COX-II inhibitors, bisphosphonates and treatment of enthesitis. The compiled agreement among experts ranged from 72% to 93%. Conclusion. Recommendations for the management of AS were developed using an evidence-based approach followed by expert/physician consensus with high level of agreement. Involvement of a larger and more representative group of rheumatologists may improve their dissemination and implementation in daily clinical practice.

In this article we describe a gouty caucasian male patient who had high levels of serum uric acid since 1970s. Serious adverse reactions to allopurinol discouraged its administration. We prescribed cyproterone acetate (CA) because of severe prostatic hypertrophy, associated with a suspect small cancer. The administration of this drug obtained persistent normalization of serum uric acid levels. Our observation, that needs to be confirmed in other cases, could suggest a possible new treatment for gout in male patients with prostatic disorders.

Objectives: To develop evidence-based European League Against Rheumatism (EULAR) recommendations for the management of Behcet disease (BD) supplemented where necessary by expert opinion. Methods: The multidisciplinary expert committee, a task force of the EULAR Standing Committee for Clinical Affairs (ESCCA), consisted of nine rheumatologists (one who was also a clinical epidemiologist and one also a Rehabilitation Medicine doctor), three ophthalmologists, one internist, one dermatologist and one neurologist, representing six European countries plus Tunisia and Korea. A patient representative was also present. Problem areas and related keywords for systematic literature research were identified. Systematic literature research was performed using Medline and the Cochrane Library databases from 1966 through to December 2006. A total of 40 initial statements were generated based on the systematic literature research. These yielded the final recommendations developed from two blind Delphi rounds of voting. Results: Nine recommendations were developed for the management of different aspects of BD. The strength of each recommendation was determined by the level of evidence and the experts' opinions. The level of agreement for each recommendation was determined using a visual analogue scale for the whole committee and for each individual aspect by the subgroups, who consider themselves experts in that field of BD. There was excellent concordance between the level of agreement of the whole group and the "experts in the field". Conclusion: Recommendations related to the eye, skin-mucosa disease and arthritis are mainly evidence based, but recommendations on vascular disease, neurological and gastrointestinal involvement are based largely on expert opinion and uncontrolled evidence from open trials and observational studies. The need for further properly designed controlled clinical trials is apparent.

The case of a 59-year-old man with definite HLA B27 negative ankylosing spondylitis (AS) and Paget's disease involving the skull, the right side of the pelvis and the proximal femur is reported. Fifteen cases with this coexistence have previously been described. Some of these, however, are patients with Paget's disease mimicking AS. It is emphasized again that attention must be paid to diagnosing AS in patients suffering from Paget's disease.

Objective. To investigate potential associations between the � 463G/A myeloperoxidase (MPO) promoter polymorphism and susceptibility to, and clinical expression of, Behcet's disease (BD). Methods. One hundred and seventy-five Italian patients who satisfied the International Study Group criteria for BD and 235 healthy age- and sex-matched blood donors were genotyped for the �463G/A promoter polymorphism of the MPO gene by molecular methods. The patients were subgrouped according to the presence or absence of clinical manifestations. Results. The distribution of allele and genotype frequencies of the MPO �463A/G polymorphism did not differ significantly between the BD patients and the healthy controls. Carriers of the �463A allele (A/A or A/G) (odds ratio (OR) 0.7, 95% confidence interval (CI) 0.5-1.1) and homozygosity for A allele (OR 0.3, 95% CI 0.1-1.3) were less frequent among BD patients than among the controls, but the difference was not statistically significant. No significant associations were found when BD patients with and those without clinical manifestations were compared. Conclusion. Our data suggest that the �463G/A promoter polymorphism of the MPO gene is not associated with susceptibility to, and clinical expression of, BD in Italian patients.

Evolution in a single environment is expected to erode genetic variability, thereby precluding adaptation to novel environments. To test this, a large population of spider mites kept on cucumber for approximately 300 generations was used to establish populations on novel host plants (tomato or pepper), and changes in traits associated to adaptation were measured after 15 generations. Using a half-sib design, we investigated whether trait changes were related to genetic variation in the base population. Juvenile survival and fecundity exhibited genetic variation and increased in experimental populations on novel hosts. Conversely, no variation was detected for host choice and developmental time and these traits did not evolve. Longevity remained unchanged on novel hosts despite the presence of genetic variation, suggesting weak selection for this trait. Hence, patterns of evolutionary changes generally matched those of genetic variation, and changes in some traits were not hindered by long-term evolution in a constant environment.

Background Therapeutic approach of Behcet9s disease (BD) depends on the clinical presentation and organ involved. The conventional immunosuppressive therapy is still the first approach to the treatment of disease and is aimed to improve the quality of life and prevent irreversible organ damage. However a variable percentage of patients with BD do not respond to traditional immunosuppressive agents or withdraw from treatment due to inefficacy or adverse events. Objectives To identify the clinical factors predicting a good clinical response to Infliximab (IFX) therapy after 12 months in patients BD refractory to conventional therapy. Methods Patients receiving IFX (5 mg/kg intravenously at weeks 0, 2, 6, and every 6-8 weeks subsequently) for BD unresponsive to conventional therapy (corticosteroids plus at least two different immunosuppressive drugs) were prospectively included. Clinical response to IFX therapy was based on the expert opinion and was graded as follows: remission, response, no response and worsening. Remission was defined as the complete disappearance of symptoms and signs of inflammation and response as at least 50% of improvement. Univariate and multivariate analyses were performed to identify factors associated with IFX good response (remission, response) at 12 months. Logistic regression analysis was performed to analyse which of the following measures at the start of treatment were associated with a good response: sex; age; disease duration; HLA-B51 status; indication for IFX treatment including uveitis, CNS involvement, severe mucocutaneous manifestations or others including arthritis, intestinal and vascular involvement; concomitant drugs including steroids (Ster), colchicine (Col), azathioprine (AZA) or cyclosporine (CSA). Results The study included 73 BD patients (47 M/26 F; mean age 33.6±10.7 yrs; mean disease duration 12.3±9.3 yrs; 71.2% HLA-B51 positive). Indication for IFX treatment were uveitis in 38 patients, severe mucocutaneous manifestations in 13, CNS involvement in 16, intestinal involvement in 2, arthritis in 2, vascular involvement in the remaining 2.At 12 months, 56 patients (76.7%) had a good response to IFX, 4 (5.5%) patients had stopped for adverse events, and 13 (17.8%) had stopped for primary or secondary inefficacy. In the univariate analysis concomitant use of AZA (95.8% vs 67.3%, p Conclusions This study has, for the first time, shown that concomitant use of AZA at the start of IFX treatment is a factor that seems to influence the probability of achieving a good therapeutic response in patients with refractory BD. Further support from larger studies is necessary so as to optimize the management of BD patients treated with IFX. Disclosure of Interest None declared

Background Behcet9s disease (BD) is a multisystemic disorder of unknow aetiology characterized by recurrent oral and genital ulcers and inflammatory eye involvement. Other manifestations include skin lesions and musculoskeletal, neurological, gastrointestinal and vascular symptoms. Although BD is diagnosed during the fertile age there is a little knowledge about the influence of pregnancy and puerperium on the disease activity and outcome. Objectives The purpose of this study was to analyse the relationship between pregnancy and BD. Methods We studied women with BD, meeting the International Study Group (ISG) criteria, seen in our department between October 1998 and December 2014. These patients were entered in a special register and were followed prospectively in order to investigate the relationship between BD and pregnancy. Data on previous pregnancies were retrospectively collected by using a standardized form. We collect data on maternal age, disease onset, infertility, disease manifestations (oral and genital ulcers, anterior and posterior uveitis, arthritis and cutaneous, neurological, gastrointestinal and vascular manifestations), therapeutic changes, maternal and neonatal complications in the 3 months before and 6 months after pregnancy. Results We studied 142 pregnancies in 86 women (mean age at disease onset 24.1±13.1 yrs, mean age at first visit 38.3±11.8 yrs, mean disease duration 14.3±11.0 yrs; 65% HLA B51 positive). Twenty-five patients had no pregnancies (a diagnosis of infertility was made in 5 of them), 31 had pregnancies after the disease onset and 30 had pregnancies before the first manifestation occurred. Only 1 patient had active disease at the time of conception. A disease flare occurred in 14.1% (20/142) and 13.4% (19/142) during and after pregnancy, respectively. Miscarragies, premature labours, and pre-eclampsia or eclampsia occurred in 17.6% (25/142), 12.7% (18/142) and 4.2% (6/142) of pregnancies of women with BD, respectively. No cases of stillbirth, neonatal death, congenital abnormalities, and neonatal BD were reported. Conclusions Our study shows that pregnancy does not affect BD activity. Furthermore, our data suggest that BD does not negatively influence pregnancy, labour and neonatal outcome. Disclosure of Interest None declared

Background The genetics of Behcet9s disease: a novel variation of ERAP1 gene in an Italian cohort Objectives The objective of the present study was to evaluate the association of ERAP1 gene with BD in an Italian cohort by investigating not only tagSNPs but all gene regions searching for additional variations probably related to the BD phenotype. Methods Genomic DNA was isolated from 21 BD patients whole blood by using a commercial kit. The patient cohort was recruited from the Rheumatology Departments of Lucania (San Carlo Hospital - Potenza and Madonna delle Grazie Hospital - Matera, Italy) and was formed by 11 males and 10 females with a mean age of 45 years. The purity of DNA was determined by means of NanoDrop spectrophotometer. Specific PCR primers was designed on the basis of ERAP1 NCBI RefSeq (NG_027839.1) in order to cover all 21 gene exons and used for in vitro amplification. PCR products were directly sequenced and bioinformatically analysed. The functional impact of the gene variants was evaluated by querying Polyphen 2 software. Results Mutation analysis of the entire ERAP1 gene underlined a novel variation within exon 7. It was found in 3/21 patients (14%) and consists in the substitution p.Phe360Cys (NG_027839.1:g.25637T>G; NP_057526.3:p.Phe360Cys). Interestingly the novel mutation was identified in association with three known tagSNPs (p.Met349val, p.Lys528Arg and p.Gln730Glu) and was predicted to have damaging effects on protein (maximum score of pathogenicity). Conclusions ERAP1 was recently considered a BD susceptibility gene. Five tagSNPs was previously reported to be related to BD phenotype. We firstly analysed an Italian BD patient cohort. In addition, we firstly investigated the entire ERAP1 gene, because searching only for common variants by GWAS allows to identify a fraction of the entire genetic burden of disease. In this study, three known gene variations were found in three patients belonging to our cohort, in association with our novel variation. This aspect suggests a significant role of the novel substitution in the pathogenesis of BD. The identification of novel functional variants of entire ERAP1 gene may lead to better stratification of BD patients by providing a diagnostic tool and a potential therapeutic target. References Gul A. Current Opinion in Rheumatology 2014; 26:56-63. Conde-Jaldon M, Montes-Cano MA, Garcia-Lozano JR, Ortiz-Fernandez L, Ortego-Centeno N, Gonzalez-Leόn R, Espinosa G, Grana-Gil G, Sanchez-Bursόn J, Gonzalez-Gay MA, Barnosi-Marin AC, Solans R, Fanlo P, Carballeira MR, Camps T, Castaneda S, Martin J, Gonzalez-Escribano MA. 2014. PlosOne; 9: 1-6. Kirino Y1, Bertsias G, Ishigatsubo Y, Mizuki N, Tugal-Tutkun I, Seyahi E, Ozyazgan Y, Sacli FS, Erer B, Inoko H, Emrence Z, Cakar A, Abaci N, Ustek D, Satorius C, Ueda A, Takeno M, Kim Y, Wood GM, Ombrello MJ, Meguro A, Gul A, Remmers EF, Kastner DL. Nat Genet. 2013; 45(2): 202-207. Carmona FD1, Martin J, Gonzalez-Gay MA. Curr Opin Rheumatol. 2015; 27(1): 10-17. Reeves E, Elliott T, James E, Edwards CJ. 2014; 60(2-3): 257-69. O9Rielly DD, Rahman P. Best Pract Res Clin Rheumatol. 2014; 28(5): 673-685. Disclosure of Interest None declared

Background The spondyloarthritis (SpA) complex also includes forms that do not meet criteria for the definite forms and are called undifferentiated SpA (uSpA). Recently the Assessment in SpondyloArthtritis international Society (ASAS) suggested criteria for axial and peripheral SpA that have substituted the Amor and the European Sondylarthropathy Study Group (ESSG) criteria for all forms of SpA. In the ASAS criteria for axial disease the entry criterion is chronic low back pain in a patient with an age less than 45 years. Only 15% of the patients included in the study for the ASAS criteria for peripheral disease were older than 45 years. Objectives The objective of our study was to evaluate the sensitivity of the ASAS criteria for axial and peripheral SpA in a cohort of consecutive patients with HLA-B27 positive uSpA with onset of disease after the age of 45 (late onset uSpA) consecutively recruited in a 12-year period in comparison with a cohort of consecutive patients with HLA-B27 positive uSpA with onset before age 45 (ordinary onset uSpA) recruited in the same period. Methods Patients HLA-B27 positive with at least one clinical manifestation of SpA and not meeting the New York criteria for ankylosing spondylitis (AS) and with a negative personal history for psoriasis, inflammatory bowel disease and preceding infection seen since January 2001 were entered in a special register and were followed prospectively. Each patient was examined at 6-month interval even if asymptomatic. Results During the 12-year recruitment period, 93 patients (35 M, 58 F; age 58.4±9.8) with late onset uSpA were seen. The first 93 consecutive patients (54 M, 39 F; age 29.6±8.6) with ordinary onset uSpA seen in the same period were evaluated for comparison. Compared to the 93 patients with ordinary onset uSpA, the 93 patients with late onset uSpA were more frequently females (62.4% vs. 41.9%, p Conclusions The ASAS classification criteria for axial and peripheral SpA showed a similar high sensitivity in patients with ordinary and late onset uSpA. In addition, this study confirms that some clinical and laboratory features of SpA may differ with the age at onset of the disease. Disclosure of Interest None declared

Background One of the most important outcomes of treatment in SpA is the retention rate of therapy in the long term. Many genetic studies in chronic polyarthritis reported a correlation between response to anti-TNF therapy and several single nucleotide polymorphisms (SNPs), but the usefulness of this finding when related to the clinical outcome is ill defined. Objectives The primary endpoint of this study was to establish a possible correlation between several genetic polymorphisms, including ones never investigated before in SpA, and the retention rate of the first TNFa blocking agent, by comparing patients needing to switch the first anti-TNFa agent (Sw) to patients not needing to switch (NSw). To distinguish these two groups, a follow-up of at least one year was required. Methods 187 consecutive SpA patients (124 males and 63 females; mean age 52±30 years) were studied. All the patients had been followed up for at least 12 months after the introduction of the first anti-TNFα agent. The mean disease duration was 30±28 years (range 2-58 years). Seventy-four (39.6%) patients were diagnosed as affected by psoriatic arthritis (PsA), 66 (35.3%) by ankylosing spondylitis (AS) and 47 (25.1%) by undifferentiated spondyloarthritis (uSpA). Six SNPs were analysed: TNFα -308A>G (rs1800629), TNFR2 196M>R, IL-6 -174G>C (rs1800795), IL-6Ra, FCGR3A 158V>F (rs396991) and TGF-beta 869T>C (rs 19822073). The chi-squared or Fisher9s exact test and Mann–Whitney9s U-test were used. Results The TNFα and the IL-6 promoter polymorphisms were significantly associated with the NSw phenotype in SpA. In particular, the TNFα -308 AA/AG genotypes were found in 24.4% NSw vs 10.9% Sw patients (p=0.03) and the GG homozygosis of the IL-6 promoter polymorphism in 49.6% NSw vs 34.4% Sw patients (p=0.047). By multivariate analysis, the TNFα -308A allele as well as the presence of the IL-6 -174GG homozygosis resulted as independent biomarkers predicting survival of the first anti-TNFα therapy in SpA (p=0.007, OR 4.4, 95%CI 1.5-13.1, and p=0.035, OR 2.1, 95%CI 1.1-4.4, respectively). Also the male gender (p=0.001, OR 3.4, 95%CI 1.6-7.1) resulted an independent variable associated with the NSw phenotype. The other SNPs, located in the genes encoding TNFR2, IL-6Ra, TGFb and FCGR3A, did not show any significant association. No association was found between the BASDAI or DAS28 response at month +6 (for axial or peripheral involvement, respectively) and all the investigated polymorphisms. The exposure time to the anti-TNFα therapies was significantly higher in NSw in comparison with Sw (NSw: 58±25.9 months vs Sw: 30.8±20.2 months, p Conclusions The TNFα -308 and the IL-6 -174 promoter polymorphisms appear significantly associated with a higher efficacy of anti-TNFa therapy in SpA, expressed by the retention rate of the first anti-TNFα agent. This confirms the importance of the genetic background to optimize the management of the biologic agents in SpA. Disclosure of Interest None declared

Diffuse idiopathic skeletal hyperostosis (DISH) and ankylosing spondylitis (AS) share the involvement of the axial skeleton and the peripheral entheses. Both diseases produce bone proliferations in the later phases of their course. Although the aspect of these bone proliferations is dissimilar, confusion of radiologic differential diagnosis between the two diseases exists mostly as a consequence of a lack of awareness of their characteristic clinical and radiographic features. The radiologic spinal findings are so different that it is possible to recognize the changes due to each disease even in patients in whom both diseases occur. On radiographs DISH is characterized by “flowing mantles” of ossifications occurring in the anterior longitudinal ligament and to a lesser extent in the paravertebral connective tissue and the peripheral part of the annulus fibrosus. Frequently, on lateral view radiolucency is noted between the new bone and the vertebral body. DISH may also involve the sacroiliac joints. The ossification of the joint capsule on the anterior surface of the joint can resemble the fusion of the sacroiliac joints on anteroposterior pelvis x-rays that may mistakenly be interpreted as post-inflammatory ankylosis of the joint (grade 4 sacroiliitis). Computed tomography (CT) can be helpful in these cases by demonstrating the normal aspect of the joint space and bony margins together with the presence of the anterior capsular ossification. On x-rays AS is characterized by the bone damage caused by sacroiliitis and by inflammation at the discovertebral junction at the attachment of the annulus fibrosus. The consequent close subchondral osteitis is radiologically characterized by a destructive vertebral lesion and sclerosis confined to the anterior corners of the vertebral bodies (“Romanus lesion”). The healing of these lesions and the adjacent periosteal reaction results in “squaring” of the vertebral bodies on lateral view of the spine. Simultaneously, the healing process proceeds in the periphery of the annulus fibrosus resulting in the formation of syndesmophytes that are vertical bony bridges joining adjacent vertebral bodies anteriorly and laterally and ultimately forming a “bamboo-spine”. The confusion between DISH and AS may also extend to the clinical field because patients with DISH can occasionally have severe limitation of spinal mobility, along with postural abnormalities that resemble long-standing advanced AS. Disclosure of Interest None declared

The legume genus Medicago interacts with soil bacteria commonly referred to as rhizobia, in a nitrogen fixing symbiosis. We analysed the diversity of symbiotic association specificity among the two organisms, and its evolution in the plant genus. Nitrogen fixation tests and molecular phylogenetic reconstructions revealed that the genus Medicago includes more symbiotic specificity groups than previously suggested and that plant specificity is highly unstable and has repeatedly switched along the diversification of this genus. A phylogenetic analysis including geographical data shows that bacterial geographical diversity distribution has a strong influence on the geographic distribution of plant species and their ability to colonize new areas. Multiple other modifications of specificity occurred along the diversification of the genus, presumably due to selection for specialization to a single bacterial biovar. Codivergence between plants and bacteria may also have taken place.

We present a study of the genetic diversity and structure of a tropical tree in an insular system. Santalum austrocaledonicum is endemic to the archipelago of New Caledonia and is exploited for oil extraction from heartwood. A total of 431 individuals over 17 populations were analysed for eight polymorphic microsatellite loci. The number of alleles per locus ranged from 3 to 33 and the observed heterozygosity per population ranged from 0.01 in Mare to 0.74 in Ile des Pins. The genetic diversity was lowest in the most recent islands, the Loyautes, and highest in the oldest island, Grande Terre, as well as the nearby small Ile des Pins. Significant departures from panmixia were observed for some loci-population combinations (per population FIS = 0-0.03 on Grande-Terre and Ile des Pins, and 0-0.67 on Loyautes). A strong genetic differentiation among all islands was observed (FST = 0.22), and the amount of differentiation increased with geographic distance in Iles Loyaute and in Grande Terre. At both population and island levels, island age and isolation seem to be the main factors influencing the amount of genetic diversity. In particular, populations from recent islands had large average FIS that could not be entirely explained by null alleles or a Wahlund effect. This result suggests that, at least in some populations, selfing occurred extensively. Conclusively, our results indicate a strong influence of insularity on the genetic diversity and structure of Santalum austrocaledonicum.

L’artrite psoriasica (AP) e sempre stata considerata una malattia piu lieve dell’artrite reumatoide (AR) e ad evoluzione non invalidante. Studi recenti hanno invece dimostrato che circa il 40% dei pazienti ha una malattia erosiva con sviluppo di deformita (1, 2). Similmente all’AR, una terapia aggressiva con uno o piu farmaci di fondo nelle fasi iniziali potrebbe ostacolare la progressione del danno articolare. Inoltre, diversamente dall’AR, la AP puo interessare anche lo scheletro assiale dove puo dare limitazione funzionale e deformita simili a quelle della spondilite anchilosante (SA). Il trattamento iniziale dell’AP prevede l’uso dei farmaci anti-infiammatori nonsteroidei (FANS) e delle infiltrazioni locali di steroidi. I farmaci di fondo sono riservati alle forme resistenti e destruenti. Methotrexate (MTX), sulfasalazina (SSZ) e ciclosporina (CsA) sono i farmaci di fondo piu frequentemente utilizzati, anche se non vi sono studi controllati che abbiano dimostrato la loro capacita di bloccare l’evoluzione erosiva dell’artrite periferica (3-8). Per quanto riguarda l’interessamento assiale tali farmaci non sono in grado di controllare i sintomi quali dolore e rigidita mattutina (5). Recentemente una nuova classe di farmaci, in grado di bloccare il tumor necrosis factor TNF (anti- TNF), si e dimostrata efficace nel rallentare l’evolutivita erosiva nella AR e nel controllare i sintomi della SA (9, 10)...

Hereditary angioedema (HAE) is an autosomal dominant disease that causes recurrent attacks of non-pitting edema of soft tissues, without pruritus. This disorder can also affect internal organs. The cause of HAE consists in quantitative or qualitative defective production of C1 inhibitor (C1-INH). Many autoimmune diseases such as systemic lupus erythematosus (SLE) (or SLE-like syndromes), Sjogren’s syndrome, scleroderma, thyroiditis, glomerulonephritis, and inflammatory bowel disease have been described in patients suffering from HAE. A concomitance with pure arthritis was previously reported only in two adult patients. Here, we describe for the first time the association between HAE and a non-rheumatoid erosive oligoarthritis involving hips and wrists.

Tumour necrosis factor α (TNFα) antagonists have been associated with reactivation of tuberculosis and development of other opportunistic infections.1–4 We have recently observed a case of septic arthritis caused by Moraxella (Branhamella) catarrhalis in a patient treated with infliximab that we describe briefly here. The patient, a 45 year old man, was referred to us in September 1999 for evaluation of an eight year history of recurrent episodes of arthritis of the knees and elbows and of recurrent episodes of inflammatory swelling with pitting oedema over the dorsum of the hand. One year before the onset of arthritis he had undergone replacement surgery for an insufficient aortic valve. Physical examination disclosed swelling, effusion, and tenderness in the right knee, together with tenosynovitis of the left tibialis posterior and flexor digitorum …

Reactive Arthritis (ReA) is an aseptic synovitis developing after a primary infection distant from the joint, mainly localized in the gastrointestinal (Enteroarthritis) or genitourinary tract (Uroarthritis). Because of either the asymmetric joint involvement, the possibility of involvement of the spine and enthesis, and the HLA-B27 association ReA is considered one of the spondylarthropathies. Recently, bacterial components or viable bacteria were found in joints during ReA. For this reason, the limits between ReA itself and infectious arthritis are now less definite. Generally accepted diagnostic and classification criteria are still lacking but the improvement in techniques for detection of bacteria increase the possibility to identify the triggering agents. Several studies have examined the role of antimicrobial drugs in ameliorating the natural course of ReA, with some positive results for Uroarthritis only. However, more conventional treatments based on NSAIDs, Sulfasalazine and steroids are effective in many cases.

Background Enthesitis in Spondylarthropathy (SpA) is underestimated and often misdiagnosed. A recent paper suggests that tenderness at entheseal sites by itself is not useful in distinguishing between psoriatic arthritis (PsA) and fibromyalgia (FM) 1 Objectives To evaluate the prevalence of clinical and ultrasonographic (US) signs of enthesitis in PsA patients compared to patients with psoriasis (Ps) without clinical signs of PsA or FM patients. Methods Study group: 243 consecutive patients (age >18 and ≤65 years), 140 with a diagnosis of PsA (CASPAR criteria), 51 with Ps, and 51 with FM were involved. Rheumatologic assessment: Leeds Enthesitis Index (LEI) and Maastricht Ankylosing Spondylitis Enthesitis Score (MASES). US B mode (Gray scale) and PDUS (Power Doppler) mode assessments were performed bilaterally on common extensor tendon insertion on the lateral epicondyle of the humerus, quadriceps tendon, patellar tendon, medial collateral ligament, and Achilles tendon and plantar fascia insertions on the calcaneus. In US B mode assessment the following abnormalities were recorded: entheseal thickening, entheseal hypoechogenicity, peritendon hypoechogenicity, bony erosions, enthesophytes, and bursae. Vascularisation by PDUS was evaluated at the following areas: cortical bone insertion, body of the tendon, bursa, junction between tendon and enthesis (pre-insertional area). Vascularization was quantified according to the number of vessels (0 =none; 1 =1-3; 2 =4-5; 3 ≥5). Results Mean age was 48.6±10.7 years. In PsA 50% of patients were females, in psoriasis 54.9%, in FM 92.2%. Height, weight and body mass index (BMI) were significantly lower in FM than in PsA. Clinical evidence of enthesopathy was present in 66.4% of PsA, 58.8% of Ps and 92.2% of FM patients. Clinical evidence of enthesopathy in all the evaluated entheses was present in 23.1% of PsA, 18.1% of Ps and 45.6% of FM patients. Entheseal abnormalities by US were present in 92.1% of PsA, 92.2% of Ps and 74.5% of FM patients. US abnormalities in all the evaluated entheses were present in 53.7% of PsA, 41.2% of Ps and 27.4% of FM patients. Enthesis vascularisation at PDUS in at least one area was observed in 59.3% of PsA, 47.16% of Ps and 35.3% of FM patients. Enthesis vascularisation in all the evaluated areas was present in 19.2% of PsA, 12.7% of Ps and 7.8% of FM patients. Conclusions Clinical evidence may overestimate active enthesitis in PsA, Ps, and FM. US and PDUS were more frequently positive in patients with PsA and Ps than in those with FM. Therefore, these examinations can be useful in differentiating pain due to enthesitis from the entheseal pain due to FM. References Marchesoni J Rheumatol 2012; 39 (4), 849-855. Acknowledgements The ULISSE study was sponsored by AbbVie.The design and study conduct support for this study was provided by AbbVie. AbbVie participated in the interpretation of data, review, and approval of the publication. Disclosure of Interest C. Salvarani Grant/research support: AbbVie, Consultant for: AbbVie, W. Grassi Grant/research support: AbbVie, Consultant for: AbbVie, A. Spadaro Grant/research support: AbbVie, Consultant for: AbbVie, R. D. Grembiale Grant/research support: AbbVie, M. Govoni Grant/research support: AbbVie, Consultant for: AbbVie, R. Ramonda Grant/research support: AbbVie, Consultant for: AbbVie, A. Marchesoni Grant/research support: AbbVie, Consultant for: AbbVie, R. Scarpa Grant/research support: AbbVie, Consultant for: AbbVie, S. De Vita Grant/research support: AbbVie, V. Saragaglia Employee of: AbbVie, R. Merolla Employee of: AbbVie, U. di Luzio Paparatti Employee of: AbbVie, I. Olivieri Consultant for: AbbVie DOI 10.1136/annrheumdis-2014-eular.2657

In order to evaluate the relationships between DISH and vertebral osteochondrosis (degenerative disc disease), the radiographs of the spine of 69 DISH patients were compared to those of 68 controls.Radiographs of 69 patients affected by DISH according to Resnick's criteria and of 68 control subjects affected by diseases other than DISH, were evaluated in order to determine the prevalence of vertebral osteochondrosis, diagnosed by the occurrence of moderate to severe reduction in the intervertebral disc height and of the extensive radiographic changes typical of degenerative disc disease, including vacuum phenomena and vertebral body marginal sclerosis. The rate ratios with 95% confidence intervals were computed, with stratification by age groups.Thirty-eight DISH patients (55.1%) and 34 controls (50%) showed vertebral osteochondrosis. Stratification by age revealed an increased prevalence of vertebral osteochondrosis in younger DISH patients with respect to controls (p0.05).Our results show that vertebral osteochondrosis may be associated with DISH and underline the differences between classification and diagnostic criteria. Moreover, it could be hypothesized that DISH plays a predisposing role in the development of vertebral osteochondrosis during the early stages of the disease, causing an early modification in the physiological curves of the spine.

Background TNF-alpha was demonstrated in up to 60% of the cells in all areas of inflamed arteries of patients with GCA, suggesting a primary role in the inflammatory process. Infliximab, a chimeric monoclonal anti-TNF-alpha, demonstrated remarkable efficacy and safety in the treatment of several rheumatic conditions associated with a chronic inflammatory response. Objectives To date, no data are available on the therapeutic employment of infliximab in patients with GCA. The aim of this study was to evaluate the efficacy of infliximab infusions in longstanding, still requiring corticosteroids (CS) GCA. Methods Four patients (3F,1M; median age:75 ys.) with longstanding (median disease duration: 4 ys.), biopsy proven, active GCA, still requiring CS and whose disease course hab been complicated by serious CS-related adverse events, were scheduled to receive 3 infusions of infliximab (3 mg/Kg) at weeks 0, 2 and 6. If a clinical and humoral remission was obtained, CS were withdrawn after the 2nd infusion. Results Three patients had a complete remission of clinical signs/symptoms and normalisation of ESR and CRP after the 2nd infusion. The remission persisted after the 3rd infusion and over the follow-up (7, 6 and 6 months, respectively). The fourth patient did not respond to therapy and withdrew from the study after the 2nd infusion. Infliximab was well tolerated without any side-effects. Conclusion Our encouraging results suggest that TNF-alpha blockade may represent a valid alternative therapeutic approach to longstanding GCA, still requiring CS and complicated by severe adverse events. The small number of patients and the open study design do not allow conclusive considerations.

Background SAPHO syndrome (synovitis, acne, pustolosis, hyperostosis, osteitis) syndrome shares manifestations and clinical association with the spondyloarthropathy (SpA) complex. Nonsteroidal anti-inflammatory drugs (NSAIDs) are the first choice but have limited effcacy. Second-line drugs have been tried with mixed results. Positive effects with pamidronate, which partly works by blocking tumour necrosis factor alpha (TNFalpha), have recently been reported. Infliximab, a chimeric anti-TNFalpha monoclonal IgG1 antibody, has recently been proved to be effective in ankylosing spondylitis (1) and psoriatic arthritis.1–3 Objectives To evaluate the effect of infliximab in patients with SAPHO syndrome resistant to NSAIDs and second-line drugs. Methods Two patients meeting criteria for SAPHO syndrome and with chest wall pain limiting normal activity despite adequate treatment with NSAIDs and who had failed second-line therapy received three intravenous infusions of infliximab (5 mg/Kg) at weeks 0, 2, and 6. One of the two patients had skin manifestations consisting in severe acne at baseline. Patients were evaluated at baseline and on days 3, 7, 14 and from then every two weeks for the following variables: anterior chest wall pain, swelling and tenderness, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Results The two patients had a complete response to therapy 3 days after the first infusion of infliximab and discontinued NSAID therapy. Chest wall swelling and tenderness remitted and CRP became normal. Sever acne dramatically improved in one week after the first infusion. Patients remained symptom-free after the third infusion up to 2 months’ follow-up. Infliximab treatment was well tolerated, with no adverse event. Conclusion Infliximab seems to be an effective drug in SAPHO syndrome. A larger, controlled, double-blind study is required. References Brandt J, et al. Ann Rheum Dis. 2000;43:1346–52 Van der Bosch F, et al. Ann Rheum Dis. 2000;59:428–33 Antoni C, et al. J Rheumatol. 2000;27(Suppl 59):24

Polymyalgia rheumatica (PMR) is a relatively common disease of the elderly affecting the synovial membrane.1 Recent studies have emphasised the prominent involvement of the extra-articular synovial structures in both proximal and distal regions of both the arms and legs.1-7 In the distal part of the arms tenosynovial membrane inflammation is responsible for carpal tunnel syndrome, distal swelling of hands and feet with or without pitting oedema, and localised episodes of distal tenosynovitis.4-7 We recently observed the case of a patient with PMR showing retrocalcaneal bursitis, which we describe briefly here. A 68 year old woman was referred to us for evaluation of a three month history of marked aching and morning stiffness in her neck, shoulder, and hip girdles associated with low grade fever. Her medical history was otherwise unremarkable except for a hereditary cerebellar cortical degeneration. Her family history was negative for rheumatic diseases, including spondarthritis. Physical examination showed tenderness and limitation of cervical and shoulder movement. The typical gait and abnormal stance of cerebellar ataxia were also present. Laboratory evaluation disclosed an erythrocyte sedimentation rate (ESR) of 72 mm/1st h (Westergren) and a C reactive protein (CRP) concentration of 80 mg/l (normal

To determine the efficacy and safety of shoulder corticosteroid injections in polymyalgia rheumatica (PMR).Twenty consecutive patients with active PMR were randomized into a 7 month, double blind, placebo controlled study. Patients received either bilateral shoulder injections of 40 mg of 6-methylprednisolone acetate or placebo (1 ml saline solution). Responders were treated weekly with the same regimen for a total of 4 bilateral injections and then followed for 6 months. Response was defined as a 70% reduction in visual analog scale (VAS) score for pain and for patient and physician global assessment, and duration of morning stiffness. Bilateral shoulder magnetic resonance imaging (MRI) was performed at different times to evaluate the response of lesions to therapy.All 10 corticosteroid treated patients responded to the first injection with a significant reduction in duration of morning stiffness, VAS pain scale, patient and physician global assessment, erythrocyte sedimentation rate, and C-reactive protein. Interleukin 6 serum levels were significantly reduced after the 2nd injection. In 5 patients, the response persisted throughout the followup period. The other 5 withdrew within 4 weeks after the 4th injection due to recurrence of symptoms. None of the 10 patients of the placebo group responded to the first injection. The difference between the 2 groups was significant (p = 0.03). No side effects were recorded. MRI showed marked improvement of shoulder lesions one week after first injection and an almost complete resolution one week after last injection in the responders.Shoulder corticosteroid injections seem to be an effective and safe therapy for PMR.

To assess the frequency and clinical manifestations of Behçet's disease (BD) during the past 10 years in a defined area of Northwestern Spain.The charts of all the patients diagnosed as having BD at the Hospital Xeral-Calde from 1988 through 1997 were reviewed. All the patients were residents of Lugo. Patients were followed from the time of diagnosis until either the patient's death or July 1, 1998. By that time patients were classified as having complete BD if they fulfilled the International Study Group Criteria (ISG) for BD. Patients with recurrent oral ulcerations plus one of the remaining 4 criteria of the ISG were classified as having an incomplete form of BD.Sixteen patients (9 men/7 women) were diagnosed as having complete BD and 7 (3 men) as having incomplete BD. The average annual incidence rate of complete BD in the Lugo region of Northwestern Spain was 0.66/100,000. Oral ulcerations with or without genital ulcerations were the most common initial manifestations. Neurological involvement was relatively frequent in men with complete BD. In contrast, a positive pathergy test was less common. Two patients with neurological involvement and 3 with uveitis had severe sequelae. However, there was a single death, apparently not related to BD.In Northwestern Spain, BD is more common than was expected and is a nonfatal disease. However, central nervous system involvement and severe ocular complications overshadow the good prognosis observed in the majority of patients.

A patient suffering from Behcet’s syndrome and undifferentiated spondyloarthritis developed dactylitis and psoriasis over the next three years.

We describe 2 cases of Behcet's disease (BD) and thrombosis who were heterozygous for the prothrombin G--A20210 mutation. In one case, progressive uncontrolled thromboses led to death. Case-control studies are needed to support the hypothesis of the role of the prothrombin A20210 allele as a risk factor for venous thrombosis in some patients with BD.

Three more cases of B27-positive patients who developed peripheral arthritis immediately after trauma are reported. The first had an exacerbation of arthritis in the right hip after falling from her motor-bike. The second had arthritis of the distal interphalangeal (DIP) joint of the right forefinger after shutting his finger in the door of his car. The third had arthritis of the right sternoclavicular joint after a road-accident while fastening her safety belt.

Orbital magnetic resonance imaging demonstrated increased signal of the optic nerve in short time inversion recovery (STIR) images of two young women with unilateral visual blurring. In both, recurrent oral and genital ulcerations and papulopustular lesions appeared within the next 14–15 months, respectively, allowing a diagnosis of Behcet's disease. Optic neuropathy may be an early manifestation of Behct's disease and clinical follow-up is crucial for its diagnosis.

We report the case of a patient with undifferentiated spondyloarthritis (uSpA) who was successfully treated with abatacept after failing all three available tumour necrosis factor (TNF)α antagonists. Her disease had begun in 2003 with right buttock pain and asymmetrical peripheral arthritis. Human leukocyte antigen (HLA) typing for the B27 antigen, antibodies to cyclic citrullinated peptide and rheumatoid factor were negative. No erosions were observed on x rays of hands and feet. Since MRI of the sacroiliac joints (SI) showed right sacroiliitis, a diagnosis of uSpA with axial and peripheral joint involvement was made. During the first 4 years of evolution, the disease was unresponsive to different drugs including non-steroidal anti-inflammatory drugs (NSAIDs), oral and intra-articular …

We first describe the onset of acute myositis in a patient suffering from systemic sclerosis after the administration of darbepoetin alpha for renal failure-related anemia. Therapeutic implications and risks are discussed.

To investigate using magnetic resonance imaging (MRI) the part played by flexor and extensor tenosynovitis and synovitis of the metatarsophalangeal (MTP), proximal interphalangeal (PIP) and distal interphalangeal (DIP) joints in producing the "sausage-like" aspect of spondyloarthropathy (SpA) toe dactylitis.Twelve sausage-like toes and corresponding contralateral toes of 7 consecutive patients meeting Amor criteria for SpA were studied by MRI.All dactylitic toes showed fluid collections in the flexor synovial sheaths on MRI. Due to the sheath distension the plantar bone to skin distance was significantly increased (p0.05) in the dactylitic toes compared to normal contralateral toes. Peritendinous soft tissues were not involved since these were significantly thicker (p0.05) in normal toes. Extensor synovial sheaths were involved in only 4 dactylitic toes. Of the 36 joints of the 12 dactylitic toes only 2 MTP joints showed capsule distension. Considering MRI as the "gold standard", examination showed 100% sensitivity and specificity for flexor sheath involvement but lacked sensitivity for extensor synovial sheaths and showed a low specificity for joint capsule distension.Like finger dactylitis, toe dactylitis may also be due to flexor tenosynovitis and synovitis of MTP, PIP, and DIP joints may not be a required condition for sausage-shaped appearance. Extensor tenosynovitis may be present in addition to flexor tenosynovitis. Physical examination is a sufficient method for diagnosing toe dactylitis.

The case of a man suffering from undifferentiated seronegative spondyloarthropathy (uSpA) without spine involvement and with iliolumbar ligament ossification is reported. Unlike a similar previously published case showing ossification only of the middle part of the left ligament, our patient had ossification of the whole course of both ligaments.

Background Till now the debate on the relationship between Behcet’s disease (BD) and spondyloarthritis (SpA) has been limited to whether the frequency of sacroiliitis and ankylosing spondylitis (AS) is greater or not in patients with BD than in controls. But if BD and SpA occur together more frequently than by change, it should be possible to find patients with BD showing different forms of SpA. In actual fact, some patients with BD and undifferentiated SpA were reported in the last two decades. Recently, the Assessment of SpondyloArthritis international Society (ASAS) developed and validated classification criteria for axial and peripheral SpA that are going to substitute the Amor criteria and the European Spondylarthropathy Study Group (ESSG) criteria for all forms of SpA. Objectives The aim of our study was to evaluate the frequency of Italian patients with BD meeting the ASAS classification criteria for SpA. Methods Consecutive patients attending the outpatient clinic for BD of the Rheumatology Department of Lucania and meeting the International Study Group (ISG) criteria were assessed. Each patient was asked about the presence of SpA or related diseases, such as psoriasis and inflammatory bowel disease (IBD), in his first-degree relatives. Each patient was evaluated for clinical manifestations of SpA including, inflammatory low back pain, spinal limitation and deformity, peripheral arthritis, peripheral enthesitis, dactylitis and acute anterior uveitis. Pelvic plain films were obtained in all patients and examined blindly and independently for sacroiliac joint changes by two observers. HLA typing was performed by PCR-SSP. Results During a 6-month period, 74 consecutive patients (49 M, 25 F; mean age 39.8±11.4 yrs; mean disease duration 17.8±8.9 yrs; 64% HLA-B51 positive) with BD were recruited. Seven (9.4%) out of them met the ASAS classification criteria for SpA. Of these, 1 had axial disease with the feature of AS and 6 peripheral SpA. In 2 of these 6, acute anterior uveitis without posterior involvement was essential for the diagnosis of SpA. The remaining 4 had psosiasis, dactylitis, enthesitis or inflammatory chronic low back pain in addition to peripheral arthritis. Two of the 7 patients with SpA features were HLA-B27 positive. Conclusions Italian patients with BD show a higher frequency of SpA, as defined by the ASAS criteria, than that expected in the general population. Disclosure of Interest None Declared

Background In the only Italian population-based epidemiologic study on Behcet’s disease (BD) carried out in a northern area, the Reggio Emilia district, the prevalence of the disease was 3.8 per 100.000 inhabitans. Objectives The aim of the present study was to evaluate the prevalence of Behcet’s disease (BD) in Basilicata (or Lucania), a Region of southern Italy. Methods Patients with BD living in Basilicata for at least 12 months prior to diagnosis were identified through the following sources: general practitioners, community-based specialists, San Carlo Hospital and Madonna delle Grazie Hospital specialists, the Basilicata centralized index and the Basilicata database for rare diseases. All identified patients were contacted by phone and were recalled to our outpatient clinic for re-evaluation. Patients were classified as having complete BD if they met the International Study Group (ISG) criteria for BD. Prevalence was established in July 2011. The prevalence rate was obtained by dividing the number of patients who had the disease by the number of individuals in the Basilicata (Lucania) population. Results By screening of a population of 597.768 subjects, 77 patients with a diagnosis of BD were identified and 60 of them (87.3%) were evaluated. Fifty-five (22 males and 33 females) out of these satisfied the ISG criteria and permitted us to obtain a prevalence rate of 9,2 cases per 100.000 inhabitans. Conclusions This cross-sectional population-based study suggests that BD is more frequent in the southern part than in the northern part of Italy and confirms that the prevalence of the disease increases in a north-to-south manner within the European continent. Disclosure of Interest None Declared

Objectives To evaluate the efficacy and safety of long-term administration of Infliximab in BD patients refractory to conventional therapy. Methods In this ongoing, prospective, longitudinal and observational study BD patients unresponsive to conventional therapy with corticosteroids plus at least two different immunosuppressive drugs were treated with Infliximab therapy (5 mg/kg intravenously at weeks 0, 2, 6, and every 6-8 weeks subsequently). Response to anti-TNF therapy was based on the expert opinion and was graded as follows: remission, response, no response and worsening. Remission was defined as the complete disappearance of symptoms and signs of inflammation and response as at least 50% of improvement. Results Since 2003, 68 BD patients (45 M/23 F; mean age 33.9±11.2 yrs; mean disease duration 12.4±9.5 yrs; 67% HLA-B51 positive) were enrolled. Indication for Infliximab treatment were uveitis in 35 patients, severe mucocutaneous manifestations in 14, CNS vasculitis in 11, optic neuritis in 1, intestinal involvement in 2, arthritis in 2, seizure in 2, vascular involvement in the remaining 1. The median duration of infliximab treatment was 25.5 months (range 1-106 months). Infliximab therapy resulted in a remission in 20 patients with uveitis, in 6 with CNS involvement, in 3 with severe mucocutaneous manifestations, in 1 with optic neuritis, in 1 with arthritis and in 1 with vascular involvement. A response was observed in 4 patients with mucocutaneous manifestations. The drug was stopped for lack of efficacy in 4 patients with severe mucocutaneous manifestations, 2 with uveitis, 2 with CNS vasculitis, 1 with seizure. Seventeen BD patients stopped infliximab therapy for loss of efficacy. Six BD patients discontinuated infliximab treatment for serious adverse events (5 for infusion reactions, 1 for bacterial pneumonia). Adverse events not requiring drug discontinuation were observed in 7 patients (mild infusion reactions in 2, upper respiratory tract infection in 6, urinary tract infection in 1). Conclusions In our experience infliximab has been showed effective and safe in a long-term follow-up in patients with refractory BD. Disclosure of Interest None Declared

Background Therapeutic targets have been defined for diseases like diabetes, hypertension or rheumatoid arthritis and adhering to them has improved outcomes. Such recommendations are unavailable for spondyloarthritis. Objectives To define the treatment target for spondyloarthritis including ankylosing spondylitis (AS) and psoriatic arthritis (PsA) and develop recommendations for achieving the target, including a treat-to-target management strategy. Methods Based on results of a systematic literature review (performed by MS) and expert opinion, a task force of expert physicians and patients developed recommendations, which were broadly discussed and voted upon in a Delphi-like process. Level of evidence, grade of recommendation and strength of recommendation were derived by respective means. Results The literature review did not reveal trials comparing a treat-to-target approach with another or no strategy, but provided indirect evidence that facilitated the development of recommendations. The task force agreed on 5 overarching principles and 11 recommendations. Based on the current perception and classification of spondyloarthritis (including PsA, axial SpA (AS and non-radiographic axial SpA), peripheral SpA), only the last 2 recommendations are separate for axial spondyloarthritis, peripheral spondyloarthritis and PsA, while principles and 9 of the recommendations form a common trunk for all types of spondyloarthritis. The main treatment target, which should be based on a shared decision with the patient, was defined as remission, with an alternative target being low disease activity. Means to assess disease activity are discussed. Regular follow-up examinations should safeguard the evolution of disease activity toward the targeted goal. Additional items relate to extraarticular and extramusculoskeletal aspects and other factors, such as comorbidity. While the level of evidence was low for all items, the mean strength of recommendation was 9-10 (10: maximum agreement) for all recommendations. A research agenda was formulated. Conclusions The recommendations shall inform patients, rheumatologists, dermatologists and other experts as well as other stakeholders about expert opinion that allows reaching optimal outcomes of spondyloarthritis including AS and PsA. Acknowledgements This work was supported by an unrestricted educational grant from Abbott. Disclosure of Interest J. Smolen Grant/research support from: Abbott, Consultant for: Abbott, J. Braun: None Declared, M. Dougados: None Declared, P. Emery: None Declared, O. FitzGerald: None Declared, P. Helliwell: None Declared, A. Kavanaugh: None Declared, T. Kvien: None Declared, R. Landewe: None Declared, T. Luger: None Declared, P. Mease: None Declared, I. Olivieri: None Declared, J. Reveille: None Declared, C. Ritchlin: None Declared, M. Rudwaleit: None Declared, M. Schoels: None Declared, J. Sieper: None Declared, M. de Wit: None Declared, D. van der Heijde: None Declared