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Background Psoriatic arthritis (PsA) is a chronic inflammatory disease associated with psoriasis (PsO). Early diagnosis and prompt therapeutic intervention are crucial for limiting PsA progression and prevention of disability. Dermatologists are in a privileged position to detect early PsA. The management of patients with PsA in the dermatology setting is widely variable. Objective To provide practical recommendations for the management of patients with PsA in the dermatology setting including early diagnosis and treatment. Methods A consensus document was written by an expert panel composed by dermatologists (n = 12) and rheumatologists (n = 6). Eleven highly relevant questions were selected and elaborated with answers/statements based on a narrative literature review. The resulting document was discussed in a face-to-face meeting adopting a nominal group technique to reach consensus (i.e. 100% agreement) using the Delphi method. Results A consensus was achieved in defining the following: the clinical characteristics differentiating inflammatory and non-inflammatory signs and symptoms of joint disease; the most important differential diagnoses of PsA in clinical practice; the most useful screening questionnaires, serum laboratory tests and imaging techniques for the detection of early PsA; the criteria for dermatologist to refer patients with PsO to rheumatologist; the criteria for the diagnosis of PsA; the selection of the indices that the dermatologist could use for measuring the activity and severity of PsA in clinical practice; when systemic steroids and/or intra-articular steroid injections are indicated in the treatment of PsA. Finally, systemic treatments including synthetic and biologic disease-modifying antirheumatic drugs to be considered for the treatment of PsA have been reported. Conclusions The implementations of these practical recommendations could be very helpful for the management of patients with PsA in the dermatology setting including early diagnosis and treatment.

Background Rheumatic diseases (RD) predominantly affect young women during reproductive age. Pregnancy, contraception and family planning (FP) are crucial for the quality of life of these patients. Objectives We aimed to investigate 9women9s health9 through a self-reported questionnaire. Answers from patients with connective tissue diseases (CTD) vs chronic arthritis (CA) were compared. Methods 24 centres distributed the questionnaire (65 multiple-choice and 12 open-answer questions) to women with RD (18–45years) regularly attending their outpatient clinics. Results Answers were collected from 249 CTD vs 149 CA patients. Their desire to have children was influenced by RD in 40% of cases: half of them reduced the number of children they wanted (Table 1). 39% CA vs 29% CTD were afraid of being mother because of disability. 24% CTD vs. 18% CA had at least one miscarriage; 21% CTD vs. 2% CA had more than one. 31% CTD and 34% CA were never asked about their desire to have children. 61% CTD vs 70% CA received counselling about contraception, given by a gynaecologist (G) (58% vs 64%), rheumatologist (R) (22% vs 14%) or both (7% vs 9%). 60% in both groups received a counselling before pregnancy: 34% vs 39% from R and G, 14% vs 22% by R. This positively changed family planning in 64% vs 59%. We created a Knowledge Index (based on the average of the normalized performed scores on 6 key questions for different sections): 55% CTD patients vs 44% CA had a medium-high score. A higher score directly correlated with the desire to became pregnant and with a multidisciplinary counselling. Conclusions This survey suggested that CTD have a major impact on FP and family size, possibly mediated by the increased rate of miscarriages as compared to CA. Concerns about reproductive issues could be positively overcome by adequate counselling. Rheumatologists should implement the discussion about FP and the compatibility of drugs with pregnancy in the management of young women with RD, especially those with CTD for whom contraception and pregnancy have particular implications. Acknowledgements Statistical analysis supported by an unrestricted grant by UCB Pharma Thanks to Patients9 Associations and Participants Disclosure of Interest None declared

Background Chronic rheumatic conditions such as rheumatoid arthritis (RA), ankylosing spondylitis (AS), and psoriatic arthritis (PsA) are associated with severe morbidity and significant impairment of patients9 health related quality of life (HRQoL). Several treatments are available but not all the patients respond positively to them. Biologic therapies such as anti-TNFα agents are shown to benefit who fail or have partial responses to standard DMARD therapy. Objectives Within a multicenter stated preferences study (CARA Study), we assessed HRQoL in patients with RA, AS and PsA, and estimated relationship of HRQOL with the different diagnoses, clinical characteristics and biological treatment experience. Methods Patients with RA, AS, or PsA, who at the time of enrollment were following a treatment (experienced) or received a first prescription (naive) of treatment with biological drugs were enrolled. Together with preferences data, clinical and HRQOL information was reported. HRQoL was assessed with the recently developed and successfully validated version of the EQ-5D-5L, which allows to obtain a description of health (in 5 domains and 5 levels of severity each), a measure (EQ-VAS) and a valuation (utility) of health. Multiple linear regression analyses were conducted to assess the association between EQ-5D VAS score and the utility with age, sex, diagnosis, treatment experience, years from symptoms onset and years from diagnosis. Results 513 patients were enrolled (mean±SD =50.0±13.6, 42.5% female). As regards the diagnosis, 33.9% had RA, 34.9% PsA and 31.2% AS. The mean±SD time from the symptoms onset was 10.8±9.4 and from the diagnosis was 8.0±8.2 years. Almost half of the patients (47.4%) were naive to the biological treatment. Patients reporting severe or extreme problems were: 7.1% in mobility, 3.6% in self-care, 10.3% in usual activities, 18.6% in pain/discomfort, 5.5% in anxiety/depression. The mean±SD of the VAS was 60.4±20.5 and of the utility was 0.773±0.116. From the regression model the VAS and utility are significantly (p Conclusions Patients naive to biological treatment have significat lower levels of HRQoL, suggesting that their current situation is not satisfactory and need to start with a more effective treatment. Disclosure of Interest L. Sinigaglia: None declared, L. Scalone: None declared, P. Sarzi-Puttini: None declared, C. Montecucco: None declared, R. Giacomelli Grant/research support from: MSD, G. Lapadula: None declared, I. Olivieri: None declared, A. Giardino Employee of: MSD Italia, G. Didoni Employee of: MSD Italia, P. Cortesi Grant/research support from: Gilead, L. Mantovani: None declared, M. Mecchia Employee of: MSD Italia

Background Rheumatic diseases (RD) affect women during reproductive age. Children9s outcome is a major topic for counselling on family planning, but no large studies are available. Objectives We aimed at assessing the long-term health conditions of children born to mothers with RD through a self-reported questionnaire. Methods 24 Rheumatology Centers distributed the questionnaire (65 multiple choice and 12 open-answer questions) to consecutive women with RD attending their outpatient clinic during September 2015. Data were compared according to maternal diagnosis (MD) -chronic arthritis (CA) or connective tissue disease (CTD)- and to the timing of pregnancy (before or after MD of RD). Results The survey yielded data about 269 children born to 184 mothers (63 CA, 121 CTD). According to MD, children had a mean age of 17.1 (±9.6 SD) and 14.4 (±9.0 SD) years at the time of interview, and male children were 52/93 (56%) and 91/176 (52%), respectively. Twenty-nine children in the CA group (31.2%) and 64 in the CTDs group (36.4%) were born after MD of RD. Pre-term delivery (before 37 weeks) was observed in 48 cases (17.8%), mostly children born to mothers with CTD (37/48, 77%). Regarding school performance, 12 children (4.5%) repeated one year of school, in 7 cases for indolence, in 3 for learning disabilities (LD)/health problems (HP), in 2 for family problems. Eleven of these children were born before MD. Overall, 9 children (3.3%) were diagnosed with a LD and 53 children were affected by HP requiring either hospitalization or evaluation by a Specialist (Table). Three children (1%) were affected by autoimmune disease. Conclusions The long-term follow-up of children born to women with RD is reassuring of an outcome similar to that of the general pediatric population (GPP). Autoimmune diseases are not frequent. Problems seem to cluster in children born to CTD, especially after MD, with a higher frequency of LD (6.3% vs 2.5–3.5% of GPP), but no particular pattern of exposure to maternal autoantibodies nor drugs was observed. Acknowledgements Statistical analysis supported by an unrestricted grant by UCB Pharma Thanks to Patients Associations and Participants to the survey Disclosure of Interest None declared

Background Extra-articular manifestations (EAMs), such as uveitis, inflammatory bowel diseases (IBD) and psoriasis (PsO), frequently complicate the disease course of patients with axial spondyloarthritis (axSpA), although prevalence data on this regard are still controversial. The occurrence of EAMs might also contribute to the decision of introducing a biologic therapy and even influence the choice between the available TNF inhibitors (TNFis). Objectives The aim of this study is to retrospectively evaluate the prevalence of EAMs in a multicentre cohort of axSpA patients treated with TNFi, investigating how these influenced the choice of treatment. Methods Clinical data from axSpA patients treated with a TNFi between May 2003 and May 2016 where obtained from a multicentre registry. Prevalence of EAMs (uveitis, IBD and PsO) was calculated at the time of TNFi prescription, evaluating their distribution according to drug subgroup. Results The study included 503 patients with axSpA (172 [34.2%] women, mean age [±SD] 40.5 [±13.2] years, mean disease duration 9.7 [±14.7] years), receiving a total of 675 lines of treatment (I-line n=503, II-line n=118, ≥ III-line n=54) with a TNFi (272 infliximab [IFX], 173 adalimumab [ADA], 89 golimumab [GOL], 141 etanercept [ETN]). At the time of TNFi introduction, 28.6% patients claimed at least one EAM (IBD 11.3%, uveitis 10.9%, and PsO 8.8%). The baseline presence of at least one EAM was associated with a more frequent prescription of an anti-TNF monoclonal antibody rather than etanercept (34.1% versus 21.9%, respectively; p=0.005). In detail, EAMs were found in 41.6, 36.9, 29.8, and 21.9% patients treated with GOL, ADA, IFX, or ETN, respectively. The prevalence of IBD was significantly higher (p=0.004) in patients treated with ADA (12.7%), IFX (14.3%), or GOL (11.2%) compared with ETN (4.9%). Uveitis was numerically more frequent in GOL (20.2%) and ADA (13.3%) rather than IFX (9.5%) and ETN (9.9%) groups. Finally, PsO prevalence was similar in patients treated with ADA (10.9%) and GOL (10.1%), and numerically lower in the ETN (7.1%) and IFX (5.9%) groups. Conclusions In our cohort of axSpA patients treated with TNFis, EAMs were highly represented. The presence of extra-articular involvement has been carefully taken into account when a TNFi was required to better control the disease. In particular, IBD and uveitis drove more frequently the choice toward an anti-TNF monoclonal antibody instead of the receptor. Disclosure of Interest None declared

Background The development of biologic therapies has created a more complex decision-making process to select the treatment option for patients. In order to optimize the appropriateness of the decisions, it is necessary to be informed and aware of the preferences of the interested parties and the influence of their experiences on their preferences for the different treatments. Objectives To estimate preferences of relevant treatment characteristics valued by the different subjects involved in the management of patients with rheumatic diseases. This abstract focuses on patients9 preferences. Methods We involved patients with rheumatoid arthritis (RA), ankylosing spondylitis (AS) or psoriatic arthritis (PsA), who according to clinical practice, at the time of data collection had for the first time a prescription of (naive), or received treatment with (experienced) biological drugs for at least 3 months in the last 12 months. Through a Discrete-Choice-Experiment, the participants valued 16 possible scenarios in which pairs of similarly effective treatments were described with 6 characteristics including 2–4 possible levels each: (1) frequency of administration; (2) mode and place of administration; (3) hospitality, service, efficiency and courtesy of health personnel; (4) frequency of reactions at the site of drug administration; (5) generalized undesired reactions or allergic reactions involving the whole body; (6) additional contribution added as healthcare taxes to be paid by all the citizens to make available the treatment to target patients. Results 513 patients from 30 centres through Italy participated, balanced for diagnosis and treatment experience (around 20% of each subgroup). Characteristics 4, 3 and 6 were the first, second and third most important ones for every subgroup, the fourth most important characteristic was 1 (experienced RA), 5 (naive AS), and 2 for the other subgroups. Across all the subgroups, patients generally preferred very satisfactory levels of (3), infrequent (4), mild (5), and no (6). Instead, for characteristics (1) and (2) the patients generally preferred the frequency, mode and place of administration that were closer to those actually experienced or prescribed. Conclusions Taking into account the different opinions of patients on at least some treatment characteristics could guide the conduction of good choices aimed to optimize benefits and to allocate efficiently resources. Disclosure of Interest L. Sinigaglia: None declared, P. Sarzi-Puttini: None declared, L. Scalone: None declared, C. Montecucco: None declared, R. Giacomelli Grant/research support from: MSD, G. Lapadula: None declared, I. Olivieri: None declared, A. Giardino Employee of: MSD Italia, P. Cortesi Grant/research support from: Gilead, L. Mantovani: None declared, M. Mecchia Employee of: MSD Italia

Objective Behcet's disease (BD) is a chronic, relapsing, inflammatory vascular disease with no pathognomonic test. Low sensitivity of the currently applied International Study Group (ISG) clinical diagnostic criteria led to their reassessment. Methods An International Team for the Revision of the International Criteria for BD (from 27 countries) submitted data from 2556 clinically diagnosed BD patients and 1163 controls with BD-mimicking diseases or presenting at least one major BD sign. These were randomly divided into training and validation sets. Logistic regression, ‘leave-one-country-out’ cross-validation and clinical judgement were employed to develop new International Criteria for BD (ICBD) with the training data. Existing and new criteria were tested for their performance in the validation set. Results For the ICBD, ocular lesions, oral aphthosis and genital aphthosis are each assigned 2 points, while skin lesions, central nervous system involvement and vascular manifestations 1 point each. The pathergy test, when used, was assigned 1 point. A patient scoring ≥4 points is classified as having BD. In the training set, 93.9% sensitivity and 92.1% specificity were assessed compared with 81.2% sensitivity and 95.9% specificity for the ISG criteria. In the validation set, ICBD demonstrated an unbiased estimate of sensitivity of 94.8% (95% CI: 93.4–95.9%), considerably higher than that of the ISG criteria (85.0%). Specificity (90.5%, 95% CI: 87.9–92.8%) was lower than that of the ISG-criteria (96.0%), yet still reasonably high. For countries with at least 90%-of-cases and controls having a pathergy test, adding 1 point for pathergy test increased the estimate of sensitivity from 95.5% to 98.5%, while barely reducing specificity from 92.1% to 91.6%. Conclusion The new proposed criteria derived from multinational data exhibits much improved sensitivity over the ISG criteria while maintaining reasonable specificity. It is proposed that the ICBD criteria to be adopted both as a guide for diagnosis and classification of BD.

BackgroundSince the publication of the European League Against Rheumatism recommendations for the pharmacological treatment of psoriatic arthritis (PsA) in 2012, new evidence and new therapeutic agents have emerged. The objective was to update these recommendations.MethodsA systematic literature review was performed regarding pharmacological treatment in PsA. Subsequently, recommendations were formulated based on the evidence and the expert opinion of the 34 Task Force members. Levels of evidence and strengths of recommendations were allocated.ResultsThe updated recommendations comprise 5 overarching principles and 10 recommendations, covering pharmacological therapies for PsA from non-steroidal anti-inflammatory drugs (NSAIDs), to conventional synthetic (csDMARD) and biological (bDMARD) disease-modifying antirheumatic drugs, whatever their mode of action, taking articular and extra-articular manifestations of PsA into account, but focusing on musculoskeletal involvement. The overarching principles address the need for shared decision-making and treatment objectives. The recommendations address csDMARDs as an initial therapy after failure of NSAIDs and local therapy for active disease, followed, if necessary, by a bDMARD or a targeted synthetic DMARD (tsDMARD). The first bDMARD would usually be a tumour necrosis factor (TNF) inhibitor. bDMARDs targeting interleukin (IL)12/23 (ustekinumab) or IL-17 pathways (secukinumab) may be used in patients for whom TNF inhibitors are inappropriate and a tsDMARD such as a phosphodiesterase 4-inhibitor (apremilast) if bDMARDs are inappropriate. If the first bDMARD strategy fails, any other bDMARD or tsDMARD may be used.ConclusionsThese recommendations provide stakeholders with an updated consensus on the pharmacological treatment of PsA and strategies to reach optimal outcomes in PsA, based on a combination of evidence and expert opinion.

Background As psoriatic disease (PD) is a condition characterized by the combination of inflammatory skin (psoriasis) and osteo-articular manifestations (psoriatic arthritis), its treatment should cover both its clinical components. Objective The objective of this study was to propose a flexible framework for the use of biological agents in PD. Methods The proposal was drawn up by a group of dermatologists and rheumatologist expert in PD and was based on existing evidence and personal opinion. Results The three TNF-a inhibitors (adalimumab, etanercept, infliximab) are effective in all of the psoriatic manifestations and should be used in the case of moderate/severe disease refractory to systemic treatment with non-biological drugs. We propose the following definitions of moderate/severe disease: PASI > 10 or BSA > 10 or DLQI > 10 for plaque-psoriasis; BSA ‡ 10 or DLQI ‡ 10 for the other psoriatic skin lesions; DLQI ‡ 10 or meaningful values of the NAPSI or mNAPSI for psoriatic nail involvement; ‡ 1 inflamed joint + patient global VAS 3 4 + physician’s judgement or arthritic joint deformities or radiographic joint damage plus ‡ 5 inflamed small joints or ‡ 1 large joints for peripheral joint involvement; ‡ 1 digit with dactylitis and ‡ 1 enthesitic sites + patient global VAS 3 4 + physician’s judgement for dactylitis and enthesitis. BASDAI ‡ 4 + physician’s judgement for spondylitis; recurrent flares or risk of developing irreversible damage for uveitis. Other assessment instruments can be used if the physician is more familiar with them and if they have been validated. Conclusion We provide a shared dermatological and rheumatological proposal for the use of biological agents in PD.

Objectives: To present and analyse the literature sources regarding the management of Behcet 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)α in mucocutaneous involvement. There were no RCTs with IFNα or tumour necrosis factor (TNF)α 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.

Objectives: To determine the type and proportion of patients with ankylosing spondylitis who rheumatologists consider to be candidates for treatment with tumour necrosis factor (TNF)-blocking agents, and to what extent this is in agreement with the ASsessment in Ankylosing Spondylitis (ASAS) international working group recommendations on initiation of treatment with anti-TNF agents. Methods: Participants were rheumatologists from 10 different countries, who were considered to be experts in treating patients with ankylosing spondylitis and in the use of anti-TNF treatment, but were unaware of the ASAS recommendations (unpublished at the time of study in 2003). The first 10 consecutive patients with ankylosing spondylitis seen by the rheumatologist were evaluated as to whether the patient was a candidate for anti-TNF treatment. Thereafter, a metrologist assessed the patient for disease activity and severity, and collected data on demographics and treatment. Results: Complete data were available for 1207 of the 1284 patients and were used for analysis. Overall, the rheumatologists indicated that they would initiate TNF-blocking agents in 49.3% of patients, ranging from 37.2% patients in Canada to 78.3% in Australia. These candidates had higher disease activity, higher levels of acute-phase reactants, worse spinal mobility, worse function, more often hip involvement and a higher prevalence of sick leave. Of all patients considered to be candidates, 40% did not fulfil ASAS recommendations with respect to previous use of non-steroidal anti-inflammatory drugs (NSAIDs; at least two NSAIDs) or Bath Ankylosing Spondylitis Disease Activity Index (⩾4). Conversely, 36% of patients who did not fulfil the NSAID or BASDAI recommendations were still considered to be candidates for TNF-blocking treatment. Objective variables, such as C reactive protein, erythrocyte sedimentation rate or magnetic resonance activity, were considered less important than disease activity in the decision on starting TNF-blocking drugs. The only important objective criterion was rapid radiographic progression. Conclusion: Rheumatologists wanted to initiate TNF-blocking drugs in roughly half of the patients with ankylosing spondylitis. However, there was a wide variation across countries and doctors. Rheumatologists considered both disease activity and severity to be determinants of starting TNF blockers, but their decision was often in disagreement with ASAS recommendations.

The spondyloarthritis (SpA) complex includes ankylosing spondylitis (AS), reactive arthritis, psoriatic arthritis, arthritis related to inflammatory bowel disease and forms that do not meet established criteria for these definite categories which are designated as undifferentiated SpA. In the early 1990s, two sets of classification criteria were suggested with the purpose to cover the whole clinical spectrum of SpA: the Amor criteria and the European Spondylarthropathy Study Group (ESSG) criteria...

Background Current recommendations for the management of axial spondyloarthritis (SpA) and psoriatic arthritis are to monitor disease activity and adjust therapy accordingly. However, treatment targets and timeframes of change have not been defined. An international expert panel has been convened to develop ‘treat-to-target’ recommendations, based on published evidence and expert opinion. Objective To review evidence on targeted treatment for axial and peripheral SpA, as well as for psoriatic skin disease. Methods We performed a systematic literature search covering Medline, Embase and Cochrane, conference abstracts and studies in http://www.clinicaltrials.gov. Results Randomised comparisons of targeted versus routine treatment are lacking. Some studies implemented treatment targets before escalating therapy: in ankylosing spondylitis, most trials used a decrease in Bath Ankylosing Spondylitis Disease Activity Index; in psoriatic arthritis, protocols primarily considered a reduction in swollen and tender joints; in psoriasis, the Modified Psoriasis Severity Score and the Psoriasis Area and Severity Index were used. Complementary evidence correlating these factors with function and radiographic damage at follow-up is sparse and equivocal. Conclusions There is a need for randomised trials that investigate the value of treat-to-target recommendations in SpA and psoriasis. Several trials have used thresholds of disease activity measures to guide treatment decisions. However, evidence on the effect of these data on long-term outcome is scarce. The search data informed the expert committee regarding the formulation of recommendations and a research agenda.

The objective of this study was to evaluate the predictive factors for achieving partial remission (PR) in patients with ankylosing spondylitis (AS) treated with anti-TNFα. We longitudinally enrolled in a multi-center study 214 AS patients, classified according to New York criteria, treated with anti-TNFα drugs adalimumab (ADA), etanercept (ETA) and infliximab (INF) with at least 12 months of follow up. PR was reached when the score was

Musculoskeletal manifestations in a population-based cohort of inflammatory bowel disease patients.Salvarani C, Vlachonikolis IG, van der Heijde DM, Fornaciari G, Macchioni P, Beltrami M, Olivieri I, Di Gennaro F, Politi P, Stockbrugger RW, Russel MG; European Collaborative IBD Study Group.Dept. of Rheumatology, Arcispedale S. Maria Nuova, Reggio Emilia, Italy. salvarani.carlo@asmn.re.itBACKGROUND: Musculoskeletal disorders are the most common extra-intestinal manifestation of inflammatory bowel disease (IBD). Wide ranges of prevalence have been reported depending on the criteria used to define spondylarthropathy and on the selection of patients. We aimed to evaluate the prevalence and clinical spectrum of musculoskeletal manifestations in an inception cohort of European IBD patients. METHODS: From 1 October 1991 to 30 September 1993, 202 IBD patients were diagnosed in three centres of two countries (Italy and The Netherlands) by means of a population-based inception cohort study. Of this group of patients, 160 (79%) were interviewed and examined by a rheumatologist and a gastroenterologist in the period June-September 1996. A total of 139/160 patients had an antero-posterior radiograph of the pelvis, and in 140/160 HLA-B27 was determined. RESULTS: 53 (33.1%) of the 160 patients had experienced at least one musculoskeletal manifestation, 29 (18.1%) satisfied the European Spondylarthropathy Study Group (ESSG) criteria for spondylarthropathy and 5 (3.1%) satisfied the modified New York criteria for ankylosing spondylitis. However, 23 (14.4%) patients developed one or more spondylarthropathy-related manifestations without fulfilling any of the classification criteria. In patients satisfying ESSG criteria a significantly higher frequency of women (P = 0.03), of ocular and liver involvement (P = 0.01 and P = 0.03, respectively), and use of immunosuppressive drugs (P = 0.02) was observed. CONCLUSION: Our study shows a high prevalence of musculoskeletal manifestations in an inception cohort of IBD patients. The clinical spectrum is broader than that defined by spondylarthropathy criteria.

Objectives. To describe a case-series of patients who presented with the typical postural abnormalities of long-standing advanced ankylosing spondylitis (AS) but were instead found to suffer from diffuse idiopathic skeletal hyperostosis (DISH). Methods. We enrolled consecutive patients who showed postural abnormalities, which at first suggested to us the diagnosis of long-standing advanced AS, although the diagnostic process led us to the correct diagnosis of DISH. Each patient had a complete physical examination and radiographs of the spine and pelvis, and was investigated for HLA-B27 locus typing. Results. From 15 June 1998 to 15 June 2006, 15 patients with DISH were seen who presented with the typical postural abnormalities of long-standing advanced AS. All patients were males with a median age of 69yrs (range 51-91). All lacked HLA-B27 and denied personal or family history of spondyloarthritis. All measurements assessing cervical, thoracic and lumbar spinal movement were abnormal. Conclusions. Patients suffering from DISH can occasionally have severe limitations of spinal mobility, along with postural abnormalities that resemble long-standing advanced AS. Thus, the differential diagnosis between DISH and advanced AS is not limited to the radiological findings and can also extend to the clinical findings in the two diseases, as is highlighted by our report.

We evaluated the prevalence of hepatitis C virus (HCV) infection in Italian patients suffering from fibromyalgia (FM), in comparison with patients affected by non-HCV related rheumatic degenerative disorders. Consecutive patients with FM and a statistically comparable group of patients suffering from peripheral osteoarthritis (OA) or sciatica due to L4-L5 or L5-S1 herniated disc were tested for HCV infection with a third-generation microparticle enzyme immunoassay (MEIA). In the positive cases, a third-generation recombinant immunoblot assay (RIBA) confirmatory test and serum HCV-RNA test were performed. Fisher's exact test was performed to compare the prevalence of HCV infection (MEIA- and RIBA-positive results) obtained in the two enrolled groups. Enrolled were 152 subjects suffering from FM and 152 patients with peripheral OA or sciatica. Anti-HCV antibodies were found in 7/152 (4.6%) patients suffering from FM and in 5/152 (3.3%) of control subjects. No statistically significant differences in HCV prevalence were detected between cases and controls. Our present report does not confirm previous data indicating an increased prevalence of HCV in FM patients and does not seem to support a significant pathogenetic role of HCV under this condition.

The case of a patient with seronegative spondyloarthropathy showing oligoarthritis of the hand together with large pitting oedema is reported. Unlike the patients with late on set peripheral spondyloarthropathy described by Dubost and Sauvezie who show minimal involvement of the axial skeleton, the patient has been suffering from AS for about twenty years.

Background Quality of Life (QoL) is reduced in patients (pts) with axial spondyloarthritis (axSpA) who suffer from pain, stiffness and fatigue in their most productive years of life. Currently, data on QoL and impact on daily activities in axSpA pts classified according to the ASAS criteria are limited. No medical research in Italy has been conducted in AxSpA pts Objectives To assess the evolution of QoL in pts suffering from axSpA over a 12-month period and disease impact on daily life activities Methods QUASAR is a multicenter, longitudinal, observational study on adult axSpA pts in Italy. This interim analysis reports baseline data. Inclusion criteria were diagnosis of axSpA (either non radiographic [nr-axSpA] or ankylosing spondylitis [AS]) based on the ASAS classification criteria and ability to understand and complete the questionnaires. The following clinical parameters were recorded: CRP, HLA-B27, pain, stiffness, disease activity (BASDAI and ASDAS), Patient and Physician Global Assessment (PtGA/PGA), comorbidities and treatment. Subjects were asked to complete the ASQoL, WPAI, MARS, EQ-5D questionnaires and a VAS on satisfaction with the treatment during routine visits. Descriptive statistics (mean±Standard Deviation [SD] or frequencies) are presented for baseline clinical characteristics, disease activity and QoL. Mann-Whitney test, t-test, χ 2 were used to calculate differences between pts groups Results A total of 512 pts (mean age 47.8±13.1 years; males 62.1%) were enrolled. 19% had a family history of SpA and 21% of psoriasis. 97 pts (18.9%) had a diagnosis of nr-axSpA. 246 (61.5%) subjects out of the 400 typed were HLA-B27 positive (48.7% and 62.7% of nr-axSpA and AS pts, respectively). Time to diagnosis since symptom onset was 5.4±2.9yrs for all pts, 3.8±3.1yrs for nr-axSpA and 5.8±2.8yrs for AS pts. 59.8% of nr-axSpA and 58.3% of AS pts had pain at the first visit. Low back pain was the most frequent type (73.3% of all pts) followed by cervical pain (35%) and dorsal pain (23.3%). 52.6% of nr-axSpA and 56.1% AS pts had morning stiffness. The following table shows the baseline demographic and characteristics of the pts involved in QUASAR: Extra Articular Manifestations (EAM) were as follows: psoriasis 17.6%, uveitis 15.8%, ulcerative colitis (UC) 4.1% and Crohn9s disease 6.5%. EAMs were present in 24.7% and 15.9% (psoriasis), 15.5% and 15.9% (uveitis), 5.2% and 3.9% (UC), 4.1% and 7.0% (Crohn9s disease) of nr-axSpA and AS pts Conclusions QUASAR is the first prospective Italian study with a large sample size that reports an overall similar burden of disease in nr-axSpA and AS patients, documented by pain, CRP, PGA/PtGA, BASDAI, ASDAS, ASQOL, and EQ-5D VAS. Our data support the importance of early diagnosis and treatment of axSpA Acknowledgement AbbVie funded the study, participated in its design/data collection/analysis/interpretation/writing publication Disclosure of Interest I. Olivieri Grant/research support from: Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merck Sharp & Dohme, Novartis, Pfizer, Roche, UCB, Consultant for: Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merck Sharp & Dohme, Novartis, Pfizer, Roche, UCB, Speakers bureau: Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merck Sharp & Dohme, Novartis, Pfizer, Roche, UCB, R. M. D9Attino Employee of: Abbvie, G. Gualberti Employee of: Abbvie, R. Merolla Shareholder of: Abbvie, Employee of: Abbvie

Background Psoriatic arthritis (PsA) is a systemic inflammatory disease mainly characterised by arthritis, dactylitis, enthesitis and skin involvement. Recently issued therapeutic guidelines (1) suggest that treatment decisions should be driven by as many manifestations of disease as possible. Objectives The aim of this study was to determine, in a multicentre Italian cohort of PsA patients, which clinical or patient reported disease outcome influence physician decision to change DMARD or biologic treatment. Methods 173 consecutive patients with PsA (103 males and 70 females, mean age 52±12 years) were enrolled in a longitudinal observational study. Seven tertiary referral clinics with expertise in PsA and SpA were involved. Detailed clinical examination, including ACR joint count, presence or absence of dactylitis and enthesitis, PASI, were recorded. Patient perception of disease was evaluated by means of global, joint and skin specific VAS scales. Detailed treatment information were also recorded. According to study protocol clinical assessment was performed at baseline and after twelve weeks in order to identify two distinct groups: changed and unchanged DMARD or biologic therapy. Results A higher number of tender joints (p Conclusions The results of this study, performed in an Italian cohort of PsA patients, suggest that rheumatologists, when they decide to change DMARD or biological treatment in PsA patients, are influenced by both objective evidence of musculoskeletal involvement and by patient reported global and joint outcomes. They do not seem to be influenced by the extent of skin dermatitis, possibly because of the generally low extent of skin involvement in PsA patient and/or because of a musculoskeletal orientated approach of rheumatologists. References Coates LC, Kavanaugh A, Mease PJ et al. Group for research and assessment of psoriasis and psoriatic arthritis: Treatment recommendations for psoriatic arthritis 2015. Arthritis Rheumatol. 2016 Jan 8. doi: 10.1002/art.39573. [Epub ahead of print] Disclosure of Interest None declared

Background Rheumatoid arthritis (RA) is characterized by an excess of cardiovascular diseases (CVD) risk, similar to type 2 diabetes (T2DM). A recent meta-analysis confirmed an elevated prevalence and incidence of T2DM in RA [1]. In this context, the necessity to recognize early T2DM in RA is driven by the evidence that CVD risk factors tends to act synergistically, thus increasing the risk conferred by each single factor [2]. However, it9s still difficult to select patients that need to underwent extensive diabetes investigation in the rheumatology setting. Objectives Aim of our work was to identify useful predictors of impaired glucose tolerance (IGT) and T2DM in RA patients with normal fasting glucose values in the rheumatology outpatient setting. Methods 100 RA patients satisfying ACR/EULAR 2010 criteria [3] were screened with FPG. Of these, 78 had normal ( ln (TG [mg/dL] x glucose [mg/dL]/2). The lipid accumulation product (LAP)[6] was calculated with the formula [WC (cm) – 58 for women or 65 for men] x TG (mmol/L). The visceral adiposity index (VAI) was calculated with the formula proposed by Amato et al [7]. Results Of the 78 RA patients recruited 57 were NGT, 18 were IGT and 3 were T2DM. After correction for age, sex, BMI, and systolic blood pressure, 2h-pG correlated with diseases duration (R=0.25, p=0.04), ESR (R=0.27, p=0.02), lnTG/HDLc (R=0.24, p=0.04), HOMA-IR (R=0.29, p=0.01), LAP (R=0.28, p=0.02) but not with lnCRP, lnTG, fasting glucose, uric acid, TyG index or VAI. In addition 2h-pG category correlated only with LAP (R=0.33, p=0.005). When A1c% was set as dependent variable, it correlated with lnTG (R=0.26, p=0.03), fasting glucose (R=0.29, p=0.0), uric acid (R=0.26, p=0.03), TyG index (R=0.25, p=0.04), LAP (R=0.30, p=0.01). Conclusions In RA patients with normal fasting glucose, LAP correlates significantly with 2hPG and predicts abnormal glucose tolerance. References Jiang, P., H. Li, and X. Li. Clin Exp Rheumatol, 2015. 33(1): p. 115–21. Baghdadi, L.R., et al. PLoS One, 2015. 10(2): p. e0117952. Aletaha, D., et al. Ann Rheum Dis, 2010. 69(9): p. 1580–8. Wallace, T.M., J.C. Levy, and D.R. Matthews. Diabetes Care, 2004. 27(6): p. 1487–95. Unger, G., et al. Endocrinol Nutr, 2014. 61(10): p. 533–40. Kahn, H.S. BMC Cardiovasc Disord, 2005. 5: p. 26. Amato, M.C., et al. Diabetes Care, 2010. 33(4): p. 920–2. Disclosure of Interest None declared

The traditional management of psoriatic arthritis (PsA) includes NSAIDs, corticosteroids and DMARDs. Advancement in the knowledge of the immunopathogenesis of PsA has been associated with the development of biologic agents which have revolutionized the management of the disease. Among biologics drugs, there are the 4 currently availablee anti-TNFα blocking agents (etanercept, infliximab, adalimumab and golimumab) which are more effective than traditional DMARDs on symptoms/signs of inflammation, quality of life, function, and in inhibiting the progression of the structural joint damage. Despite of the high cost, TNF inhibitors are costeffective on both the musculoskeletal and skin manifestations of psoriatic disease.

The case of a 49-year-old man affected by Behçet's syndrome (BS) without any clinical or radiological evidence of ankylosing spondylitis, exhibiting a peripheral enthesitis typical of seronegative spondyloarthropathy (SpA) is reported. The diagnosis of SpA is supported by computed tomographic evidence of sacroiliitis. This case confirms our hypothesis that patients with BS may have other forms of SpA than AS.

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

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.

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.

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.

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

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

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

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

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.