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Background The Treat to Target strategy (T2T) for RA includes an early diagnosis and start of Disease Modifying Antirheumatic Drugs (DMARDs) targeted to achieve remission (or low inflammatory activity) defined by composite measures, and periodic adjustment of treatment in order to reach pre-defined goals. Objectives The aim of our work was to develop a tool to evaluate the quality of care in patients with RA according to the T2T strategy. Methods Firstly, the scientific committee of the project proposed a series of quality criteria about different aspects of RA managing, like time to diagnosis and to starting the first DMARD, establishment of therapeutic targets, therapy intensification according to a pre-defined target, optimal use of methotrexate, evaluation of comorbidities and the use of safety measures before biological therapy. In a second phase, this set of criteria was submitted for evaluation by a group of 20 experts who, in two Delphi rounds, scored the relevance of the criteria and the feasibility of being obtained by retrospective evaluation of clinical records. Those criteria with a median score ≥7.5 in the Delphi rounds, both for relevance and feasibility, an IQ range ≤2.5, and a degree of agreement (score ≥8) ≥80%, were selected. Results Initially, 37 quality criteria were proposed. They were submitted to a first Delphi round. Twelve of them needed a second round, since they did not reach consensus initially. After the analysis of the second round, a consensus was reached for 31 criteria. Some of these criteria were: a period

Background Primary Sjogren syndrome (pSS) is a systemic autoimmune disease affecting primarily the exocrine glandular system. More than half of the patients with 10 or more years of follow up show extra-glandular manifestations, which will be determinant for the prognosis. Several validated methods are available in order to assess activity in pSS, including the Sjogren9s Syndrome Disease Activity Index (SSDAI) and the EULAR Sjogren9s Syndrome Disease Activity Index ESSDAI). Objectives The aim of our study was to evaluate disease activity in a representative cohort of patients followed in rheumatology departments. Methods We conducted a multicenter descriptive transversal study of a cohort of Spanish pSS patients fulfilling European/American criteria. It is a randomised register of patients obtained from 33 spanish rheumatology departments. We used SSDAI and ESSDAI in every patient. Informed consent was obtained and local ethics committees approved the study. Variables were analysed by descriptive statistical methods, using means, medians, and rates, with their deviations and interquartile ranges (p25-p75). Results A total of 437 patients were included. Median score for SSDAI was 1 (0-3, p25-p75) and for ESSDAI was 2 (0-4, p25-p75). More than 95% of patients did not show fever, night sweating or weight loss. Twenty percent of them manifested weakness. Less than 5% of patients suffered glandular inflammatory disease or lymphadenopathy. Thirty two percent referred joint pain with stiffness but only 3% had true sinovitis. Fourteen patients (3%) showed cutaneous involvement. Fourteen percent of patients developed pleural involvement. High resolution CT was performed in 89 patients and showed interstitial disease in less than 4% percent of them. Serum creatinine was elevated in 3% of the cases. Seven patients developed tubular acidosis and 2 patients glomerular involvement. Less than two percent of patients had central nervous system involvement and 3% had peripheral nervous system involvement. Twenty seven percent of patients developed some kind of cytopenia: anaemia 14%, lymphopenia 14%, neutropenia 7% and thrombocytopenia, 9%. Twenty three percent had monoclonal component and/or hypoclomplementemia and/or hypergammaglobulinemia and/or high Ig G levels. Four percent showed cryoglobulinemia and/or hypergammaglobulinemia and/or high Ig G levels and/or recent hypogammaglobulinemia or a recent IgG levels fall. Conclusions pSS patients show low scores at the systemic damage indexes. Most affected domains were haematological, biological and articular. Considering parenchymatous and pleural involvement, lung is the organ most frequently involved. Disclosure of Interest None declared

Background There are scanty data about infection in Systemic Lupus Erythematosus (SLE) patients from large multicenter cohorts. Objectives To describe the prevalence of severe infection (SInf), investigate associated factors and clinical meaning in a large SLE cohort from Spanish Rheumatology Society Lupus Registry (RELESSER). Methods Patients with Sinf were compared with patients without SInf in terms of severity, damage, comorbidities and demographic characteristics (bivariate analysis and Cox regression to survival until the first infection). Results A total of 3.658 SLE patients included, 90% female, median age 32.9 years (DQ 9.7), 93% Caucasian and 5% Hispanic (Amerindian,mestizo). Mean follow-up (months) was 120.2 (SD: ±87.6). A total of 705 (19.3%) of the patients suffered ≥1 SInf, with an overall of 1.227 SInf. The density of incidence was 29.2 (95%CI: 27.6 - 30.9) infections/ 1000 patients-year. The survival until second infection was lower than survival until first infection (log rank p Conclusions SInf remains a frequent and potentially fatal complication of SLE and/or immunosuppressive therapies, and it9s a marker of more severe disease. Respiratory bacterial infections are the most common SInf in SLE, but bloodstream infections are the most common mortal ones. A previous infectious event seems to increment the risk of a subsequent infection in SLE. SInf are more common in male and Hispanics and is associated age, tobacco use and other co-morbidities. Antimalarials use exerts a time-dependent protective effect Disclosure of Interest None declared

Hypertrophic osteoarthropathy (HOA) is a condition characterised by digital clubbing, polyarthralgias, periostosis, occasionally arthritis,1,2 and cutaneous signs of autonomic disorders (sweating, flushing, blanching).3 HOA is classified as either primary (hereditary or occasionally idiopathic in adults) or secondary (often associated with neoplastic or infectious diseases).4 In the industrialised world, 90% of cases are associated with an underlying malignancy, usually a pulmonary neoplasm,1 but also cardiovascular and gastrointestinal diseases.4 Here, we describe the case of a patient presenting with HOA as the first manifestation of a gastrointestinal stromal tumour (GIST). A 47 year old white man consulted the rheumatologist in January 2005 with a 2 month history of fatigue and bilateral ankle and knee arthritis. Physical examination was normal, except for arthritis in knees and ankles. Blood and urine routine …

Current evidence indicates that regulatory T cells (Tregs) actively suppress autoreactive lymphocytes that escape central tolerance.1,2 Tregs are characterised by a high intensity CD25 constitutive expression3 on the surface of CD4 T lymphocytes, which distinguishes them from the non-regulatory CD4+CD25+ T lymphocytes.4 Because of their important role in the maintenance of tolerance, it has been suggested that Tregs are decreased in patients with systemic lupus erythematosus (SLE).5 We have quantified Tregs in patients with SLE and controls and evaluated their association with disease activity and treatment. Thirty three patients with SLE (26 women, 7 men) and 14 healthy volunteers matched for age and sex (11 women, 3 men) with a mean age of 39.9 and 36 years, respectively, were studied. All patients fulfilled at least four of the American College of Rheumatology 1982 revised criteria for the classification of SLE.6 Disease activity at the time of blood sampling was assessed using the SLE Disease Activity Index (SLEDAI).7 …

Pulmonary tuberculosis (TB) is a common worldwide lung infection. The radiological features show considerable variation, but in most cases they are characteristic enough to suggest the diagnosis. Classically, tuberculosis is divided into primary, common in childhood, and postprimary, usually presenting in adults. The most characteristic radiological feature in primary tuberculosis is lymphadenopathy. On enhanced CT, hilar and mediastinal nodes with a central hypodense area suggest the diagnosis. Cavitation is the hallmark of postprimary tuberculosis and appears in around half of patients. Patchy, poorly defined consolidation in the apical and posterior segments of the upper lobes, and in the superior segment of the lower lobe is also commonly observed. Several complications are associated with tuberculous infection, such as hematogenous dissemination (miliary tuberculosis) or extension to the pleura, resulting in pleural effusion. Late complications of tuberculosis comprise a heterogeneous group of processes including tuberculoma, bronchial stenosis bronchiectasis, broncholithiasis, aspergilloma, bronchoesophageal fistula and fibrosing mediastinitis. Radiology provides essential information for the management and follow up of these patients and is extremely valuable for monitoring complications.

Two cases of mucormycosis in patients with exclusively renal involvement and advanced HIV infection are reported. In both cases the clinical course was relatively benign; in one case a tendency towards regression with no specific treatment was observed, and in the other case the infection resolved after nephrectomy and antifungal therapy. Renal mucormycosis should be considered in the differential diagnosis of diffuse renal enlargement in HIV-infected patients.

Objectives To develop evidence-based recommendations for the detection and management of comorbidity in rheumatoid arthritis (RA) in daily practice. Methods We used a modified RAND/UCLA methodology and systematic review. Discussion groups established recommendations, which were prioritized, refined, and graded in a large group of users by a 2-round Delphi survey. Results The first 9/21 recommendations with agreement (A), and level of evidence (LE) are listed in Table 1. Conclusions We established recommendations that cover what, how, when and by whom to investigate and manage comorbidity in RA. Additional materials, such as tables and checklists, will facilitate their implementation. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3089

Background Osteoporosis is a well-known comorbidity in patients with rheumatoid arthritis (RA). A high percentage of patients with RA have low bone mineral density in axial and peripheral skeleton, which confers an increased fracture risk. In its further development, other risk factors such as age, menopause, disease activity and, especially, some of the drugs used for RA treatment, are involved. Objectives To study the prevalence of morphometric vertebral fractures (MVF) in a cohort of patients with RA and to identify demographic, analytical, densitometric and RA-related risk markers for vertebral fractures. Methods A cross-sectional cohort study in routine clinical practice conditions was designed. All patients fulfilled the ACR 2010 classification criteria for RA. We performed a questionnaire of fracture risk factors, an analytical study including serological bone markers (Procollagen Type I Intact N-terminal Propedtide and b-Cross Laps) and a hip and thoracolumbar spine bone densitometry. Patients filled out the Health Assessment Questionnaire (HAQ) and DAS28 was calculated. MVF was defined as a reduction of at least 20% in vertebral body height. A univariate analysis was performed using the presence of MVF as the dependent variable. Statistical significance was established by t-Student (parametric variables) and Mann-Whitney (nonparametric variables) tests. We considered significant a p Results We included 78 patients (77% women, mean age 59 years, duration of RA: 10 years). Vertebral fractures were found in 11 patients (14%). The presence of MVF was associated with a higher mean age (74 vs. 56, p Conclusions Around 15% of patients with RA may develop MVF. Age, duration of RA, densitometric osteoporosis, vitamin D levels, vitamin D supplements and bisphosphonates are associated with such fractures. Disclosure of Interest None Declared

Background Ultrasonography has been recently introduced in rheumatology. Since 1996 the Spanish Society Rheumatology has developed a training program and today, 15 years later, we feel that it is time to analyze its impact in clinical practice. Objectives To know the situation of ultrasonography in Spanish rheumatology and to sound out the opinion of Spanish rheumatologist about it. Methods A National survey was addressed to every rheumatology units in hospitals with at least 200 beds. The questionnaire studied: a) the availability and the model of the ultrasound equipment b) the degree of training, c) the purpose and areas of ultrasound examination, d) the usefulness of the technique, and e) the relevance of ultrasonography in the training of residents. Results 169 units of the 234 surveyed answered the questionnaire (72%). The availability of ultrasound equipment was high. In fact, 90% of the rheumatology units had ultrasound. The mean number of rheumatologist doing ultrasound in the respondent units was 1.2. Half of the units had a rheumatologist especially dedicated to performing ultrasound, in the other half there were several rheumatologists doing it. Training plans have enabled the incorporation of ultrasound to clinical practice, but there are points which could be improved and people with advanced o intermediate skill are still low, 34% of ultrasonographers. The utility in routine clinical practice was valued at 7.8 (scale 0-10), and ultrasound was integrated in taking diagnostic decisions as shows Figure 1. There is a broad consensus on the need to include ultrasonography in the official training of residents. The Health Administration is adding ultrasound to the portfolio of rheumatology specialty services. Figure 1. Answers about use of ultrasound (%) in clinical practice. Conclusions In just 15 years, ultrasonography has become available in 90% of rheumatology units from previous inexistence.Its use isextensive, integrated into clinical practice and linked to decision making processes. Training plans have been effective, but areas for improvement were identified, and there is a broad consensus on the need to integrate ultrasound in residents’ training program. Disclosure of Interest E. De Miguel Grant/Research support from: The study was supported by an unrestricted grant for Abbott., J. Andreu: None Declared, E. Naredo: None Declared, I. Moller: None Declared

Insomma se il modello non riesce a trasformare la realta, la realta dovrebbe riuscire a trasformare il modello…. Se le cose stanno cosi, il modello dei modelli dovra servire a ottenere dei modelli trasparenti, diafani, sottili como ragnatele; magari addiritura a dissolvere i modelli, anzi a dissolversi. (Italo Calvino, 1983) In summary, if the model does not succeed in transforming reality, reality must succeed in transforming the model…. If this is how things stand, the model of models must serve to achieve transparent models, diaphanous, subtle as spider webs, or perhaps even to dissolve models, or indeed to dissolve itself.

The ability to stimulate an Mls-1 mixed lymphocyte reaction (MLR) is predominantly expressed by low density B lymphocytes in the spleen and peritoneal cavity of normal adult mice, and is absent in splenic B cells 1 month after lethal irradiation and reconstitution from autologous bone marrow. Coreconstitution of these mice with normal syngeneic peritoneal cells restores the stimulatory potential of splenic B cells, but sorted CD5 + or CD5 − IgM + lymphocytes from peritoneum are equally good stimulators, suggesting that functional Mls-1 expression may require long life spans and selection. Bone-marrow-reconstituted DBA/2 mice that fail to express Mls-1 antigens in the periphery nevertheless maintain T-cell receptor Vβ 6 and 8.1 deletions among the newly formed T cells. These findings led us to directly investigate the Mls stimulatory ability of purified antigen-presenting cell populations inside the thymus. We report here that thymic B lymphocytes seem to represent the only intrathymic cell population able to stimulate Mls-1 MLR.

Recent observations suggest that minor cell populations located in the thymus play critical roles in T-cell development and T-cell repertoire selection (Moller, 1988). Although known for a long time (Miller and Osoba, 1967), little attention has been paid to intrathymic B lymphocytes (TBL). This subpopulation is found throughout life, and represents 0.1–1% of all “thymocytes” and up to 30–60% of the intrathymic cells left after negative selection with anti-Thy-1 antibodies (Isaacson et al., 1987; Miyaba-Inaba et al., 1983; Andreu et al., submitted). In the past, thymic B lymphocytes (TBL) have been considered as “recirculating” B cells, and their thymus localization as being devoid of particular significance. However, their presence at very early ontogenic stages, as well as the appearance of subclasses of antibody-secreting cells in newborn thymuses before any other lymphoid tissues (table I), and other findings discussed below suggest their specific localization and physiological role inside the thymus.

Previous observations indicate that "CD5 B lymphocytes" are preferentially clustered in gut-related mesenchymal areas, such as peritoneum, thymus and tonsils. We have now found that pleuropericardial spaces contain an homogeneous population of large-sized, noncycling, nonsecretory B cells, expressing very high levels of surface IgM, little or no IgD, Mac-1 and low levels of B220. This phenotype and the over-representation of some antibody clonotypes suggest that the pleuropericardial cavity contains a pure "CD5 B cell" population. In all mouse strains analyzed, however, many of these cells are CD5-. These findings, together with the common origin of peritoneum and pleural layers in the primitive coelomic cavity, suggest that such B cells differentiate locally from intraembryonic precursors; we propose to designate them as "coelomic", to distinguish them from "stromal", bone marrow-derived B cells.

A case of coexistence of adult onset Still's disease and idiopathic inflammatory myopathy is reported in a patient with antecedent of Graves-Basedow disease. Although myalgias are common during the flares of adult onset Still's disease, a review of literature only disclosed two previous cases of polymyositis associated to adult onset Still's disease. A high index of suspicion is needed in order to diagnose such a rare association which has relevant prognostic and therapeutic implications. Treatment with methotrexate was started in order to control myopathy, resulting in improvement of both polymyositis and adult onset Still disease. A possible role of methotrexate in the management of adult onset Still's disease is suggested.

Osteoid osteoma is a benign tumor that may occur in almost any part of any bone. Diagnosis can be difficult, particularly when it is intra-articular. In these cases, osteoid osteoma may present initially with symptoms suggestive of inflammatory arthritis, degenerative joint disease, neoplasm, or infection. We report a case of intra-articular osteoid osteoma in a teenager who presented with a monoarthritis of the elbow. The patient did not have a typical pattern of pain, did not have the typical relief with anti-inflammatory agents, and misinterpretation of the radiographs led to a delay in the diagnosis and definitive treatment. Surgical excision confirmed the diagnosis and resulted in resolution of the monoarthritis. Intra-articular osteoid osteoma must be taken into account in cases of unexplained chronic monoarthritis, with a sterile and relatively noninflammatory synovial fluid. A definitive diagnosis depends on surgical excision and histologic confirmation. Ahigh index of suspicion is necessary to make the diagnosis.