J.A. Pereira da Silva

Characterization of damage in Portuguese lupus patients: analysis of a national lupus registry.

Background: Although the survival rate has considerably improved, many patients with systemic lupus erythematosus (SLE) develop irreversible organ damage. Objectives: The objectives of this paper are to characterize cumulative damage in SLE patients and identify variables associated with its presence and severity. Methods: A cross-sectional analysis of SLE patients from the Portuguese Lupus register Reuma.pt/SLE in whom damage assessment using the SLICC/ACR-Disability Index (SDI) was available was performed. Predictor factors for damage, defined as SDI ≥ 1, were determined by logistic regression analyses. A sub-analysis of patients with severe damage (SDI ≥ 3) was also performed. Results: In total, 976 patients were included. SDI was ≥1 in 365 patients, of whom 89 had severe damage. Musculoskeletal (24.4%), neuropsychiatric (24.1%) and ocular (17.2%) domains were the most commonly affected. Older age, longer disease duration, renal involvement, presence of antiphospholipid antibodies and current therapy with steroids were independently associated with SDI ≥ 1. The subpopulation with severe damage had, in addition, a greater interval between the first manifestation attributable to SLE and the clinical diagnosis as well as and more frequently early retirement due to SLE. Conclusions: This large lupus cohort confirmed that demographic and clinical characteristics as well as medication are independently associated with damage. Additionally, premature retirement occurs more often in patients with SDI ≥ 3. Diagnosis delay might contribute to damage accrual.

Suppressing inflammation in rheumatoid arthritis: Does patient global assessment blur the target? A practice-based call for a paradigm change

In current management paradigms of rheumatoid arthritis (RA), patient global assessment (PGA) is crucial to decide whether a patient has attained remission (target) or needs reinforced therapy. We investigated whether the clinical and psychological determinants of PGA are appropriate to support this important role.

A complex case of hepatitis in a patient with systemic lupus erythematosus.

Abstract: Liver involvement in patients with systemic lupus erythematosus (SLE) is considered rare. Previous treatment with potentially hepatotoxic drugs or viral hepatitis have usually been implicated as the main causes of liver disease in SLE patients. On the other hand, even after careful exclusion of these aetiologies, the problem remains whether to classify the patient as having a primary liver disease with associated autoimmune clinical and laboratory features resembling SLE, such as autoimmune hepatitis, or as having liver disease as a manifestation of SLE.  We report the case of an elderly woman who presented with acute hepatitis, who had been diagnosed with SLE 14 years ago and who also had Sjo¨gren’s syndrome and anti-phospholipid’s syndrome for several years. The histology depicted chronic active hepatitis and, after drug-induced hepatitis and viral hepatitis were excluded, the serological and clinical features were shown to be typical of liver damage caused by SLE. The patient was treated with azathioprine 100mg/d and prednisone 30 mg/d. The clinical symptoms resolved in 10 days and the laboratory values were normal at the end of the first month of therapy. Prednisone was progressively reduced, during a period of 4 months, to 10 mg/d but azathioprine was kept to the same dose. One year after the diagnoses the patient is still in remission.  Although uncommon, hepatic involvement is well recognised in SLE. The interest of this case lies in the differential diagnosis and recognition of this condition, which deserves an agressive treatment.

Global functional status in rheumatoid arthritis: disease duration and patient age.

Abstract With the aim of clarifying whether patient age could be an additional explanation for the differences in the clinical expression of rheumatoid arthritis (RA) found in different populations, we evaluated the possibility of patient age being a significant factor associated with global functional status, independent from disease duration. Our present results suggest that both disease duration and patient age are major factors in the global functional status of patients with RA, and that patient age is particularly important when a subgroup of patients with more than 60 years of age and more than 20 years of disease duration is considered. These data are relevant when comparing two different RA groups: not only should we have a similar mean age and mean disease duration, but also the subgroups of patients more than 60 years of age and with more than 20 years of disease duration should correspond to equivalent proportions in the populations studied.

Spondyloarthritis and rheumatoid arthritis: different clinical manifestations, similar cytokine network

Background The reasons for the phenotypic differences between spondyloarthritis (SpA) and rheumatoid arthritis (RA) are still unclear. Slight divergences in cytokine networks driving the pathologies might contribute to the distinct clinical manifestations and may represent new treatment opportunities. Objectives The main goal of this work was to compare serum and synovial cytokines in established SpA and RA patients. Materials and methods The concentration of a panel of cytokines was measured in the serum of SpA and RA patients, as well as in synovial fluid from SpA, RA and osteoarthritis patients. Results The authors found that SpA and RA patients shared a similar pattern of cytokine concentration, with the exception of higher levels of interleukin-21 in the synovial fluid of RA patients. Conclusions Our results suggest that although SpA and RA are chronic inflammatory diseases with clearly distinct clinical manifestations, both pathologies share a similar cytokine profile, including Th17-related cytokines.

Disease amelioration in the K/BxN mouse model of spontaneous chronic arthritis after CD8 T cell depletion

CD8 T cells are part of the T cell pool infiltrating the rheumatoid synovial membrane. Moreover, CD8 T cells have been linked to the formation of synovial ectopic germinal centres in rheumatoid arthritis (RA), and they produce proinflammatory cytokines. Hence, CD8 T cells appear to be intimately involved in initiating and maintaining the chronic inflammation in the RA synovium. Nevertheless, studies on animal models of …

AB1364 Adaptation and validation of the rheumatoid arthritis quality of life (RAQOL) scale for portugal

Background Rheumatoid Arthritis (RA) is a chronic inflammatory disease that has a major impact on patients’ quality of life. The Rheumatoid Arthritis Quality of Life Questionnaire (RAQoL) is a patient-centric outcome measure, specific to RA. The measure has not previously been available for use with Portuguese RA patients. Objectives To produce a Portuguese version of the RAQoL that is acceptable to Portuguese patients and demonstrates sound psychometric properties. Methods The dual panel methodology was used to translate the UK RAQoL into Portuguese. This involved conducting a bilingual panel (providing the initial translation into Portuguese) followed by a lay panel (where items are assessed for comprehension and acceptability). Cognitive debriefing interviews were conducted with Portuguese RA patients to determine the face and content validity of the translated scale. A large-scale postal validation survey was carried out to establish the psychometric properties of the Portuguese RAQoL. The measure was administered on two occasions to RA patients, alongside a comparator instrument – the Nottingham Health Profile (NHP). Internal consistency was assessed using Cronbach’s alpha coefficient. Spearman’s Rank correlation coefficient was employed to assess test-retest reliability. Convergent validity was tested by correlating RAQoL scores with those on the NHP sections. Known group validity was assessed using non-parametric tests for independent samples. This involved determining the ability of the RAQoL to distinguish between patients that differed according to their self-perceived severity of RA and general health. Results The translation panels produced a Portuguese version of the RAQoL that was easily understood and considered natural by native speakers. Interviewees considered the new language version to be relevant and appropriate. One hundred and seventy-eight RA patients (82% female) took part in the postal validation survey with a mean age of 56.6 (range 25 to 79) years. The Portuguese RAQoL demonstrated excellent internal consistency (Cronbach’s α=0.95) and test-retest reliability (r=0.92), indicating that the measure produces low levels of random measurement error. RAQoL scores correlated most strongly with scores on the NHP Physical mobility scale (r=0.77) and showed moderately strong correlations with the Emotional reactions, Pain and Energy level section scores. Non-parametric tests for independent samples demonstrated significant differences in RAQoL scores between patients who differed according to their self-perceived RA severity (p<0.001) and general health (p<0.001). Conclusions The Portuguese version of the RAQoL was found to be a comprehensive, reliable and valid questionnaire. The new language version is recommended for use in routine clinical practice and for research purposes, to assess quality of life in Portuguese RA patients. Disclosure of Interest None declared

THU0489 The impact of calcium intake and physical exercise on peak bone mineral density

Background Regular exercise and adequate nutrition are frequently prescribed as strategies to optimise peak bone mass and maintain bone and muscle health throughout life. Objectives The aim of our work was to determine the relationship of clinically assessed milk intake and physical exercise with bone mineral density (BMD) in young adults. Methods This cross-sectional study included members of the general population aged between 20 and 30 years from the Portuguese cohort SAOL (individuals aged 18+years randomly selected from a local county of Coimbra, Portugal). No exclusion criteria were applied. Individuals were asked to describe their milk intake (up to 2 glass/day and 2+glass/day, corresponding to up to and more than 480 mg/day), regular physical activity (categorised as none-to-moderate and at least intense physical activity) and strenuous sports practice (up to 2 hour/week and 2+hour/week) from the age of 10 to 25. They underwent a Dual-energy x-ray absorptiometry (DEXA) of the lumbar spine (LS) and proximal femur (PF). Categorical data is presented as proportions/percentages and continuous variable as median ±standard deviation. Differences between groups were assessed by Mann–Whitney U test. Potential predictors of a higher BMD of PF and LS were identified using multiple linear regression analysis. P-values<0.05 were considered statistically significant. Results We included 259 individuals (mean age of 24.7±2.7 years, 60.6% being female). The majority (82.6%), described having a moderate regular physical activity (equivalent to working as a mailman), practicing strenuous sports at least 2 hours per week (81.1%) and ingesting at least two glasses of milk per day (83.4%). The current BMD of the PF and LS were 0.8±0.13 and 0.99±0.11, respectively. On univariate analysis, the only significant association related the PF BMD and milk intake (p=0.008). Multiple linear regression analysis showed that while physical activity and sports practice did not predict BMD values, milk intake persisted as a predictor of a higher BMD of PF (p=0.022), even after including other explanatory variables. No statistically significant predictors were found for BMD of the LS. Conclusions Our study showed that clinically assessed milk intake between the ages of 10 and 25 years, but not physical exercise, is a significant predictor of higher bone mineral density assessed by DEXA at the PF. These results do not exclude a positive impact of exercise upon peak bone mass, but suggest that its retrospective evaluation in a clinical setting should not be taken as reassurance that a good peak bone mass was achieved in early adulthood. Disclosure of Interest None declared

Encephalopathy with upper body hypertonia and myoclonus in patient with systemic lupus erythematosus and anti-CASPR2.

Systemic lupus erythematosus (SLE) may involve the nervous system but there are no specific biomarkers of neuroSLE. Limbic encephalitis has been rarely associated with SLE. We present a case of a 22-year-old black woman where typical SLE psychosis evolved to an encephalopathy with atypical features, normal MRI, electroencephalogram slowing and frontal and occipito-temporal hypometabolism on fluorodeoxyglucose positron emission tomography (FDG PET). Memory deficits, bizarre behaviour, psychosis, neuromyotonia and movement disorders have been described in autoimmune central nervous system disorders and associated with specific antibodies. Brain MRI may be normal and cortical brain hypometabolism on FDG PET scans has been reported. We have not found any report of limbic encephalitis or other SLE neurological manifestation associated to positive titres of anti-CASPR2 antibodies and this may warrant systematic investigation. In the rare cases of limbic encephalitis associated with SLE no specific antibodies were documented. Anti-CASPR2 antibodies have been associated not only with limbic encephalitis but also with neuromyotonia and Morvan syndrome. Although our patient had a specific pattern of tone abnormalities with an impressive cervical and upper limb hypertonicity and flaccid lower limbs, no myotonic discharges were found. We did not find any association between myoclonus and anti-CASPR2 antibodies. We cannot exclude that a non determined autoantibody could have played a role; however, clinical and FDG PET improvement supports an antibody-mediated injury, in this case of neuroSLE.

AB0581 Two years existence of reuma.pt/vasculitis – the portuguese registry of vasculitis

Background The vasculitides are a group of relatively rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is an electronic clinical record, created in 2008, which currently includes specific protocols for 11 diseases and >16000 patients registered from 79 national and international rheumatology centres. Since October 2014, a dedicated protocol to vasculitis has been created as part of the European Vasculitis Society initiative of having compatible European registries. Objectives To describe the structure of Reuma.pt/Vasculitis and characterize the patients registered over the last two years. Methods We developed a dedicated web-based software to enable prospective collection and central storage of anonymised data from patients with vasculitis. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, imaging and laboratory tests, outcome measures of prognosis, damage, disease activity and quality of life, and treatment were collected. We performed a cross-sectional descriptive analysis of all patients registered up to January 2017. Results A total of 492 patients, with 1114 visits, from 11 centres were registered in Reuma.pt/Vasculitis. The mean age was 53±20 years at last visit; 68% were females. The diagnoses followed the 2012 Chapel Hill Consensus nomenclature (Table 1). The most common diagnoses were Behçets disease (BD) (39%) and giant cell arteritis (GCA) (20%). Patients with BD met the International Study Group 1990 criteria, the International Criteria for BD 2006 and 2013 in 84%, 95% and 95% of cases, respectively. Patients with GCA met the 1990 American College of Rheumatology criteria in 95% of cases. Data on vascular ultrasound was available in 74% of patients; 73% compatible with the diagnosis. Assessment of the Birmingham Vasculitis Activity Score (BVAS) and Vasculitis Damage Index (VDI) was available for all vasculitides and the Five Factor Score calculation of survival rate for ANCA associated vasculitis (AAV) and polyarteritis nodosa (PAN). The mean BVAS at first visit was 18±7 for AAV and 15±9 for PAN; the mean VDI at last visit was 3±2 for AAV and 2±2 for PAN. Health related quality of life assessments (SF-36, EQD5, FACIT and HADS) were also collected. Treatment registry with the disease assessment variables shown in graphics was available for all patients; only 6% were under biologic treatment. Conclusions Reuma.pt/Vasculitis is a registry adapted for routine care, allowing an efficient data repository at a national level with the potential to link with other international databases. It facilitates research, trials recruitment, service planning and benchmarking. Disclosure of Interest None declared