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Summary Vitamin D deficiency is prevalent worldwide, but its prevalence is unknown in adult Portuguese population. In Portugal, 66% of adults present Vitamin D insufficiency/deficiency. Winter, living in Azores, older age, and obesity were the most important risk factors. It highlights the need of strategies to prevent vitamin D deficiency in Portugal. Objective To estimate the prevalence and risk factors of vitamin D deficiency in the adult Portuguese population. Methods Adults (≥ 18 years old) from the EpiReumaPt Study (2011–2013) were included. Standardized questionnaires on sociodemographic and lifestyle features were obtained. Serum 25-hydroxyvitamin D [25(OH)D] concentrations were evaluated using ADVIA Centaur VitD competitive immunoassay (Siemens Healthineers) in 2015–2017 as 25 (OH)D Level 0: ≤ 10 ng/mL; Level 1: 11–19 ng/mL; Level 2: 20–29 ng/mL, and Level 3: ≥ 30 ng/mL. Weighted multinomial regression analysis was conducted to evaluate the association between socio-demographic and lifestyle variables and vitamin D status. Results Based on weighted analysis, the estimated prevalence of levels of 25(OH)D ≤ 10, < 20, and < 30 ng/mL was 21.2, 66.6, and 96.4%, respectively. The strongest independent predictors of serum 25 (OH)D ≤ 10 ng/mL were living in the Azores archipelagos (OR 9.39; 95%CI 1.27–69.6) and having the blood sample collection in winter (OR 18.53; 95%CI 7.83–43.87) or spring (11.55; 95%CI 5.18–25.74). Other significant predictors included older age (OR 5.65, 95%CI 2.08–15.35), obesity (OR 2.61; 95%CI 1.35–5.08), current smoking (OR 2.33; 95%CI 1.23–4.43), and female gender (OR 1.9, 95%CI 1.1–3.28). Conversely, physical exercise (OR 0.48, 95%CI 0.28–0.81) and occasional alcohol intake (OR 0.48, 95%CI 0.29–0.81) were associated with a lower risk of 25(OH)D ≤ 10 ng/mL. Conclusion Vitamin D deficiency/insufficiency [25(OH)D < 20 ng/ml] is highly prevalent in Portugal, affecting > 60% of all Portuguese adults, with strong geographical and seasonal variation. This study highlights the need to critically assess the relevance of vitamin D deficiency as a public health problem and the urgent need for a wide and scientifically robust debate about the most appropriate interventions at the individual and societal levels. info:eu-repo/semantics/publishedVersion

OBJECTIVES: To describe differences in clinical presentation between men and women in a large group of patients with early (

The study rationale was to provide a detailed overview of the costs, quality of life and mortality of hip fractures in Portugal. Mean individual fracture-related costs were estimated at 13,434 [12,290; 14,576] for the first year and 5985 [4982; 7045] for the second year following the fracture. INTRODUCTION: Osteoporotic fractures represent a remarkable burden to health care systems and societies worldwide, which will tend to increase as life expectancy expands and lifestyle changes favour osteoporosis. The cost-effectiveness evaluation of intervention strategies demands accurate data on the epidemiological and economical reality to be addressed. METHODS: Information was collected retrospectively on consumption of resources and changes in quality of life attributable to fracture as well as mortality, regarding 186 patients randomly selected to represent the distribution of hip fractures in the Portuguese population, in terms of gender, age and geographical provenience. Data were cross-tabulated with socio-demographic variables and individual resource consumption to estimate the burden of disease. A societal perspective was adopted, including direct and indirect costs. Multivariate analyses were carried out to assess the main determinants of health-related quality of life (HrQoL). RESULTS: Mean individual fracture-related costs were estimated at 13,434 [12,290; 14,576] for the first year and 5985 [4982; 7045] for the second year following the fracture. In 2011 the economic burden attributable to osteoporotic hip fractures in Portugal could be estimated at 216 million. Mean reduction in HrQoL 12 months after fracture was estimated at 0.34. Regression analysis showed that age was associated with a higher loss of HrQoL, whereas education had the opposing effect. We observed 12 % excess mortality in the first year after hip fracture, when compared to the gender and age-matched general population. CONCLUSIONS: Results of this study indicate that osteoporotic hip fractures are, also in Portugal, despite its low incidence of fractures and cost per event, associated with a high societal burden, in terms of costs, loss in HrQoL and mortality. These data provide valuable input to the design and selection of fracture prevention strategies.

Herein, we describe a 44-year-old female diagnosed with histologically proven coexistence of primary Sjögren's syndrome and sarcoidosis with pulmonary and muscular involvement. The differential diagnosis may be difficult, but this is not an exceptional case, which highlights the need to critically revise the consideration of sarcoidosis as an exclusion for primary Sjögren's syndrome, as established in current classification criteria.

Low- to medium-dose glucocorticoids have been shown to have not only anti-inflammatory but also disease-modifying properties in rheumatoid arthritis. The evidence for the benefit of its early use in combination with disease-modifying antirheumatic drugs underlines the need for a close evaluation of their risk-benefit ratio. Over time, numerous myths and fears about glucocorticoid toxicity in rheumatoid arthritis have arisen from observational studies, and many concerns have been unduly extrapolated from observations with higher-dose treatment. Furthermore, we cannot exclude the possibility of a powerful effect of bias by indication in these studies. Low- to medium-dose glucocorticoid regimens continued to be evaluated in randomized clinical trials, particularly in early disease, but these studies also have relevant methodological limitations in assessing safety, particularly due to small size and/or short duration. At present, the evidence on which to support clear recommendations about glucocorticoid toxicity remains remarkably weak. A large prospective pragmatic trial dedicated to the toxicity of low-dose glucocorticoids is dearly needed. Meanwhile, adherence to recommendations on standardized methodologies for registration and report of glucocorticoid adverse events is essential for improving our knowledge and competence in the best management of these important medications.

Objective. SLE has a relapsing-remitting course with disease activity flares over time. This study aims to identify clinical predictors of SLE flares.Methods. This prospective cohort study over 24 months included all SLE patients on follow-up at one academic lupus clinic. Flare was defined as an increase in SLEDAI-2K score ≥4 points. Baseline clinical and demographic parameters were compared using survival analysis for time-to-flare outcome with univariate log-rank tests. Variables with significant differences were further evaluated as predictors with multivariate Cox regression models adjusting for potential confounding or contributing factors and hazard ratio (HR) calculation.Results. A total of 202 SLE patients were included. Over the follow-up period, 1083 visits were documented and 16.8% of patients presented with flares. In multivariate analysis, the following parameters emerged as flare predictors: SLE diagnosis up to 25 years of age (HR = 2.14, P = 0.03), lupus nephritis previous to baseline visit (HR = 4.78, P < 0.0001) and immunosuppressor treatment for severe SLE (HR = 3.22, P < 0.001). Baseline disease activity, disease duration and treatment with prednisone or HCQ were not predictive factors.Conclusion. Patients with an SLE diagnosis before age 25 years, lupus nephritis or immunosuppressor treatment for severe SLE present greater HRs for flares, suggesting the need for tighter clinical monitoring. Current immunosuppressive strategies seem to be inefficient in providing flare prevention.

This study was designed to investigate the functional heterogeneity of human Th17 and how their plasticity shapes the nature of immune cell responses to inflammation and autoimmune diseases, such as systemic lupus erythematosus (SLE). We evaluated functional Th17 cell subsets based on the profile of cytokine production in peripheral blood (PB), bone marrow aspirates (BM) and lymph node biopsies (LN) from healthy individuals (n = 35) and PB from SLE patients (n = 34). Data were analysed by an automated method for merging and calculation of flow cytometric data, allowing us to identify eight Th17 subpopulations. Normal BM presented lower frequencies of Th17 (p = 0.006 and p = 0.05) and lower amount of IL-17 per cell (p = 0.03 and p = 0.02), compared to normal PB and LN biopsies. In the latter tissues were found increased proportions of Th17 producing TNF-α or TNF-α/IL-2 or IFN-γ/TNF-α/IL-2, while in BM, Th17 producing other cytokines than IL-17 was clearly decreased. In SLE patients, the frequency of Th17 was higher than in control, but the levels of IL-17 per cell were significantly reduced (p

Clinical trials published over the last 5 years support the main conclusion of a comprehensive review on glucocorticoid safety published in 2006: there is little if any solid evidence to support the fear that low-dose glucocorticoids are associated with significant toxicity when used appropriately in inflammatory rheumatic diseases. In fact, most of the recent randomised-controlled research underlines the influence of the underlying inflammatory process in the occurrence of 'adverse events' such as osteoporosis, fractures, hypertension and glucose intolerance. This 'confounding by indication' is inherent to the field and questions the validity of the observational data, that seems to drive currently common concepts about low-dose glucocorticoid toxicity. Decisive conclusions cannot, in any case, be achieved at this stage because the clinical trials available are of limited duration and dimension and have not been designed specifically to address toxicity. Toxicity with low-dose glucocorticoids needs to be kept under careful clinical surveillance while we expect such trials to be produced. Meanwhile, the risks of stopping these medications, even on longstanding well controlled disease, need also to be considered, as underlined by withdrawal trials recently published.

OBJECTIVES: To develop Portuguese evidence-based recommendations for the use of methotrexate (MTX) in daily clinical practice in rheumatic disorders. METHODS: The Portuguese project was integrated in the multinational 3E Initiative (Evidence, Expertise, Exchange) 2007-2008 where a total of 751 rheumatologists from 17 countries have participated. Ten clinical questions concerning the use of MTX in rheumatic diseases were formulated and the Portuguese group added three more questions. A systematic literature search in Medline, Embase, Cochrane Library and 2005-2007 ACR/EULAR meeting abstracts was conducted. Selected articles were systematically reviewed and the evidence was appraised according to the Oxford Levels of Evidence. In Portugal, a national meeting was held in Obidos on February 15th and 16th, 2008, involving 50 rheumatologists who discussed and voted by Dephi method the recommendations. Finally, the agreement among the rheumatologists and the potential impact on their clinical practice was assessed. RESULTS: Thirteen national key recommendations on the use of MTX were formulated: work-up before starting MTX, optimal dosage and route of administration, use of folic acid, monitoring, management of hepatotoxicity, long-term safety, mono versus combination therapy, management in the peri-operative period, during infections, before/during pregnancy and after clinical remission, screening and treatment of tuberculosis and the role of MTX as a steroid-sparing agent in rheumatic diseases. DISCUSSION: The Portuguese recommendations for the use of MTX in daily clinical practice were developed, which are evidence-based and supported by a panel of 50 rheumatologists, enhancing their validity and practical use. This project was integrated in a multinational initiative that led to the recent publication of ten multinational recommendations which differ from ours in some specific aspects.

Introdução: A diabetes mellitus tipo 2 constitui actualmente um grave problema de saúde pública, pela sua elevada incidência e prevalência. A evolução do conhecimento científico conduziu à introdução recente de novos fármacos, assim como à revisão das recomendações internacionais para o tratamento da hiperglicémia e dos objectivos terapêuticos a atingir. No dia 13 de Dezembro de 2008, realizou-se no Palace Hotel do Buçaco um Workshop Multidisciplinar subordinado ao tema “Diabetes e Osteoporose”, dirigido a internos de Endocrinologia, Medicina Interna e Reumatologia. Foram discutidos de forma interactiva dois casos clínicos, um na área da Diabetologia e outro de Reumatologia. Apresentamos os pontos fundamentais da discussão do caso clínico de Diabetes. Caso Clínico: Doente do sexo feminino, 52 anos, obesa, observada em Consulta de Diabetes por apresentar A1c de 8%, apesar da dieta e da medicação (metformina).Apresentava uma tensão arterial (TA) de 150/95 mmHg, colesterol total (CL–T) 250 mg/dL, triglicerídeos (TG) 220 mg/dL, HDL 35 mg/dL e LDL 160 mg/dL. Questão: Quais as medidas terapêuticas a adoptar? Passados 6 meses, apresentava HbA1c 7,8%, CL-T 200 mg/dL,TG 190 mg/dL, HDL 40 mg/dL, LDL 130 mg/dL e microalbuminúria 30 mg/24h. Questão: Quais as modificações terapêuticas a instituir? Um ano depois, regressa à consulta, medicada com prednisolona por patologia reumatismal, com glicémias de 300 mg/dL em jejum e A1c 10,2%. Questão: Qual a estratégia terapêutica a adoptar? Dois anos mais tarde, a doente apresenta A1c 12%, CL-T 280 mg/dL, HDL 30 mg/dL, LDL 180 mg/dL e TG 200 mg/dL,TA 160/95 mmHg. Antecedentes de Acidente Vascular Cerebral, encontrando-se novamente medicada com antidiabéticos orais. Questão: Perante este quadro, o que prescrever? Conclusões: A diabetes mellitus implica uma abordagem multifactorial, dirigida à hiperglicemia e aos restantes factores de risco associados: dislipidemia, hipertensão arterial, hipercoagulabilidade, insulinorresistência e obesidade. De acordo com a Sociedade Europeia para o Estudo da Diabetes e com Sociedade Portuguesa de Diabetologia, o objectivo é alcançar uma A1c inferior a 6,5%, no sentido de prevenir e evitar a progressão das complicações. Os novos antidiabéticos orais conduziram a um aumento das opções terapêuticas disponíveis e implicaram uma revisão das recomendações internacionais. info:eu-repo/semantics/publishedVersion

OBJECTIVES: Although several algorithms have been proposed to select postmenopausal women PMW for dual-energy x-ray absorptiometry DEXA measurements information on their utility in clinical practice is scarce. Our aim was to assess the utility and the economic repercussion of the use of five of these algorithms in Portuguese postmenopausal women. METHODS: We included 588 PMW and selected five simple algorithms ORAI ABONE Body Weight Criterion OSTA and a modified version of OSTA (OST). Sensitivities specificities predictive values areas under the receiver operating characteristic curve (AUROC) and economic estimates were computed. RESULTS: Sensitivities ranged between 71.2%-80.8% and AUROC between 0.611-0.674. In PMW aged > or = 65 years (Y) the use of any of the algorithms would cause extra costs or a residual saving. In PMW aged > or = 55 and < 65Y considering total savings ABONE had the best performance but considering savings per preventable fracture, ORAI assumed the lead, followed by BWC. In the age group > or = 40 and < 55Y, the most profitable option considering total savings would be not doing DEXA to anyone; considering savings per preventable fracture, BWC figures as the most useful. CONCLUSIONS: This study provides evidence for the validity of all the selected tools as useful algorithms to select PMW for DEXA. On the basis of our results and considering the importance of simplicity in the applicability of an algorithm we would suggest the following strategy in Portuguese PMW: 1) Aged > or = 65Y: perform DEXA irrespective of other risk factors. 2) Aged < 65Y: perform DEXA if body weight < 70Kg.

OBJECTIVES: To determine the frequency of ovarian failure in pre-menopausal women after cyclophosphamide (cyc) treatment for systemic lupus erythematosus (SLE); identify risk factors for this complication; estimate the occurrence and viability of pregnancy during and after treatment. METHODS: Review of the data of women treated with intravenous cyc in the department of Rheumatology of Hospitais da Universidade de Coimbra, updated by interview. Information on demographic features; gynaecologic and obstetrical history; characteristics of the disease; duration and side effects of treatment were obtained. Ovarian failure was defined as a lack of menses for, at least, four months and the diagnosis was confirmed by hormonal measurements. RESULTS: Nineteen pre-menopausal women were treated with intravenous cyc in our department. The mean age at the time of cyc initiation was 28.4 years. Lupus nephritis was the most common indication for cyc treatment (89.5%). The mean number of pulses was 9.3 over a period of 16.8 months. The mean cumulative dosage was 6.973 mg. Three patients developed ovarian failure. Those women were older than the others (P=0.0016). One patient became pregnant while on treatment. Two women delivered healthy children after cyc withdrawal. CONCLUSION: Ovarian failure developed in 15.8% of our patients. As described in the literature, the age at cyc initiation appears to be a determinant risk factor. Pregnancy may occur during cyc therapy, and thus, an effective contraception is mandatory. After cyc withdrawal, pregnancy is possible with a favourable outcome.

OBJECTIVE: To develop evidence-based recommendations for the management of systemic glucocorticoid (GC) therapy in rheumatic diseases. METHODS: The multidisciplinary guideline development group from 11 European countries, Canada and the USA consisted of 15 rheumatologists, 1 internist, 1 rheumatologist-epidemiologist, 1 health professional, 1 patient and 1 research fellow. The Delphi method was used to agree on 10 key propositions related to the safe use of GCs. A systematic literature search of PUBMED, EMBASE, CINAHL, and Cochrane Library was then used to identify the best available research evidence to support each of the 10 propositions. The strength of recommendation was given according to research evidence, clinical expertise and perceived patient preference. RESULTS: The 10 propositions were generated through three Delphi rounds and included patient education, risk factors, adverse effects, concomitant therapy (ie, non-steroidal anti-inflammatory drugs, gastroprotection and cyclo-oxygenase-2 selective inhibitors, calcium and vitamin D, bisphosphonates) and special safety advice (ie, adrenal insufficiency, pregnancy, growth impairment). CONCLUSION: Ten key recommendations for the management of systemic GC-therapy were formulated using a combination of systematically retrieved research evidence and expert consensus. There are areas of importance that have little evidence (ie, dosing and tapering strategies, timing, risk factors and monitoring for adverse effects, perioperative GC-replacement) and need further research; therefore also a research agenda was composed.

BACKGROUND: A 31-year-old woman presented with a 5-year history of painful ulcerations, palpable purpura, porcelain-white atrophic scars of the malleolar region and dorsal aspect of the feet, livedo reticularis on the limbs, arthralgia, xerophthalmia, and xerostomia. METHODS: Skin biopsy revealed vessel wall hyalinization and thrombosis of the microvasculature with a very scarce dermal inflammatory infiltrate. Biopsy of the oral mucosa showed mononuclear infiltration of an intralobular duct of a salivary gland. RESULTS: Laboratory studies, including autoantibodies and inflammation markers, were normal, except for a positive rheumatoid factor. Coagulation screening revealed C677T methylenetetrahydrofolate reductase (MTHFR) mutation, with a normal serum homocysteine. The patient was treated with oral methylprednisolone (32 mg/day with progressive reduction) and enoxaparin (20 mg/day subcutaneously), with complete ulcer healing within 4 months. CONCLUSION: Livedoid vasculitis or vasculopathy has not been referred to previously in association with Sjögren's syndrome, but may be associated with other autoimmune disorders and anomalies of coagulation, namely factor V Leiden mutation, protein C deficiency, and MTHFR mutation, associated or not with hyperhomocysteinemia, a condition that seems to confer an increased risk of recurrent arterial and venous thrombosis. We stress the importance of anticoagulant therapy for ulcer healing and for the prevention of other thrombotic events.

A 52-year-old man, with the diagnosis of Ankylosing Spondylitis, since the age of 22, was admitted due to progressive neurological symptoms that had started two years before: paresthesias, impaired sensation and muscle weakness of the lower limbs; burning abdominal and lumbar pain; together with bladder, bowel and sexual dysfunction. Imaging investigations revealed severe lesions from D1 to L5: epidural calcification; spinal cord compression and syringomyelia. Based on this case, the authors review the neurological complications of Ankylosing Spondylitis.

Adverse effects of glucocorticoids have been abundantly reported. Published reports on low dose glucocorticoid treatment show that few of the commonly held beliefs about their incidence, prevalence, and impact are supported by clear scientific evidence. Safety data from recent randomised controlled clinical trials of low dose glucocorticoid treatment in RA suggest that adverse effects associated with this drug are modest, and often not statistically different from those of placebo.

We collected population-based young normal hip and spine BMD data from 17 centres across Europe to assess between centre differences and to compare reference values with the US NHANES-III data. There was strong evidence of between country heterogeneity, but not between centres within countries. Hip BMD mean values were lower in European women, but SD's differed little from the NHANES-III USA results in both sexes. It may be necessary to adjust NHANES-III based T-scores by adding/subtracting a country-specific adjustment factor. INTRODUCTION: It remains unclear whether young normal BMD reference values specific to an American population can be validly used for T-score calculation in Europeans. METHODS: We collected population based BMD data from 1163 men and 329 women aged 19-29 years from 17 centres across Europe to compare mean and SD values with the NHANES-III study USA results. BMD(g/cm2) was measured at the hip and spine using DXA densitometers cross-calibrated with the European Spine Phantom (ESP). The only exclusions were for technically inadequate scans. A linear regression model was used to derive reference values. To allow for direct comparison with published NHANES III study data, the cross-calibrated BMD values were converted using the ESP equations to Hologic QDR 1000 units. RESULTS: In men, the overall mean(SD) BMD values expressed in Hologic-QDR1000 units of measurement, were: femoral neck 0.912(0.132); trochanter 0.793(0.124); and L2-L4 spine 1.027(0.123). The respective estimates in women were: 0.826(0.115); 0.670(0.093); and 0.983(0.107). However the I2 statistic for heterogeneity indicated moderate to strong evidence of between-centre heterogeneity. There was, however, no significant heterogeneity observed between centres within countries, suggesting that this variation arose from national differences. Compared to the NHANES III population-based US data, the mean values in women were significantly lower at both sites due to some lower national European means. However, at all sites and in both sexes the SD's were very similar between the US and Europe. There was some evidence that recruiting volunteers resulted in biased values in women. CONCLUSION: Our T-score normal values for the lumbar spine (L2-L4) should be more reliable for spine-specific risk assessment than some non-representative normal ranges, and should be evaluated for that purpose in Europe. If T-scores are to be used to compare individual data with ranges seen in normal young subjects of the same nationality, it may be necessary to adjust femoral NHANES III-based T-scores by adding (or subtracting) a country-specific adjustment factor. In risk assessment it is probably sufficient to use NHANES III-based hip T-scores, as supplied for the hip by densitometer manufacturers, interpreting them in light of recent international meta-analysis data on the relationship between BMD and fracture risk.

Soft tissue rheumatism includes a wide spectrum of common lesions of the tendons, enthesis, tendon sheaths, bursae, ligaments and fasciae as well as nerve compression syndromes. Studies on the pathogenesis of these lesions do not support a major role for inflammation, thus questioning the rationale for glucocorticoid injections. This chapter reviews current indications for local glucocorticoid injections and available evidence on its efficacy, as well as contraindications and potential risks. Randomised controlled studies of good methodological quality are rare and there is limited scientific evidence to support the superiority of glucocorticoid injections over alternative treatments. The basic principles of the glucocorticoid injection method are outlined, together with a description of the practical procedure for the more common conditions.

As doenças reumáticas são frequentemente acompanhadas de manifestações do foro emocional e afectivo que podem alterar de forma dramática a apresentação clínica e, por essa via, o diagnóstico e orientação terapêutica. Noutros casos, as manifestações músculo-esqueléticas podem ser vistas como resultado de somatização de conflitos emocionais. A dicotomia físico/psíquico é fortemente artificial. A abordagem das doenças reumáticas numa perspectiva bio-psico-social, bastante mais consentânea com a realidade, é a que garante melhores resultados numa perspectiva global do doente, isto é, da sua qualidade de vida. A investigação biomédica recente na fronteira entre a mente e os sistemas endócrino e imunológico vieram revelar vias bioquímicas insuspeitadas que permitem começar a desenhar a profunda interacção existente entre estas esferas. É este o objectivo deste artigo.

Gender and sex hormones are strongly related to the incidence and progression of autoimmune rheumatic diseases. Although sex steroids have been shown to have direct effects on the immune system, their influence in vivo may be mediated via interactions with third party systems including the hypothalamic-pituitary-adrenal axis. Such interactions are well demonstrated in experimental animals. In humans, there is increasing, although indirect, evidence that these interactions also occur. Possible interactions at the cell and gene level, with mutual antagonism or synergy between cortico- and gonadal steroids, open new exciting hypotheses that await clarification.

In rheumatology and other medical specialties there is a discrepancy between the widespread use and the imprecise designation of glucocorticoid treatment regimens. Verbal descriptions of glucocorticoid treatment regimens used in various phases of diseases vary between countries and institutions. Given this background, a workshop under the auspices of the EULAR Standing Committee on International Clinical Studies including Therapeutic Trials was held to discuss this issue and to seek a consensus on nomenclature for glucocorticoid treatment. This report summarises the panel's discussion and recognises that answers derived from consensus conferences are not definitive. Nevertheless, recommendations on glucocorticoid treatment are presented that (1) reflect current and best knowledge available and (2) take into account current clinical practice. A question-answer rationale presentation style has been chosen to convey the messages, to summarise the meeting in a readable format, and to avoid dogmatism.

Behçet disease is a recurrent systemic vasculitis of unknown etiology, that involves vessels of nearly all sizes and types. Because of this, disease manifestations can occur at many sites throughout the body. Central nervous system (CNS) involvement may be parenchymal or nonparenchymal and has a global prevalence that ranges from 3% to 10%. Main signs of CNS involvement are pyramidal and those resulting from brain stem lesions. Aseptic meningitis, mental changes, sphincter disturbances, pseudobulbar syndrome, and deep sensory abnormalities may be seen. Analysis of cerebrospinal fluid, computed tomography (CT), magnetic resonance imaging (MRI), single-photon emission computed tomography (SPECT) and brain angiography offer assistance in the diagnosis. The course of disease can be primary progressive, secondary progressive or have a relapsing-remitting profile. Boluses of methylprednisolone for three days followed by cyclophosphamide are the treatment of choice. This papers discusses these aspects of neuro-Behcet on the basis of complex clinical case

As doenças infecciosas continuam a ser uma causa importante de morbilidade e de mortalidade nos doentes reumáticos imunodeprimidos. Assim a prevenção das infecções e a avaliação da importância da vacinação devem ser uma prioridade na prática clínica. Neste artigo são apresentadas as principais indicações de vacinação em doentes reumáticos, actualmente recomendadas, salientando a sua eficácia, segurança, doses óptimas, esquema de posologia e factores adjuvantes. O uso de vacinas vivas está contra-indicado nos doentes reumáticos imunodeprimidos, e só deve ser administradas após 3 meses de suspensão da terapêutica imunosupressora. A reintrodução da terapêutica imunossupressora deverá ser efectuada 2 semanas após o uso de uma vacina viva. Se o doente for imunizado durante a toma de imunossupressores pode não atingir uma resposta imune adequada, devendo-se assumir que não está imunizado e repetir a imunização após 3 meses de suspensão da terapêutica. As vacinas que usem uma toxina ou um microorganismo inactivo estão indicadas e na maioria dos casos são bem toleradas e eficazes. As vacinas do tétano, difteria, pneumococica e influenza são eficazes e o seu potencial benefício e de segurança justificam o seu uso nos doentes reumáticos imunodeprimidos.

Genetic hemochromatosis is not a rare disease and represents a frequently underestimated cause of arthropathy. Joint involvement is one of the most frequent manifestations of the disease and presents typical clinical and radiological features that strongly suggest the diagnosis. Joint complaints are often the first clinical manifestation of GH. Their identification may be crucial to establish the diagnosis in the pre-cirrhotic phase and to institute appropriate therapy to prevent organ damage and associated mortality. Recent identification of the genetic defect responsible for the disease is leading to new insights into the pathogenesis of GH and the associated arthropathy.

Estrogen deficiency is the main cause of post-menopausal osteoporosis and hormone replacement therapy is consensually regarded as the first line choice for its prevention. Hormone-replacement has also been shown to prevent bone loss associated with rheumatoid arthritis (RA) and corticotherapy. The rationale for its use in RA is reinforced by evidence of beneficial effects on disease activity. Estrogens also have bone-protective effects in males. C-19 progestagens seem to potentiate the skeletal effects of estrogens whereas C-21 derivatives appear to have no significant influence. Progestagens may be particularly useful in the prevention of steroid-induced osteopenia. Testosterone is an effective treatment for male osteoporosis associated with hypogonadism and is also promising in the treatment of osteoporosis in aged eugonadal males. The association of this hormone potentiates the effects of estrogens on bone mineral density in post-menopausal women. However, anabolic androgens have been preferred in this condition, due to a lower androgenizing effect. They represent a valid therapeutic alternative, particularly in cases of cortical osteoporosis with low bone turnover and in the aged and frail. In general, the different modalities of hormonal therapy clearly deserve a higher degree of preference in the treatment and prevention of osteoporosis than common present practice in our country. It is time to reassess this matter, clear unfounded fears and reinforce the physiological and scientific foundations of our therapeutic options for osteoporosis.

A core operational definition of active and healthy aging (AHA) is needed to conduct comparisons. A conceptual AHA framework proposed by the European Innovation Partnership on Active and Healthy Ageing Reference Site Network includes several items such as functioning (individual capability and underlying body systems), well-being, activities and participation, and diseases (including non-communicable diseases, frailty, mental and oral health disorders). The instruments proposed to assess the conceptual framework of AHA have common applicability and availability attributes. The approach includes core and optional domains/instruments depending on the needs and the questions. A major common domain is function, as measured by the World Health Organization Disability Assessment Schedule 2.0 (WHODAS 2.0). WHODAS 2.0 can be used across all diseases and healthy individuals. It covers many of the AHA dimensions proposed by the Reference Site network. However, WHODAS 2.0 does not include all dimensions proposed for AHA assessment. The second common domain is health-related quality of life (HRQoL). A report of the AHA questionnaire in the form of a spider net has been proposed to facilitate usual comparisons across individuals and groups of interest. (C) 2015 Published by Elsevier Inc. on behalf of AMDA - The Society for Post-Acute and Long-Term Care Medicine.