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Cytomegalovirus (CMV) infection is a common and typically benign disease in immunocompetent individuals. However, immunocompromised patients are at a greater risk of reactivation, leading to more severe outcomes. Patients with rheumatic diseases have a particularly high risk of opportunistic infections due to both the inherent immunosuppressive state conveyed by the disease itself and the use of potent immunosuppressant drugs, such as glucocorticoids, cyclophosphamide, and rituximab. Limited data are available regarding prophylactic or preemptive treatment of CMV infection in patients with rheumatic diseases. In this article the authors present two cases of rheumatic conditions complicated by CMV infection. The first case describes a patient with eosinophilic granulomatosis with polyangiitis, previously treated with glucocorticoids and cyclophosphamide, who developed CMV colitis with bowel perforation. The second case involves a woman with systemic lupus erythematosus who was diagnosed with CMV meningitis. Both cases reinforce the importance of establishing guidelines for surveillance and prophylaxis of CMV infection in these patients.

Aim: To estimate the disease specific prevalence of undiagnosed rheumatic and musculoskeletal diseases (RMDs) in Portugal and determine if people with undiagnosed RMDs have worse quality of life, physical function and higher health resources consumption, than people without RMDs., Methods: A subgroup analysis of EpiReumaPt was made that included all participants≥18 years evaluated by a rheumatologist. Participants were stratified into three groups: undiagnosed RMDs; previously diagnosed RMDs; non-RMDs. A descriptive analysis of the three groups was performed. To estimate the prevalence of undiagnosed RMDs, weighted proportion were computed considering the sample design. The three groups were compared (Undiagnosed RMDs vs non-RMDs; Previously diagnosed RMDs vs non-RMDs) for health related quality of life (HRQoL) (EQ5D), physical function (HAQ), mental health (HADS) and health resources consumption. The effect of being undiagnosed for these outcomes was assessed in multivariable models adjusted for age, gender, geographical region and years of education (reference: non-RMD)., Results: A total of 3877 participants were included. The prevalence of undiagnosed RMDs was 29%. Compared to participants without RMDs, undiagnosed participants had lower HRQoL (EQ-5D: β (95% CI)=-0.07 (-0.103,-0.043)) and physical function (HAQ: β (95% CI)=0.10 (0.05, 0.15)), more anxiety (OR (95% CI)=2.3 (1.4, 3.7)) and depression symptoms (OR (95% CI)=1.4 (0.8, 2.4)). Undiagnosed RMDs participants were more likely to visit an orthopedist (OR (95% CI)=2.0 (1.1, 3.5)) and had a higher number of orthopedic appointments (IRR (95% CI)=2.5 (1.3, 4.9)) than participants without RMDs., Conclusion: Patients with undiagnosed RMDs are frequent in Portugal, have worse HRQoL, physical function and mental health than people without RMDs. Undiagnosed patients are nonetheless consumers of health resources and tend to seek help from specialties other than rheumatology. Increasing the awareness of RMDs might promote their early identification and treatment leading to both personal and societal benefits.

Background: The vasculitides are a group of 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 a web-based electronic clinical record, created in 2008, which currently includes specific modules for 12 diseases and > 20,000 patients registered from 79 rheumatology centres. On October 2014, a dedicated module for vasculitis was created as part of the European Vasculitis Society collaborative network, enabling prospective collection and central storage of encrypted data from patients with this condition. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, assessment tools, and treatment were collected. We aim to describe the structure of Reuma.pt/vasculitis and characterize the patients registered since its development., Results: A total of 687 patients, with 1945 visits, from 13 centres were registered; mean age was 53.4 ± 19.3 years at last visit and 68.7% were females. The most common diagnoses were Behçet's disease (BD) (42.5%) and giant cell arteritis (GCA) (17.8%). Patients with BD met the International Study Group criteria and the International Criteria for BD in 85.3 and 97.2% of cases, respectively. Within the most common small- and medium-vessel vasculitides registered, median [interquartile range] Birmingham Vasculitis Activity Score (BVAS) at first visit was highest in patients with ANCA-associated vasculitis (AAV) (17.0 [12.0]); there were no differences in the proportion of patients with AAV or polyarteritis nodosa who relapsed (BVAS≥1) or had a major relapse (≥1 major BVAS item) during prospective assessment (p = 1.00, p = 0.479). Biologic treatment was prescribed in 0.8% of patients with GCA, 26.7% of patients with AAV, and 7.6% of patients with BD. There were 34 (4.9%) deaths reported., Conclusions: Reuma.pt/vasculitis is a bespoke web-based registry adapted for routine care of patients with this form of rare and complex diseases, 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.

Objective: To establish in a global setting the relationships between countries' socioeconomic status (SES), measured biological disease modifying antirheumatic drug (bDMARD)-usage and disease outcomes. To assess if prescription and reimbursement rules and generic access to medication relates to a countries' bDMARD-usage., Methods: Data on disease activity and drug use from countries that had contributed at least 100 patients were extracted from the METEOR database. Mean disease outcomes of all available patients at the final visit were calculated on a per-country basis. A questionnaire was sent to at least two rheumatologists per country inquiring about DMARD-prices, access to treatment and valid regulations for prescription and reimbursement., Results: Data from 20 379 patients living in 12 different countries showed that countries' SES was positively associated with measured disease activity (meanDAS28), but not always with physical functioning (HAQ-score). A lower country's SES, stricter rules for prescription and reimbursement of bDMARDs as well as worse affordability of bDMARDs were associated with lower bDMARD-usage. bDMARD-usage was negatively associated with disease activity (although not with physical functioning), but the association was moderate at best., Conclusions: Disease activity in patients with rheumatoid arthritis as well as bDMARD-usage varies across countries worldwide. The (negative) relationship between countries' bDMARD-usage and level of disease activity is complex and under the influence of many factors, including-but not limited to-countries' SES, affordability of bDMARDs and valid prescription and reimbursement rules for bDMARDs., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: To measure early retirement due to self-reported rheumatic diseases (RDs) and to estimate the respective indirect costs and years of working life lost (YWLL)., Methods: We used individual level data from the national, cross-sectional, population-based EpiReumaPt study (September 2011-December 2013) where 10,661 inhabitants were randomly surveyed in order to capture and characterize all cases of RD within a representative sample of the Portuguese population. In this analysis, we used all participants aged between 50 and 64 years, near the official retirement age. A national database was used to calculate productivity values by gender, age and region, using the human capital approach. YWLL were estimated as the difference between each participant's current age and the respective retirement age, while the potential years of working life lost (PYWLL) were given by the difference between official and actual retirement ages. We also calculated the percentage of time in inactivity (inactivity ratio = YWLL/Active age-range [15-64 years old])., Results: 29.9% of the Portuguese population with ages between 50 and 64 years were retired with 13.1% self-reporting retirement due to RD. The estimated annual indirect cost following premature retirement attributed to RD was €910 million (€555 per capita; €1625 per self-reported RD patient and €13,592 per early retiree due to RD). Females contributed with 84% for these costs (€766 million; €882 per capita vs €187 from males). We observed a total number of 389,939 accumulated YWLL (228 per 1000 inhabitants) and 684,960 PYWLL (401 per 1000 inhabitants). The mean YWLL and PYWLL inactivity ratios were 12% and 21%, respectively. RD patients with higher values of disability have the highest risk of early retirement., Conclusions: Early retirement attributed to self-reported RD amounts to approximately 0.5% of the national gross domestic product (GDP) in 2013, due to large YWLL. Both the public health concern and the economic impact highlight the need to prioritize investments in health and social protection policies targeting patients with rheumatic conditions., (Copyright © 2016 The Royal Society for Public Health. Published by Elsevier Ltd. All rights reserved.)

Introduction: Interstitial lung disease (ILD) contributes significantly to morbidity and mortality in connective tissue disease (CTD). Early detection and accurate diagnosis are essential for informing treatment decisions and prognosis in this setting. Clear guidance on CTD-ILD screening, however, is lacking., Objective: To establish recommendations for CTD-ILD screening based on the current evidence., Method: Following an extensive literature research and evaluation of articles selected for their recency and relevance to the characterization, screening, and management of CTD-ILD, an expert panel formed by six pulmonologists from the Portuguese Society of Pulmonology, six rheumatologists from the Portuguese Society of Rheumatology, and six radiologists from the Portuguese Society of Radiology and Nuclear Medicine participated in a multidisciplinary discussion to produce a joint statement on screening recommendations for ILD in CTD., Results: The expert panel achieved consensus on when and how to screen for ILD in patients with systemic sclerosis, rheumatoid arthritis, mixed connective tissue disease, Sjögren syndrome, idiopathic inflammatory myopathies and systemic lupus erythematous., Conclusions: Despite the lack of data on screening for CTD-ILD, an expert panel of pulmonologists, rheumatologists and radiologists agreed on a series of screening recommendations to support decision-making and enable early diagnosis of ILD to ultimately improve outcomes and prognosis in patients with CTD.

Background: The aim of this article was to describe and discuss several strategies and standard operating procedures undertaken in the EpiReumaPt study - which was the first Portuguese, national, cross-sectional population-based study of Rheumatic and Musculoskeletal Diseases (RMD)., Methods: The technical procedures, legal issues, management and practical questions were studied, analyzed and discussed with relevant stakeholders. During the 1st phase of EpiReumaPt the coordination team and Centro de Estudos de Sondagens e Opinião (CESOP) worked to recruit and interview 10,661 subjects. The 2nd phase involved the participation of a multidisciplinary team, several local authorities, a specialized vehicle ("mobile unit") and a specific software program for the clinical appointments. The development of specific recruitment strategies improved the participation rate. Blood samples were collected and sent to Biobanco-IMM and to a central lab for immediate measurements. In the 3rd phase the RMD diagnosis were validated by a team of three experienced rheumatologists - clinical data, imaging and lab test results were revised according to previously published classification criteria., Conclusion: EpiReumaPt was a nationwide project successfully conducted, which followed critical logistic/coordination and research strategies. EpiReumaPt methodology and coordination could be used as an example for other epidemiologic endeavors and public health policies.

B-cells play an important role not only in cellular but also in humoral immunity through differentiation into plasma cells and antibody production. B-cell depletion may, theoretically, change the course of systemic rheumatic diseases (SRD) in which self-reactive antibodies are part of the pathogenic pathway. In Rheumatology, anti-B-cell antibody Rituximab is currently licensed for the treatment of rheumatoid arthritis, however there is growing evidence of its potential use in other SRD. The authors present a case series of eight patients in which Rituximab was used off-label including overlap syndrome Rhupus, systemic lupus erythematosus and Wegener's granulomatosis. In the end, a brief literature review about this subject is performed.

Poncet's disease is a reactive polyarthritis associated to active tuberculosis (TB), with excluded presence of mycobacterium in the joints and bones affected, and no other cause for the clinical arthritis. It is a frequently forgotten entity, especially in countries with low tuberculosis prevalence. It is described a case report of a man of Guinea-Bissau that presented symmetrical generalized polyarthralgias and hands swelling, clinical sacroiliítis and multiple enthesopathies. Serum and urinary biochemical testing were normal. Immunologic studies were negative and serum levels of angiotensin-converting enzyme and calcium were normal. Sexual transmitted diseases were excluded. The initial diagnose was undifferentiated polyarthritis or reactive arthritis. A persistent and refractory monoarthritis of the right wrist demanded a direct and cultural exam of the synovial fluid, synovial biopsy and protein chain reaction for TB that were negative. After he developed right wrist arthritis with purulent synovial fluid positive to TB in the direct exam and the detection of M. tuberculosis in the cultural exam. Granula was found in his chest radiograph. He was diagnosed a Poncet's disease reactive to a pulmonary TB infection.

Introduction: Rheumatic diseases (RD) are conditions with a variety of clinical manifestations and prognosis influenced by several factors. Cohorts and registries have been already established in some countries and have contributed to important knowledge about the disease course and the long-term outcomes of RD. This paper introduces the CoReumaPt project and sets the first step towards the creation of a prospective cohort study including the main RD occurring in the Portuguese population. CoReumaPt will allow outcomes research of chronic RD and the assessment of factors influencing the development and progression of RD. It will also allow to further evaluate the economic impact and the burden of RD in Portugal. CoReumaPt will be linked to Reuma.pt, the National Register of Rheumatic Diseases from the Portuguese Society of Rheumatology., Methods: An open cohort will be created, initially composed by the randomly selected population of the crosssectional National Epidemiological Rheumatic Diseases study (EpiReumaPt) and afterwards by other sources, namely through self- and physician's referral. Follow-up with annual self-administered questionnaires will be performed, in order to systematically collect and analyze outcomes of interest, mainly patient-reported outcomes. Data concerning less frequent assessments, such as radiographs and biomarkers, will also be assembled., Conclusions: CoReumaPt will be a valuable resource for scientific research and will deliver pivotal information to improve public health policies concerning the prevention and the management of RD in Portugal.

Rheumatic diseases (RDs) are among the most common diseases. In the developed world, they are the leading cause of disability and consume a large amount of health and social resources. Therefore, it is crucial to assess the impact of RDs on the general population in what refers to their prevalence, and repercussion on quality of life and function. However, no nationwide epidemiological studies on RDs have ever been performed in Portugal. With this research project we aim to estimate the prevalence of different RDs in Portugal, as well as to determine the burden of RDs, more specifically their impact on quality of life and functional and work capacity. A cross-sectional study will be performed, using a random sample of the Portuguese population. RDs will be screened through a structured interview, and subjects with a positive screening will be examined by a trained rheumatologist to establish the final diagnosis. The knowledge of the prevalence of RDs will contribute to the development of specific health plans for the current and future management of these diseases.

The high prevalence of inflammatory rheumatic diseases in women of childbearing age increases the risk of exposure to antirheumatic agents during conception pregnancy and breast feeding. The decision for pharmacological treatment initiation maintenance should be the result between the severity of maternal disease and the risk benefits with treatment. The aim of this paper was to review recent literature about drug fetal safety profile strength the importance of monitoring the pregnancy in patients with inflammatory rheumatic diseases and stress the need for further research in this area.