Rheuma SPACE - Standard Practice Aiming Clinical Excellence: the first Portuguese Rheumatology Department evaluation.

The Portuguese Rheumatology Society (SPR) embraced quality as a major goal and launched, in early 2015, a program to aim for excellence in global clinical care: Rheuma SPACE - Standard Practice Aiming Clinical Excellence. Evaluating daily reality is the first step in a quality development timeline, ultimately contributing for health gains. Herein we describe the results of the evaluation of the quality indicators defined for this project and the improvement strategies identified. The Rheuma SPACE project included three phases: 1) establishing a set of quality indicators and an excellence quality model; 2) assessment of the current care at Rheumatology departments concerning the defined quality indicators in the scope of the excellence model; and 3) elaboration of global and customized reports for each participating Rheumatology department, resulting in the identification of improvement opportunities. Ten Rheumatology departments, countrywide, including larger and smaller institutions, were asked to participate in Rheuma SPACE. This resulted in an individual report for each department along with global benchmarking practices analysis. Furthermore, a list of improvement initiatives was developed. We concluded that departments lack physicians and need exclusively dedicated nurses. Time dedicated to research and audit activities should be specifically allocated. Internal contracting is well established, and professionals are committed to targets. Processes are still suboptimal, needing standardization of triage criteria, more frequent follow-up, as well as better medical records and multidisciplinary coverage. Regarding outcomes, patients are satisfied with the provided care and professionals with the working environment. However, department facilities for the former, and career related aspects, for the latter should improve. With this innovative study conducted in Portugal we expect to have enlightened tailored opportunities for improvement, ensure patient-focused practices and be able to define the indispensable quality requirements for excellence.

Rheuma SPACE - Standard Practice Aiming Clinical Excellence: description of the methodological approach.

BACKGROUND: Quality of care is a key component of the right to health, and the route to equity and dignity. The aim of the project Rheuma SPACE - Standard Practice Aiming Clinical Excellence was to develop a set of quality indicators focused in rheumatoid arthritis care and apply them to rheumatology departments of the Portuguese National Health Service in order to benchmark the care for these patients. This article details the methodology that was applied. METHODOLOGY: This was a single country, three-phase project, each phase comprising multiple steps. The first step defined quality indicators and the excellence quality model to be used. It involved a literature search for international benchmarking of quality of care initiatives and indicators, followed by a pre-selection of an initial set of indicators. The set of indicators was latter on narrowed after an online Delphi round with all Portuguese rheumatologists and two consensus meetings involving the study task force. A set of 26 quality indicators was defined, within the three classic Donabedian dimensions of healthcare quality: Structure (9), Processes (11), and Outcomes (6). These indicators cover eleven domains of quality of care: personnel and organizational structure, training and research, facilities, equipment and information technology, budgeting and financial resources, access to care, clinical records, patient communication, multidisciplinary management, clinical outcomes, and patient and personnel satisfaction. Decision on quality and excellence thresholds for each of the 26 quality indicators was agreed upon a consensus meeting gathering principal investigators of the eight Rheumatology Departments that decided to participate, task force core set members and invited representatives of all Portuguese Departments/Units. Rheumatoid arthritis was the chosen disease model of the project based on the reliability of the outcomes to be measured in the context of this condition. The second step was the assessment of the participating Rheumatology Departments. During eighteen months, research teams applied the 26 quality indicators to their own Departments. The third step comprised data analysis and the elaboration of individual Rheumatology Department reports and of a global public report. RESULTS: Eight Departments, comprising 80 specialists, 20 residents and 30 nurses, covering 5.904.080 inhabitants, underwent quality evaluation. More than one thousand patients (1,325) and 113 health professionals' surveys were analysed, as well as data from 570 clinical records and 3,927 medical appointments on rheumatoid arthritis patients. DISCUSSION: 26 quality indicators were used for the first evaluation of Portuguese Rheumatology Departments, turning Rheuma SPACE into a pioneer project. Data analysis and benchmarking will be the subject of a further publication.

PORTUGUESE RECOMMENDATIONS FOR THE USE OF BIOLOGICAL THERAPIES IN PATIENTS WITH PSORIATIC ARTHRITIS--2015 UPDATE.

OBJECTIVE: To update recommendations for the treatment of psoriatic arthritis with biological therapies, endorsed by the Portuguese Society of Rheumatology (SPR). METHODS: These treatment recommendations were formulated by Portuguese rheumatologists based on literature evidence and consensus opinion. At a national meeting the 16 recommendations included in this document were discussed and updated. The level of agreement among Portuguese Rheumatologists was assessed using an online survey. A draft of the full text of the recommendations was then circulated and suggestions were incorporated. A final version was again circulated before publication. RESULTS: A consensus was achieved regarding the initiation, assessment of response and switching biological therapies in patients with psoriatic arthritis (PsA). Specific recommendations were developed for several disease domains: peripheral arthritis, axial disease, enthesitis and dactylitis. CONCLUSION: These recommendations may be used for guidance in deciding which patients with PsA should be treated with biological therapies. They cover a rapidly evolving area of therapeutic intervention. As more evidence becomes available and more biological therapies are licensed, these recommendations will have to be updated.

Osteoarthritis medical labelling and health-related quality of life in the general population.

BACKGROUND: Osteoarthritis is the most common chronic joint disease. In the absence of an effective medical treatment and due to the chronic nature of this condition, an osteoarthritis medical diagnosis may finally result in decreased health-related quality of life. Therefore, the aim of this study was to measure the impact of the osteoarthritis medical labelling on physical and mental health-related quality of life. METHODS: Subjects (n = 1132, 58.7% women) were approached as participants of an urban population-based cohort (EPIPorto). Self-reported information on previous diagnosis of knee, hip or hand osteoarthritis was obtained and rheumatologists established knee, hip or hand osteoarthritis clinical diagnosis in symptomatic individuals. Physical and mental dimensions of health-related quality of life were evaluated using the self-administered Medical Outcomes Study: 36-Item Short Form Survey. Crude and adjusted linear regression coefficients (beta) and the corresponding 95% confidence intervals (95% CI) were computed to estimate the associations between being labelled as an osteoarthritis case and health-related quality of life. RESULTS: Regardless of disease medical labelling, individuals with osteoarthritis scored significantly lower physical health-related quality of life when compared to those without joint disease (kneeunexposed: beta = -5.3, 95% CI: -7.6, -3.1; kneeexposed: beta = -6.0, 95% CI: -8.4, -3.7; hipunexposed: beta = -6.0, 95% CI: -9.8, -2.3; hipexposed: beta = -11.0, 95% CI: -15.6, -6.4; handunexposed: beta = -4.3, 95% CI: -6.5, -2.0; handexposed: beta = -4.3, 95% CI: -6.6, -2.1). The same was not observed regarding mental health-related quality of life. Among subjects with clinically confirmed osteoarthritis, the medical labelling of this joint disease was not significantly associated to health-related quality of life. CONCLUSIONS: The labelling of knee, hip and hand osteoarthritis diagnosis may not add specific benefit to osteoarthritis patients in terms of its capability to improve health-related quality of life.

Prognostic value of antinuclear antibodies in juvenile idiopathic arthritis and anterior uveitis. Results from a systematic literature review.

AIMS: To analyze the prognostic role of antinuclear antibodies (ANA) for the onset of uveitis in the context of juvenile idiopathic arthritis (JIA), its correlation with uveitis course and severity and its prognostic role for the development of arthritis in children with uveitis. METHODS: We conducted a systematic review analysis of the literature on the prognostic value of ANA on JIA associated uveitis and its complications. We included series published between January 1990 and December 2011 reporting the prognostic value of ANA positivity on uveitis in consecutive patients diagnosed with JIA. RESULTS: We identified 246 studies from our search, of which 25 were selected for detailed analysis and only 9 fulfilled the inclusion criteria. Some authors have mentioned that uveitis could preceed arthritis and that ANA positivity might represent a predictive factor for subsequent joint involvement. A chronic course and insidious onset of uveitis are predictors for an association with JIA. Although recognized as a possible predictor of uveitis development, presence of positive ANA does not represent a predictor of severity. CONCLUSIONS: The presence of ANAs seems to be a risk factor for ocular involvement in patients with JIA. These autoantibodies, however, did not have any correlation with the recurrence of either idiopathic anterior uveitis or JIA-related uveitis and cannot be used as a marker to predict the clinical course of ocular inflammation. Any analysis of the literature is subjected to the limitations of each of the studies under evaluation. A large, prospective population-based study of JIA patients would be certainly ideal.

Monoclonal gammopathy and rheumatic diseases.

It is common to find monoclonal gammopathy in the investigation workout for an unrelated disorder. There are studies relating rheumatic diseases and therapies which show an increased risk of monoclonal gammopathy. The specific mechanisms are not well understood but chronic antigen stimulation assumes an important role. Specific rheumatic diseases have consistently been associated with lymphoproliferative disorders but less attention has been paid to the possible association between monoclonal gammopathy of undetermined significance and multiple myeloma. We reviewed previous studies regarding monoclonal gammopathy and different rheumatic diseases and treatments associated focusing on prevalence, risk factors and possible pathogenic mechanisms. The clinical approach of a monoclonal gammopathy and its follow-up are explained.

The Portuguese Society of Rheumatology position paper on the use of biosimilars.

Biotechnological drugs have become a fundamental resource for the treatment of rheumatic patients. Patent expiry of some of these drugs created the opportunity for biopharmaceutical manufacturers to develop biosimilar drugs intended to be as efficacious as the originator product but with a lower cost to healthcare systems. Due to the complex manufacturing process and highly intricate structure of biologicals, a biosimilar can never be an exact copy of its reference product. Consequently, regulatory authorities issued strict preclinical and clinical guidelines to ensure safety and efficacy equivalence and, in September 2013, the biosimilar of infliximab was the first biosimilar monoclonal antibody to be authorized for use in the European Union. The current document is a position statement of the "Sociedade Portuguesa de Reumatologia" (Portuguese Society of Rheumatology) on the use of biosimilar drugs in rheumatic diseases. Two systematic literature reviews were performed, one concerning clinical trials and the other one concerning international position papers on biosimilars. The results were presented and discussed in a national meeting and a final position document was discussed, written and approved by Portuguese rheumatologists. Briefly, this position statement is contrary to automatic substitution of the originator by the biosimilar, defends either a different INN or the prescription by brand name, supports that switching between biosimilars and the originator molecule should be done after at least 6 months of treatment and based on the attending physician decision and after adequate patient information, recommends the registration of all biosimilar treated patients in Reuma.pt for efficacy, safety and immunogenicity surveillance, following the strategy already ongoing for originators, and opposes to extrapolation of indications approved to the originator to completely different diseases and/or age groups without adequate pre-clinical, safety or efficacy data.

2011 Portuguese recommendations for the use of biological therapies in patients with psoriatic arthritis.

OBJECTIVE: To develop recommendations for the treatment of psoriatic arthritis (PsA) with biological therapies, endorsed by the Portuguese Society of Rheumatology. METHODS: These treatment recommendations were formulated by Portuguese rheumatologists based on literature evidence and consensus opinion. A draft of the recommendations was first circulated to all Portuguese rheumatologists and their suggestions were incorporated in the draft. At a national meeting the recommendations were discussed and all attending rheumatologists voted on the level of agreement for each recommendation. A second draft was again circulated before publication. RESULTS: A consensus was achieved regarding the initiation, assessment of response and switching biological therapies in patients with PsA. Specific recommendations were developed for several disease domains: peripheral arthritis, axial disease, enthesitis and dactylitis. CONCLUSION: These recommendations may be used for guidance in deciding which patients with PsA should be treated with biological therapies. They cover a rapidly evolving area of therapeutic intervention. As more evidence becomes available and more biological therapies are licensed, these recommendations will have to be updated.

Portuguese recommendations for the use of biological therapies in patients with axial spondyloarthritis--December 2011 update.

OBJECTIVE: To develop recommendations for the treatment of axial spondyloarthritis with biological therapies, endorsed by the Portuguese Society of Rheumatology. METHODS: These treatment recommendations were formulated by Portuguese rheumatologists based on literature evidence and consensus opinion. A draft of the recommendations and supporting evidence was first circulated to all Portuguese rheumatologists and their suggestions were incorporated in the draft. Secondly, at a national meeting the recommendations were presented, discussed and revised. Finally, the document resulting from this meeting was again circulated to all Portuguese rheumatologists, who anonymously voted online on the level of agreement with the recommendations. RESULTS: A consensus was achieved regarding the initiation, assessment of response and switching biological therapies in patients with axial spondyloarthritis. CONCLUSION: These recommendations may be used for guidance in deciding which patients with axial spondyloarthritis should be treated with biological therapies. They cover a rapidly evolving area of therapeutic intervention. As more evidence becomes available and more biological therapies are licensed, these recommendations will have to be updated.

[Complex Regional Pain Syndrome--an unusual etiology]. / Síndrome Doloroso Regional Complexo Tipo 1--uma etiologia incomum.

Complex Regional Pain Syndrome type 1 is characterised by neuropathic pain associated with autonomic dysfunctions. It frequently appears after major or minor trauma and more rarely may originate from conditions that compromise the central nervous system. Although few treatments have shown to be effective, their institution at an early stage is decisive for their success. For this reason it is important to ensure timely recognition of this clinical entity, attempting to identify its cause and the predominant underlying physiopathological mechanisms whenever possible. The authors describe a case of complex regional pain syndrome type 1, emphasising the rarity of this clinical situation in association with a Parkinsonian Syndrome.

Portuguese guidelines for the use of biological agents in rheumatoid arthritis - October 2011 update.

The authors present the revised version of the Portuguese Society of Rheumatology (SPR) guidelines for the treatment of Rheumatoid Arthritis (RA) with biological therapies. In these guidelines the criteria for introduction and maintenance of biological agents are discussed as well as the contraindications and procedures in the case of nonresponders. Biological treatment (with a tumour necrosis factor antagonist, abatacept or tocilizumab) should be considered in RA patients with a disease activity score 28 (DAS 28) equal to or greater than 3.2 despite treatment with at least 20mg-weekly-dose of methotrexate (MTX) for at least 3 months or, if such treatment is not possible, after 3 months of other conventional disease modifying drug or combination therapy. A DAS 28 score between 2.6 and 3.2 with a significant functional or radiological deterioration under treatment with conventional regimens could also constitute an indication for biological treatment. The treatment goal should be remission or, if that is not achievable, at least a low disease activity, defined by a DAS28 lower than 3.2, without significative functional or radiological worsening. The response criteria, at the end of the first 3 months of treatment, are a decrease of at least 0.6 in the DAS28 score. After 6 months of treatment res­ponse criteria is defined as a decrease greater than 1.2 in the DAS28 score. Non-responders, in accordance to the Rheumatologist's clinical opinion, should try a switch to another biological agent (tumour necrosis factor antagonist, abatacept, rituximab or tocilizumab).

[Practical guide for the use of biological agents in rheumatoid arthritis - December 2011 update]. / Guia prático de utilização de terapêuticas biotecnológicas na artrite reumatóide - actualização de Dezembro 2011.

The authors review the practical aspects of biological therapy use for rheumatoid arthritis patients, commenting safety issues before and after treatment initiation and the best treatment strategies to optimize efficacy.

Portuguese guidelines for the use of biological agents in rheumatoid arthritis - March 2010 update.

The authors present the revised version of the Portuguese Society of Rheumatology (SPR) guidelines for the treatment of rheumatoid arthritis (RA) with biological therapies. In these guidelines the criteria for introduction and maintenance of biological agents are discussed as well as the contraindications and procedures in the case of non-responders. Biological treatment should be considered in RA patients with a disease activity score 28 (DAS 28) superior to 3.2 despite treatment with 20mg/week of methotrexate (MTX) for at least 3 months or, if such treatment is not possible, after 6 months of other conventional disease modifying drug or combination therapy. A DAS 28 score between 2.6 and 3.2 with a significant functional or radiological deterioration under treatment with conventional regimens could also constitute an indication for biological treatment. The treatment goal should be remission or, if that is not achievable, at least a low disease activity, characterized by a DAS28 lower than 3.2, without significative functional or radiological worsening. The response criteria, at the end of the first 3 months of treatment, are a decrease of 0.6 in the DAS28 score. After 6 months of treatment response criteria is defined as a decrease of more than 1.2 in the DAS28 score. Non-responders, in accordance to the Rheumatologist's clinical opinion, should try a switch to another biological agent (tumour necrosis factor antagonist, abatacept, rituximab or tocilizumab).

Methotrexate treatment in rheumatoid arthritis: management in clinical remission, common infection and tuberculosis. Results from a systematic literature review.

This work was performed as part of the Portuguese participation in the 3E Initiative 2007-2008, dedicated to the use of methotrexate (MTX) in rheumatic conditions. Three questions raised by Portuguese rheumatologists and considered relevant to clinical practice remained out of the selection of a set of ten key questions formulated to further establish multinational recommendations on the use of MTX in rheumatic diseases. The authors collected and analyzed all the evidence available by using a systematic literature search methodology and selection criteria concerning the following issues in rheumatoid arthritis (RA): (1) the management of MTX after clinical remission; (2) the management of MTX during infections and (3) the screening and treatment of tuberculosis in patients on MTX treatment. A total of 1,862 references were identified, of which 163 were selected for detailed analysis and 12 included in the final review. The evidence was appraised according to the Oxford Centre for Evidence-based Medicine (EBM) levels of evidence. Although with limited evidence, the authors concluded that: (1) extending the interval for MTX therapy may be a valid alternative regimen in a subset of RA patients in clinical remission (EBM level 2b); (2) MTX may be safe during some common infections in RA patients (EBM level 3b/4); (3) screening and treatment of TB in patients on MTX should be similar to the general population (EBM level 4). The evidence available to support clinical decisions in this area is very limited in number and quality. There is a need for further research and while that is unavailable, practical decisions have to rely on experience and expert opinion.

[Acute febrile neutrophilic dermatosis - Sweet's syndrome]. / Dermatose neutrofílica aguda febril - síndrome de Sweet.

In Rheumatology there are several diseases that frequently develop cutaneous manifestations creating diagnostic difficulties. The Sweet's syndrome appears as archetype of the neutrophilic dermatosis,which is a group of not infectious illnesses, characterized for a dermic neutrophilic and angiocentric infiltrated. The four main features that define this syndrome are: cutaneous eruption, fever, peripheral neutrophilia and dermic neutrophilic infiltrated without vasculitis on skin biopsy. The authors describe a typical clinical case of Sweet's syndrome, pointing out the multiplicity of clinical situations that can simulate this pathology, making difficult its diagnosis and highlighting the need for suspicious in patients with both musculoskeletal and cutaneous involvement.

[Rasburicase for tophaceus gout treatment]. / Rasburicase no tratamento de gota tofácea.

The main obstacle to the treatment of hyperuricemia in patients with allergy to allopurinol is the limited availability of equally efficient alternative drugs. The authors present a clinical case of a patient with incapacitant tophaceus gout, allergy to allopurinol and contraindication for uricosurics, who was treated with rasburicase, an urato-oxidase recombinant, based on experience with this drug in tumoral lisis and in some cases reports of tophaceus gout. The authors also enhance the fact of being a patient with a sequelar hemiplegia of a previous cerebrovascular disease that presents tophus only in the not paretic member. The management of patients with allergy to allopurinol can be a clinical challenge, and the monthly rasburicase perfusions may be an alternative treatment of serious gout not treatable for other ways.

Macroscopic features of knee synovitis in early untreated Behçet disease and psoriatic arthritis.

In a previous study, we found that synovial immunopathology differs between Behçet disease (BD) and psoriatic arthritis (PsA). The objective of this study is to describe the macroscopic features of early untreated knee synovitis in BD and PsA. Fourteen consecutive patients with active early knee synovitis (seven BD and seven PsA) undergoing rheumatologic arthroscopy were assessed. The following macroscopic synovial features were evaluated and scored by analyzing the video recordings of each procedure: capillary hyperaemia, morphology of synovitis, vascular pattern, fibrinoid membranes, and topographic distribution of these features. Video-recording of 35 early untreated arthritis patients with different diagnoses were also studied looking for BD-like macroscopic features. Six out of seven BD patients had extensive fibrinoid membranes and large areas of erythematous synovitis without villi or a distinctive vascular pattern, while PsA patients had diffuse erythematous villous synovitis with a tortuous vascular morphology. None of the 35 patients with early untreated arthritis exhibited all the characteristic features of BD synovitis. This exploratory study shows some distinctive features between BD and PsA knee synovitis that confirm macroscopic differences in patients with previously reported immunopathological differences.

Camurati-Engelmann disease: typical manifestations of a rare disease / Doença de Camurati-Engelmann: manifestações típicas de uma doença rara

A displasia diafisária progressiva ou doença de Camurati-Engelmann é uma doença rara, caracterizada por dor e fraqueza muscular dos membros afetados e espessamento progressivo da cortical diafisária dos ossos longos. Os autores descrevem o caso clínico de um doente com manifestações iniciais da doença na infância, mas cujo diagnóstico só foi estabelecido durante a idade adulta, depois de evoluída a doença e após surgirem as mesmas manifestações em um dos filhos. Salienta-se a dificuldade no diagnóstico e a relevância do diagnóstico diferencial com outras doenças que cursam com osteoesclerose e/ou hiperostose. Na literatura é rara a descrição da sua evolução ao longo dos anos.

Camurati-Engelmann Disease or progressive diaphyseal dysplasia is a rare disease, characterized by limb pain and muscular weakness, and cortical thickening of the diaphyses of long bones. The authors report a case of a male patient with manifestations since his childhood, whose diagnosis was established later on, when he was an adult, with the disease already progressed, and when the same manifestations began in one of his sons. The importance of the differential diagnosis regarding other diseases concurrent with osteosclerotic and/or hyperostotic changes is emphasized here. Description of its evolution along the years is rarely found in the literature.