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1.
Clin Rheumatol ; 42(8): 2125-2134, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37154983

RESUMO

INTRODUCTION/OBJECTIVES: The study aims to define the clinical and subclinical calcinosis prevalence, the sensitivity of radiographed site and clinical method for its diagnosis, and the phenotype of Portuguese systemic sclerosis (SSc) patients with calcinosis. METHOD: A cross-sectional multicenter study was conducted with SSc patients fulfilling Leroy/Medsger 2001 or ACR/EULAR 2013 classification criteria, registered in the Reuma.pt. Calcinosis was assessed through clinical examination and radiographs of hands, elbows, knees, and feet. Independent parametric or non-parametric tests, multivariate logistic regression, and sensitivity calculation of radiographed site and clinical method for calcinosis detection were performed. RESULTS: We included 226 patients. Clinical calcinosis was described in 63 (28.1%) and radiological calcinosis in 91 (40.3%) patients, of which 37 (40.7%) were subclinical. The most sensitive location to detect calcinosis was the hand (74.7%). Sensitivity of the clinical method was 58.2%. Calcinosis patients were more often female (p = 0.008) and older (p < 0.001) and had more frequently longer disease duration (p < 0.001), limited SSc (p = 0.017), telangiectasia (p = 0.039), digital ulcers (p = 0.001), esophageal (p < 0.001) and intestinal (p = 0.003) involvements, osteoporosis (p = 0.028), and late capillaroscopic pattern (p < 0.001). In multivariate analysis, digital ulcers (OR 2.63, 95% CI 1.02-6.78, p = 0.045) predicted overall calcinosis, esophageal involvement (OR 3.52, 95% CI 1.28-9.67, p = 0.015) and osteoporosis (OR 4.1, 95% CI 1.2-14.2, p = 0.027) predicted hand calcinosis, and late capillaroscopic pattern (OR 7.6, 95% CI 1.7-34.9, p = 0.009) predicted knee calcinosis. Anti-nuclear antibody positivity was associated with less knee calcinosis (OR 0.021, 95% CI 0.001-0477, p = 0.015). CONCLUSIONS: Subclinical calcinosis high prevalence suggests that calcinosis is underdiagnosed and radiographic screening might be relevant. Multifactorial pathogenesis may explain calcinosis predictors' variability. Key Points • Prevalence of subclinical calcinosis in SSc patients is substantial. • Hand radiographs are more sensitive to detect calcinosis than other locations or clinical method. • Digital ulcers were associated with overall calcinosis, esophageal involvement and osteoporosis were associated with hand calcinosis, and late sclerodermic pattern in nailfold capillaroscopy was associated with knee calcinosis. • Anti-nuclear antibody positivity may be a protective factor for knee calcinosis.


Assuntos
Calcinose , Osteoporose , Escleroderma Sistêmico , Feminino , Humanos , Estudos Transversais , Portugal , Calcinose/complicações , Calcinose/diagnóstico por imagem , Osteoporose/complicações
2.
ARP Rheumatol ; 2(1): 78-82, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36802346

RESUMO

Arthritis in the paediatric population is the hallmark of many rheumatic inflammatory diseases, as well as other cutaneous, infectious, or neoplastic conditions. It can be quite devastating, whereby prompt recognition and treatment of these disorders are essential. However, arthritis can sometimes be mistaken for other cutaneous or genetic conditions leading to misdiagnosis and overtreatment. Pachydermodactyly is a rare and benign form of digital fibromatosis, usually manifested by swelling of the proximal interphalangeal joints of both hands, mimicking arthritis. The authors report a case of a 12-year-old boy with a one-year history of painless swelling of the proximal interphalangeal joints of both hands that was referred to the Paediatric Rheumatology department due to the suspicion of juvenile idiopathic arthritis. The diagnostic work-up was unremarkable, and the patient remained asymptomatic over an 18-month follow-up period. A diagnosis of pachydermodactyly was assumed and no treatment was introduced, given the benign nature of the disorder and absence of symptoms. Therefore, it was possible to safely discharge the patient from the Paediatric Rheumatology clinic.


Assuntos
Artrite Juvenil , Dermatite , Fibroma , Dermatopatias , Masculino , Humanos , Criança , Artrite Juvenil/diagnóstico , Pele , Fibroma/diagnóstico , Articulações dos Dedos/diagnóstico por imagem
3.
ARP Rheumatol ; 1(ARP Rheumatology, nº3 2022): 251-254, 2022 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-36057086

RESUMO

Clinically amyopathic dermatomyositis (CADM) is a rare condition characterized by dermatomyositis skin lesions without clinically apparent muscle involvement. Respiratory involvement is common, occurring in about half of the cases. Spontaneous pneumomediastinum (PnM) is a rare, and often fatal, complication of CADM. We report a case of a 61-year-old female patient who was diagnosed with anti-melanoma differentiation- associated gene 5 antibody-associated CADM and interstitial lung disease. She developed an extensive spontaneous PnM with subcutaneous emphysema. The patient was treated with a conservative approach which was, initially, successful in reducing the size of the PnM. However, the patient died from an eventual nosocomial pneumonia requiring mechanical ventilation. This case illustrates that improving the management of CADM associated PnM, remains a major unmet need.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Enfisema Mediastínico , Enfisema Subcutâneo , Feminino , Humanos , Pessoa de Meia-Idade , Dermatomiosite/complicações , Enfisema Mediastínico/diagnóstico , Doenças Pulmonares Intersticiais/complicações , Enfisema Subcutâneo/diagnóstico
4.
Adv Rheumatol ; 62: 20, 2022. tab
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1383511

RESUMO

Abstract Background: Rheumatic diseases are associated with an increase in overall risks of tuberculosis (TB). The aim of this study was to evaluate the frequency of TB and the frequency of latent TB infection (LTBI), in clinical practice, for juvenile idiopathic arthritis (JIA) patients from high and low risk of TB incidence endemic countries. Methods: This is an international, multicenter, cross-sectional, observational study of data collection from Brazil and Registry of Portugal at REUMA.PT. The inclusion criteria were patients with Juvenile Idiopathic Arthritis (JIA) with age ≤ 18 years who underwent screening for Mycobacterium tuberculosis infection [tuberculin skin test (TST) and/or interferon gamma release assay (IGRA)]. Chest X-rays and history of exposure to TB were also assessed. Results: 292 JIA patients were included; mean age 14.3 years, mean disease duration 7.5 years, 194 patients (66.4%) performed only TST, 14 (4.8%) only IGRA and 84 (28.8%) both. The frequency of LTBI (10.6%) and TB was similar between the two countries. The reasons for TB screening were different; in Brazil it was performed more often at JIA onset while in Portugal it was performed when starting Disease Modified Anti-Rheumatic Drugs (DMARD) treatment (p < 0.001). Isoniazid therapy was prescribed in 40 (13.7%) patients (31 with LTBI and 9 with epidemiologic risks and/or due to contact with sick people). Only three patients (1%) developed active TB. Conclusion: We found nearly 10% of patients with LTBI, a small percentage of patients with treatment due to epide-miologic risks and only 1% with active TB. Distinct reasons and screening methods for LTBI were observed between the two countries.

5.
RMD Open ; 6(1)2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-32144137

RESUMO

OBJECTIVES: To compare definitions of high disease activity of the Ankylosing Spondylitis Disease Activity Score (ASDAS) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) in selecting patients for treatment with biologic disease-modifying antirheumatic drugs (bDMARDs). METHODS: Patients from Rheumatic Diseases Portuguese Register (Reuma.pt) with a clinical diagnosis of axial spondyloarthritis (axSpA) were included. Four subgroups (cross-tabulation between ASDAS (≥2.1) and BASDAI (≥4) definitions of high disease activity) were compared regarding baseline characteristics and response to bDMARDs at 3 and 6 months estimated in multivariable regression models. RESULTS: Of the 594 patients included, the majority (82%) had both BASDAI≥4 and ASDAS ≥2.1. The frequency of ASDAS ≥2.1, if BASDAI<4 was much larger than the opposite (ie, ASDAS <2.1, if BASDAI≥4): 62% vs 0.8%. Compared to patients fulfilling both definitions, those with ASDAS ≥2.1 only were more likely to be male (77% vs 51%), human leucocyte antigen B27 positive (79% vs 65%) and have a higher C reactive protein (2.9 (SD 3.5) vs 2.1 (2.9)). Among bDMARD-treated patients (n=359), responses across subgroups were globally overlapping, except for the most 'stringent' outcomes. Patients captured only by ASDAS responded better compared to patients fulfilling both definitions (eg, ASDAS inactive disease at 3 months: 61% vs 25% and at 6 months: 42% vs 25%). CONCLUSION: The ASDAS definition of high disease activity is more inclusive than the BASDAI definition in selecting patients with axSpA for bDMARD treatment. The additionally 'captured' patients respond better and have higher likelihood of predictors thereof. These results support using ASDAS≥2.1 as a criterion for treatment decisions.


Assuntos
Antirreumáticos/uso terapêutico , Seleção de Pacientes , Índice de Gravidade de Doença , Espondilite Anquilosante/tratamento farmacológico , Inibidores do Fator de Necrose Tumoral/uso terapêutico , Adulto , Proteína C-Reativa/metabolismo , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estudos Prospectivos , Espondilite Anquilosante/metabolismo , Espondilite Anquilosante/fisiopatologia , Inquéritos e Questionários
6.
Acta Reumatol Port ; 42(2): 127-140, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28535545

RESUMO

BACKGROUND: Methotrexate (MTX) is the first-line drug in the treatment of rheumatoid arthritis (RA) and the most commonly prescribed disease modifying anti-rheumatic drug. Moreover, it is also used as an adjuvant drug in patients under biologic therapies, enhancing the efficacy of biologic agents. OBJECTIVES: To review the literature and update the Portuguese recommendations for the use of MTX in rheumatic diseases first published in 2009. METHODS: The first Portuguese guidelines for the use of MTX in rheumatic diseases were published in 2009 and were integrated in the multinational 3E Initiative (Evidence Expertise Exchange) project. The Portuguese rheumatologists based on literature evidence and consensus opinion formulated 13 recommendations. At a national meeting, the recommendations included in this document were further discussed and updated. The document resulting from this meeting circulated to all Portuguese rheumatologists, who anonymously voted online on the level of agreement with the updated recommendations. RESULTS: Results presented in this article are mainly in accordance with previous guidelines, with some new information regarding hepatitis B infection during MTX treatment, pulmonary toxicity monitoring, hepatotoxicity management, association with hematologic neoplasms, combination therapy and tuberculosis screening during treatment. CONCLUSION: The present recommendations combine scientific evidence with expert opinion and attained desirable agreement among Portuguese rheumatologists. The regular update of these recommendations is essential in order to keep them a valid and useful tool in daily practice.


Assuntos
Antirreumáticos/uso terapêutico , Metotrexato/uso terapêutico , Doenças Reumáticas/tratamento farmacológico , Humanos , Portugal , Guias de Prática Clínica como Assunto
7.
RMD Open ; 2(1): e000166, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26848402

RESUMO

OBJECTIVES: To estimate the national prevalence of rheumatic and musculoskeletal diseases (RMDs) in the adult Portuguese population and to determine their impact on health-related quality of life (HRQoL), physical function, anxiety and depression. METHODS: EpiReumaPt is a national health survey with a three-stage approach. First, 10 661 adult participants were randomly selected. Trained interviewers undertook structured face-to-face questionnaires that included screening for RMDs and assessments of health-related quality of life, physical function, anxiety and depression. Second, positive screenings for ≥1 RMD plus 20% negative screenings were invited to be evaluated by a rheumatologist. Finally, three rheumatologists revised all the information and confirmed the diagnoses according to validated criteria. Estimates were computed as weighted proportions, taking the sampling design into account. RESULTS: The disease-specific prevalence rates (and 95% CIs) of RMDs in the adult Portuguese population were: low back pain, 26.4% (23.3% to 29.5%); periarticular disease, 15.8% (13.5% to 18.0%); knee osteoarthritis (OA), 12.4% (11.0% to 13.8%); osteoporosis, 10.2% (9.0% to 11.3%); hand OA, 8.7% (7.5% to 9.9%); hip OA, 2.9% (2.3% to 3.6%); fibromyalgia, 1.7% (1.1% to 2.1%); spondyloarthritis, 1.6% (1.2% to 2.1%); gout, 1.3% (1.0% to 1.6%); rheumatoid arthritis, 0.7% (0.5% to 0.9%); systemic lupus erythaematosus, 0.1% (0.1% to 0.2%) and polymyalgia rheumatica, 0.1% (0.0% to 0.2%). After multivariable adjustment, participants with RMDs had significantly lower EQ5D scores (ß=-0.09; p<0.001) and higher HAQ scores (ß=0.13; p<0.001) than participants without RMDs. RMDs were also significantly associated with the presence of anxiety symptoms (OR=3.5; p=0.006). CONCLUSIONS: RMDs are highly prevalent in Portugal and are associated not only with significant physical function and mental health impairment but also with poor HRQoL, leading to more health resource consumption. The EpiReumaPt study emphasises the burden of RMDs in Portugal and the need to increase RMD awareness, being a strong argument to encourage policymakers to increase the amount of resources allocated to the treatment of rheumatic patients.

8.
Rheumatology (Oxford) ; 51(11): 2020-6, 2012 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-22843791

RESUMO

OBJECTIVES: Adalimumab, etanercept and infliximab are effective TNF inhibitors (TNFis) in the treatment of RA, but no randomized clinical trials have compared the three agents. Prior observational data are not consistent. We compared their effectiveness over 1 year in a prospective cohort. METHODS: Analyses were performed on subjects' first episode of TNFi use in the Rheumatic Diseases Portuguese Register, Reuma.pt. The primary outcome was the proportion of patients with European League Against Rheumatism good response sustained at two consecutive observations separated by 3 months during the first year of TNFi use. Comparisons were performed using conventional adjusted logistic regression, as well as matching subjects across the three agents using a propensity score. In addition, baseline predictors of treatment response to TNFi were identified. RESULTS: The study cohort included 617 RA patients, 250 starting etanercept, 206 infliximab and 161 adalimumab. Good response was achieved by 59.6% for adalimumab, 59.2% for etanercept and 51.9% for infliximab (P = 0.21). The modelled probability of good response did not significantly differ across agents (etanercept vs adalimumab OR = 0.97, 95% CI 0.55, 1.71; etanercept vs infliximab OR = 1.25, 95% CI 0.74, 2.12; infliximab vs adalimumab OR = 0.80, 95% CI 0.47, 1.36). Matched propensity score analyses also showed no significant treatment response differences. Greater educational attainment was a predictor of better response, while smoking, presence of ACPA, glucocorticoid use and worse physician assessment of disease activity at baseline each predicted a reduced likelihood of treatment response. CONCLUSION: Over 1 year, we found no difference in effectiveness between adalimumab, etanercept and infliximab.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Imunoglobulina G/uso terapêutico , Receptores do Fator de Necrose Tumoral/uso terapêutico , Adalimumab , Adulto , Análise de Variância , Etanercepte , Feminino , Humanos , Infliximab , Masculino , Pessoa de Meia-Idade , Pontuação de Propensão , Estudos Prospectivos , Resultado do Tratamento , Fator de Necrose Tumoral alfa/antagonistas & inibidores
9.
Acta Reumatol Port ; 36(4): 385-8, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-22472929

RESUMO

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).


Assuntos
Artrite Reumatoide/tratamento farmacológico , Produtos Biológicos/uso terapêutico , Humanos , Portugal
10.
Clin Exp Rheumatol ; 28(3): 408-10, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-20576227

RESUMO

The use of tumour necrosis factor alpha (TNF-alpha) blockade to treat patients with both psoriasis and psoriatic arthritis (PsA) is now well established. However, paradoxical case reports of new-onset or exacerbation of psoriasis during all TNF-alpha blockers therapy have been published. We now review the literature and add a description of three PsA patients whose arthritis had responded well to TNF blockade but in whom major exacerbation of their psoriatic skin lesions occurred.


Assuntos
Antirreumáticos/efeitos adversos , Artrite Psoriásica/tratamento farmacológico , Imunoglobulina G/efeitos adversos , Psoríase/induzido quimicamente , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adulto , Etanercepte , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Psoríase/patologia , Receptores do Fator de Necrose Tumoral , Pele/patologia , Adulto Jovem
11.
Acta Reumatol Port ; 35(1): 95-8, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-20505635

RESUMO

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).


Assuntos
Artrite Reumatoide/tratamento farmacológico , Terapia Biológica , Humanos
12.
Rheumatology (Oxford) ; 49(8): 1502-4, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20427294

RESUMO

OBJECTIVE: To compare the clinical results after treatment with B-cell depleting therapy in patients with membranous (WHO Class V) vs proliferative (WHO Class III or IV) lupus nephritis (LN). METHODS: Data were compiled from two European centres on all patients with LN who were treated with i.v. rituximab (RTX) in a combination protocol with i.v. cyclophosphamide and steroids. Laboratory and serological evaluations were performed at 3, 6 and 12 months of follow-up. No immunosuppressive drugs were given before B-cell repopulation. RESULTS: Forty-three patients, 28 with proliferate and 15 with membranous LN by renal biopsy, were evaluated. Six months after treatment with RTX, both the membranous and the proliferative LN patients had a significant reduction in proteinuria and an increase in serum albumin. The main improvements were observed during the first 6 months and only minor non-significant changes in albumin and proteinuria were observed thereafter. As expected, the patients with membranous nephritis had lower anti-dsDNA titres and higher complement C3 levels at baseline, but in both groups a significant reduction in anti-dsDNA titre and improvements in complement C3 levels were seen during the first 6 months after treatment; the kinetics of improvement were similar in both groups. CONCLUSION: The clinical course following B-cell depleting therapy is strikingly similar between patients with membranous and those with proliferative LN. These observational data suggest that, if controlled studies confirm the efficacy of B-cell depleting therapy in proliferative nephritis, clinicians may reasonably consider such therapy in membranous LN.


Assuntos
Anticorpos Monoclonais Murinos/uso terapêutico , Linfócitos B/metabolismo , Ciclofosfamida/uso terapêutico , Imunossupressores/uso terapêutico , Nefrite Lúpica/tratamento farmacológico , Esteroides/uso terapêutico , Adolescente , Adulto , Ensaios Clínicos como Assunto , Estudos de Coortes , Progressão da Doença , Quimioterapia Combinada , Europa (Continente) , Seguimentos , Humanos , Nefrite Lúpica/classificação , Pessoa de Meia-Idade , Proteinúria/sangue , Rituximab , Albumina Sérica/análise , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
13.
Best Pract Res Clin Rheumatol ; 24(6): 829-40, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-21665129

RESUMO

The study of the descriptive epidemiology of chronic widespread pain (CWP) in several countries is of interest, as the occurrence of this condition varies among different populations. However, reports of pain prevalence are not consensual: it is clear that chronic musculoskeletal pain is frequent all over the world, varying from 4.2% to 13.3%. The reasons for the prevalence differences in CWP might include genetic and/or environmental factors. Multifactorial aetiopathogenesis of CWP and fibromyalgia syndrome (FMS) certainly includes genetic susceptibility and environmental influences. The risk factors for the occurrence and maintenance of CWP/FMS include female gender, increasing age, family history of chronic pain, several causes of distress, obesity and poorest mental and/or physical status. On the other hand, risk factors that negatively influence the outcome of CWP/FMS are: high levels of psychological distress, presence of somatisation, presence of fatigue, poor sleep, higher number of painful sites and pain intensity, poorest mental status and functional capacity, presence of co-morbid conditions and highest number of primary-care consultations. Mild alcohol consumption and individualised social support seem to have a protective effect on the outcome of CWP/FMS.


Assuntos
Fibromialgia/epidemiologia , Dor/epidemiologia , Causalidade , Doença Crônica , Feminino , Fibromialgia/fisiopatologia , Saúde Global , Humanos , Incidência , Masculino , Dor/fisiopatologia , Prevalência , Fatores de Risco , Taxa de Sobrevida
14.
Acta Reumatol Port ; 35(5): 475-96, 2010.
Artigo em Português | MEDLINE | ID: mdl-21245816

RESUMO

OBJECTIVE: The 3E (Evidence, Expertise, Exchange) Initiative is a multinational effort of rheumatologists aimed at developing evidence-based recommendations addressing specific questions relevant to clinical practice. The objective of the Portuguese contribution for the 3E Initiative was to develop evidence-based recommendations on how to investigate, follow-up and treat undifferentiated peripheral inflammatory arthritis (UPIA) adapted to local reality and develop additional recommendations considered relevant in the national context. METHODS: An international scientific committee from 17 countries selected a set of questions concerning the diagnosis and monitoring of UPIA using a Delphi procedure. Evidence-based answers to each question were sought by a systematic literature search, performed in Medline, Embase, the Cochrane Library and ACR/EULAR 2007-2009 meeting abstracts. Relevant articles were reviewed for quality assessment, data extraction and synthesis. In a national meeting, a panel of 63 Portuguese rheumatologists used the evidence which was gathered to develop recommendations, and filled the gaps in the evidence with their expert opinion. Finally, national recommendations were formulated and agreement among the participants was assessed. RESULTS: A total of 54754 references were identified, of which 267 were systematically reviewed. Thirteen national key recommendations about the investigation, follow-up and treatment of UPIA were formulated. One recommendation addressed differential diagnosis and investigations prior to the established operational diagnosis of UPIA, eight recommendations were related to the diagnostic and prognostic value of clinical and laboratory assessments in established UPIA (history and physical examination, acute phase reactants, serologies, autoantibodies, radiographs, magnetic resonance imaging and ultrasound, genetic markers and synovial biopsy), one recommendation highlighted predictors of persistence (chronicity), one addressed monitoring of clinical disease activity in UPIA, one aimed to find an useful method/score to predict a definitive diagnosis and the last one was related to treatment. CONCLUSION: Portuguese evidence-based recommendations for the management of UPIA in everyday practice were developed. Their dissemination and implementation in daily clinical practice should help to improve practice uniformity and optimize the management of UPIA patients.


Assuntos
Artrite/diagnóstico , Artrite/terapia , Humanos , Guias de Prática Clínica como Assunto
15.
Ann N Y Acad Sci ; 1173: 581-8, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19758203

RESUMO

Ankylosing spondylitis (AS) is a chronic inflammatory disease in which genetic factors play a central role. The efficacy of TNF blockers has reoriented research in this field in order to explain the influence of TNF in AS pathogenesis. The objective of this study was to access the influence of single nucleotide polymorphisms (SNPs) at positions -308 and -238 of the promoter region of TNF gene on AS susceptibility and prognosis. SNPs were determined by restriction fragment length polymorphisms in patients and controls. AS patients exhibited a decreased frequency of the A allele at position -238 (10%) when compared with controls (18%), suggesting that this could be a protective factor for disease susceptibility. In addition, the -308 GA/AA genotypes were associated with later disease onset in AS patients. These results suggest that TNF gene promoter polymorphisms at positions -238 and -308 could have a small influence on AS susceptibility and prognosis.


Assuntos
Polimorfismo de Nucleotídeo Único , Regiões Promotoras Genéticas/genética , Espondilite Anquilosante/genética , Fator de Necrose Tumoral alfa/genética , Adulto , Ensaio de Imunoadsorção Enzimática , Feminino , Frequência do Gene , Predisposição Genética para Doença/genética , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase , Polimorfismo de Fragmento de Restrição , Prognóstico , Índice de Gravidade de Doença , Espondilite Anquilosante/sangue , Espondilite Anquilosante/patologia , Fator de Necrose Tumoral alfa/sangue
16.
Acta Reumatol Port ; 34(2A): 276-80, 2009.
Artigo em Português | MEDLINE | ID: mdl-19569283

RESUMO

Juvenile dermatomyositis (JDM) is a rare systemic disease of unknown etiology characterized by inflammation of the muscle, skin and digestive tract, with variable outcome. The diagnostic criteria include proximal symmetrical muscular weakness, characteristic skin rashes, elevation of skeletal muscle enzymes and specific electromyographic and muscle biopsy abnormalities. Pulmonary and gastro-intestinal involvements, calcinosis and generalized edema usually indicate severe disease. Recent data suggest an association between the genotype -308 AA of the Tumour Necrosis Factor (TNF) gene and disease chronicity. We present a case of a 14 year-old female with JDM and generalized oedema which is a rare manifestation of the disease and it is associated to a poor outcome.


Assuntos
Dermatomiosite/complicações , Edema/complicações , Dermatopatias/complicações , Adolescente , Feminino , Humanos
17.
J Rheumatol ; 36(4): 837-42, 2009 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19208590

RESUMO

OBJECTIVE: Considering the relevance of tumor necrosis factor-alpha (TNF-alpha) in the pathophysiology of juvenile idiopathic arthritis (JIA), it is likely that polymorphisms in its promoter area may be relevant in disease susceptibility and activity. We investigated if clinical measures of JIA activity and TNF-alpha serum concentrations were associated with TNF-alpha -308 genotypes. METHODS: Portuguese patients with JIA in 5 pediatric rheumatology centers were recruited consecutively, along with a control group of healthy subjects. Demographic and clinical data and blood samples were collected from each patient. DNA was extracted for analysis of TNF-alpha gene promoter polymorphisms at position -308 by restriction fragment-length polymorphism. RESULTS: One hundred fourteen patients and 117 controls were evaluated; 57% of patients presented the oligoarticular subtype, 25% the polyarticular subtype, 8% the systemic subtype, and 9% had enthesitis-related arthritis and 5% psoriatic arthritis. Twenty-four percent of the patients presented the -308 GA/AA genotypes and 76% the -308 GG genotype, similar to findings in controls. Patients with the -308 GA/AA genotype had higher degree of functional impairment, erythrocyte sedimentation rate, 100-mm visual analog scale score for disease activity, and TNF-alpha levels compared to those with the -308 GG genotype. CONCLUSION: TNF-alpha -308 GA/AA genotypes were found to be related to higher inflammatory activity and worse measures of disease activity in Portuguese patients with JIA. They were not associated with susceptibility to JIA.


Assuntos
Artrite Juvenil , Genótipo , Polimorfismo de Nucleotídeo Único , Fator de Necrose Tumoral alfa , Adolescente , Artrite Juvenil/sangue , Artrite Juvenil/genética , Criança , Feminino , Predisposição Genética para Doença , Humanos , Portugal , Regiões Promotoras Genéticas/genética , Índice de Gravidade de Doença , Fator de Necrose Tumoral alfa/sangue , Fator de Necrose Tumoral alfa/genética
18.
Acta Reumatol Port ; 33(4): 430-4, 2008.
Artigo em Português | MEDLINE | ID: mdl-19078861

RESUMO

AIM: To verify if the response to TNFalpha inhibitors is influenced by the presence of IgM rheumatoid factor (RF), in patients with RA. MATERIAL AND METHODS: In this study, the patients with the diagnosis of RA treated with TNFa inhibitors followed in our hospital were recruited. A protocol was applied including demographic, clinical and laboratory data, in order to calculate DAS 28. The presence/absence of IgM RF and associated therapies were record. RESULTS: Fifty-seven patients, 52 female, with a mean duration of anti-TNFa treatment of 30,9+/-15,9 months were studied. Twenty-four patients were being treated with infliximab, 17 with adalimumab and 16 with etanercept. Forty-one patients had IgM RF detectable in serum (RF positive group). In the RF positive group, the variation of DAS 28 was -1,75 +/- 1,53 vs -1,04 +/- 1,76 in the RF negative group (p=0,135). The mean duration of anti-TNFalpha treatment was similar in both groups (31,9+/-15,9 vs 29,5+/-16,16 months). Patients who were treated with methotrexate presented a higher variation of DAS 28 (-1,87 +/- 1,70 vs -0,80 +/- 1,09; p=0,041) and this variation was dose dependent (p=0,056). CONCLUSIONS: Despite needing a replication in a larger cohort, our results suggest that the presence of IgM RF in the serum did not interfere with the response to treatment with TNFalpha inhibitors.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Reumatoide/imunologia , Imunoglobulina G/sangue , Fator Reumatoide/sangue , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adalimumab , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais Humanizados , Artrite Reumatoide/sangue , Artrite Reumatoide/tratamento farmacológico , Etanercepte , Feminino , Humanos , Imunoglobulina G/uso terapêutico , Infliximab , Masculino , Pessoa de Meia-Idade , Receptores do Fator de Necrose Tumoral/uso terapêutico , Estudos Retrospectivos
19.
Acta Reumatol Port ; 32(4): 381-4, 2007.
Artigo em Português | MEDLINE | ID: mdl-18159206

RESUMO

Among the many radiological findings seen in Rheumatoid Arthritis RA small subchondral geodes and erosions are typical. Large geodes are far less common abnormalities and their presence may indicate diagnostic and therapeutic difficulties. We present a case report of a 55-year old woman with seronegative RA that developed a large geode in the knee with extensive joint destruction.


Assuntos
Artrite Reumatoide/diagnóstico por imagem , Cistos/diagnóstico por imagem , Artropatias/diagnóstico por imagem , Artrite Reumatoide/complicações , Cistos/etiologia , Feminino , Humanos , Artropatias/etiologia , Pessoa de Meia-Idade , Radiografia
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