Self-management programs for hands in systemic sclerosis - literature review/ Programas de autogestão das mãos na esclerose sistémica - revisão de literatura

Introduction: Self-management programs are interesting strategies to prevent or reduce hand commitment in Systemic Sclerosis (SSc). Objective: Describe the current research on self-management hand care program for patients with SSc. Methods: We conducted a systematic literature review focusing on self-management programs for SSc. Results: The MEDLINE search allowed the identification of 13 papers, after applying the exclusion criteria, 8 papers was selected for our analyses. Conclusion: Although the small number of studies, there are promising data regarding the benefits of self-management programs for hands in SSc

Bisphosphonate-related osteonecrosis of the jaws

Bisphosphonates are potent inhibitors of bone resorption, and are used in the treatment of osteoporosis and other diseases that cause bone mass loss, such as Paget's disease, bone metastases, and multiple myeloma, to prevent pathological fractures. Since 2003, avascular osteonecrosis of the jaw has been associated with the use of bisphosphonates, mainly intravenous. According to the literature, the occurrence of osteonecrosis of the jaw has ranged from 0.8% to 12% of the patients on bisphosphonates, most of them on prolonged use. Physicians and odontologists should be aware of that potential complication in dental treatment.Os bisfosfonatos são potentes inibidores da reabsorção óssea e são utilizados no tratamento da osteoporose e de outras doenças que causam a perda de massa óssea, como doença de Paget, metástases ósseas e mieloma múltiplo, prevenindo fraturas patológicas. Desde 2003, estudos associam osteonecrose avascular dos ossos maxilares a seu uso, principalmente intravenoso. Na literatura, há relatos de ocorrência variando de 0,8% a 12%, dos pacientes, na sua maioria em uso prolongado. Médicos e dentistas devem estar cientes dessa potencial complicação no tratamento odontológico.40440

Pregnancy profile in ankylosing spondylitis

OBJECTIVE: To analyze the pregnancy profile in a group of female patients with ankylosing spondylitis (AS) followed at a referral university center. METHODS: Retrospective study analyzing 40 women with the diagnosis of AS according to the New York modified criteria followed at the Spondyloarthropathies Outpatient Clinic of the State University of Campinas in the period between 1990-2004. The number of pregnancies, deliveries and miscarriages was investigated, as well as the disease behavior during the pregnancy in those patients that got pregnant after AS onset. RESULTS: Among the 40 AS patients, 35 (87.5%) were Caucasians and 27 (67.5%) were HLA-B27 positive; 3 patients (7.5%) referred familial history of AS. The mean age of onset was 29.9 years old and the mean disease duration was 15.5 years. Nine patients (22.5%) never got pregnant; just one patient decided not to get pregnant because of AS. Thirty-one patients presented 83 pregnancies (an average of 2.7 pregnancies/patient), being seven after disease onset. There were 70 deliveries and 12 spontaneous miscarriages, but no miscarriages after AS onset. Among the 5 patients that got pregnant after disease onset, just 1 patient referred worsening of AS, related to hip involvement. CONCLUSIONS: In the present casuistic, fertility seemed not to be affected before disease onset and most patients decided not to get pregnant after AS onset.OBJETIVO: avaliar o perfil gestacional numa casuística de mulheres com espondilite anquilosante (EA) acompanhadas em um hospital universitário de referência. MÉTODOS: estudo retrospectivo avaliando 40 mulheres com EA, segundo os critérios modificados de New York, acompanhadas no Ambulatório de Espondiloartropatias da FCM-UNICAMP no período entre 1990 e 2004. Foram avaliados os dados referentes ao número de gestações, partos e abortos, bem como o comportamento da doença durante a gestação naquelas pacientes que engravidaram após o início da EA. RESULTADOS: dentre as 40 mulheres avaliadas, 35 (87,5%) eram brancas e 27 (67,5%) eram HLA-B27 positivo, sendo que somente três (7,5%) referiam história familiar de EA. A média de idade de início foi de 29,9 anos e o tempo médio de doença foi de 15,5 anos. Nove pacientes (22,5%) nunca haviam engravidado, sendo que apenas uma paciente decidiu não engravidar por causa da EA. Trinta e uma pacientes apresentaram 83 gestações (média de 2,7 gestações/paciente), sendo sete após o início da doença. Ocorreram 71 partos e 12 abortos espontâneos (nenhum após o início da doença). Dentre as cinco pacientes que engravidaram após o início da EA, apenas uma apresentou piora da doença, associada ao comprometimento das coxofemorais. CONCLUSÕES: a análise do perfil gestacional da presente casuística indica que a fertilidade parece não ter sido afetada antes do início da EA e que a maioria das pacientes decide não engravidar após o início da doença.10310

Characterization of uveitis in ankylosing spondylitis

OBJECTIVE: To analyze the clinical picture and outcome of uveitis in a series of 207 patients with ankylosing spondylitis (AS). METHODS: Retrospective study (1988-2001) analyzing 207 patients with diagnosis of AS according to the modified New York criteria. All patients were submited to complete clinical (axial and peripheral involvement, heel enthesopathies, extra-articular manifestations) and radiological (sacroiliac, lumbar, dorsal and cervical spine) investigation, HLA-B27 evaluation (and respective alleles, whenever possible), were searched. These data were compared with the occurrence of uveitis during the follow-up of the AS patients. RESULTS: Thirty patients (14.5%) presented 55 episodes of acute unilateral anterior uveitis. Twenty seven patients had active articular disease at the moment of the uveitis crisis, while three patients were inactive. One patient, in treatment of lung tuberculosis, presented a single episode of posterior uveitis. Among the uveitis in AS patients, there was predominance of male sex (82.6%), Caucasoid race (77.8%), adult-onset AS (83.1%), positive HLA-B27 (79.2%) and absence of familial history of AS (84.5%). There was statistical association between uveitis and juvenile-onset AS (p = 0.0094) and achillean (p = 0.0003) and plantar (p = 0.0067) enthesopathies. No patient presented severe ophthalmologic sequelae of uveitis. Sulfasalazine (1 to 2 g, daily) was prescribed to 16 patients, for a minimum period of six months. CONCLUSIONS: Acute anterior uveitis was common in the follow-up of AS patients in this casuistic, associated to the juvenile-onset of the disease and to the enthesophatic involvement of the lower limbs.OBJETIVO: analisar a apresentação clínica e a evolução da uveíte em uma população de 207 pacientes com espondilite anquilosante (EA). MÉTODOS: estudo retrospectivo (1988-2001) analisando 207 pacientes com o diagnóstico de EA segundo os critérios de Nova York modificados. Todos apresentavam investigação clínica (envolvimento axial e periférico, entesopatias, manifestações extra-articulares) e radiológica (sacroilíaca, coluna lombar, dorsal e cervical) completas, além da pesquisa do HLA-B27 (com seus respectivos alelos, quando possível). Os dados foram comparados com a presença de uveíte durante o período de seguimento dos pacientes. RESULTADOS: trinta pacientes (14,5%) apresentaram 55 episódios de uveíte anterior aguda unilateral durante o seguimento ambulatorial. Vinte e sete pacientes encontravam-se em atividade articular da EA no momento da crise de uveíte, enquanto três pacientes encontravam-se inativos do ponto de vista articular. Um único paciente, em tratamento de tuberculose pulmonar, apresentou um episódio de uveíte posterior. Entre os pacientes com uveíte, houve predomínio de sexo masculino (82,6%), raça caucasóide (77,8%), início da EA na idade adulta (83,1%), HLA-B27 positivo (79,2%), sem história familiar de EA (84,5%). Houve associação estatística entre uveíte e idade de início juvenil (p = 0,0094) e entesopatias aquileana (p = 0,0003) e plantar (p = 0,0067). Nenhum paciente apresentou seqüela ocular grave da uveíte. Dezesseis pacientes fizeram uso de sulfassalazina (1,0 g/dia a 2,0 g/dia), por prazo mínimo de seis meses. CONCLUSÕES: a uveíte anterior aguda foi comum na evolução da EA nesta casuística, estando associada preferencialmente à EA de início juvenil e ao acometimento articular entesopático de membros inferiores.34334

Association analysis of anti-Epstein-Barr nuclear antigen-1 antibodies, anti-cyclic citrullinated peptide antibodies, the shared epitope and smoking status in Brazilian patients with rheumatoid arthritis

INTRODUCTION: Epstein-Barr virus exposure appears to be an environmental trigger for rheumatoid arthritis that interacts with other risk factors. Relationships among anti-cyclic citrullinated peptide antibodies, the shared epitope, and smoking status have been observed in patients with rheumatoid arthritis from different populations. OBJECTIVE: To perform an association analysis of anti-Epstein-Barr nuclear antigen-1 antibodies, anti-cyclic citrullinated peptide antibodies, the shared epitope, and smoking status in Brazilian patients with rheumatoid arthritis. METHODS: In a case-control study, 140 rheumatoid arthritis patients and 143 healthy volunteers who were matched for age, sex, and ethnicity were recruited. Anti-Epstein-Barr nuclear antigen-1 antibodies and anti-cyclic citrullinated peptide antibodies were examined using an enzyme-linked immunosorbent assay, and shared epitope alleles were identified by genotyping. Smoking information was collected from all subjects. A comparative analysis of anti-Epstein-Barr nuclear antigen-1 antibodies, anti-cyclic citrullinated peptide antibodies, the shared epitope, and smoking status was performed in the patient group. Logistic regression analysis models were used to analyze the risk of rheumatoid arthritis. RESULTS: Anti-Epstein-Barr nuclear antigen-1 antibodies were not associated with anti-cyclic citrullinated peptide antibodies, shared epitope alleles, or smoking status. Anti-cyclic citrullinated peptide antibody positivity was significantly higher in smoking patients with shared epitope alleles (OR = 3.82). In a multivariate logistic regression analysis using stepwise selection, only anti-cyclic citrullinated peptide antibodies were found to be independently associated with rheumatoid arthritis (OR = 247.9). CONCLUSION: Anti-Epstein-Barr nuclear antigen-1 antibodies did not increase the risk of rheumatoid arthritis and were not associated with the rheumatoid arthritis risk factors studied. Smoking and shared epitope alleles were correlated with anti-cyclic citrullinated peptide-antibody-positive rheumatoid arthritis. Of the risk factors, only anticyclic citrullinated peptides antibodies were independently associated with rheumatoid arthritis susceptibility

Rheumatoid pneumoconiosis (Caplan's syndrome) with a classical presentation

Although rare, rheumatoid pneumoconiosis, also known as Caplan's syndrome, can occur in workers exposed to silica, as well as in patients with silicosis, coal workers' pneumoconiosis or asbestosis. Prevalence is higher among patients with silicosis, despite the fact that it was originally described in coal workers with pneumoconiosis. The classical finding that defines this syndrome is that of rheumatoid nodules in the lungs, regardless of whether there are small rounded opacities suggestive of pneumoconiosis or large opacities consistent with massive pulmonary fibrosis, with or without clinical rheumatoid arthritis. We describe the case of a female patient with rheumatoid arthritis, diagnosed 34 years after 7 years of occupational exposure to silica at a porcelain plant. A chest X-ray showed circular opacities of 1-5 cm in diameter, bilaterally distributed at the periphery of the lungs. A CT-guided thoracic punch biopsy of one of those nodules revealed that it was rheumatoid nodule surrounded by a palisade of macrophages, which is typical of Caplan's syndrome. Aspects of diagnosis, classification and occurrence of this syndrome are discussed, emphasizing the importance of the occupational anamnesis of patients with rheumatoid arthritis and lung opacities on chest X-rays.Apesar de rara, a pneumoconiose reumatoide, também chamada de síndrome de Caplan, pode ser diagnosticada entre trabalhadores expostos à sílica e entre pacientes com silicose, pneumoconiose dos mineiros de carvão e asbestose. A maior prevalência ocorre entre os silicóticos, apesar de ter sido descrita inicialmente em mineiros de carvão com pneumoconiose. O achado que define o tipo clássico da síndrome é a presença de nódulos reumatoides nos pulmões, independente da presença ou não de pequenas opacidades pneumoconióticas, ou de grandes opacidades de fibrose pulmonar maciça, associada ou não a um quadro de artrite reumatoide em atividade. Relatamos o caso de uma mulher com quadro de artrite reumatoide, diagnosticada 34 anos após exposição à sílica livre em uma indústria de porcelana por 7 anos, apresentando radiograma de tórax com opacidades arredondadas de 1 a 5 cm de diâmetro, distribuídas na periferia de ambos os pulmões. A biópsia transtorácica guiada por TC de um dos nódulos revelou tratar-se de nódulo reumatoide com macrófagos em paliçada, típico da síndrome de Caplan. São discutidos aspectos de diagnóstico da síndrome, classificação e ocorrência, ressaltando a importância da anamnese ocupacional em casos de artrite reumatoide com opacidades radiológicas pulmonares.94294

Coexistence of hypothyroidism and polymyositis

Patients with hypothyroidism frequently present with musculoskeletal complaints and elevated serum levels of muscle enzymes, like creatine kinase (CK). With adequate hormonal treatment, the maintenance of high values of CK and muscle symptoms are uncommon. This work reports a 47-year-old woman with hypothyroidism, proximal muscle weakness and increased CK, who didn't improved with levothyroxine treatment. The diagnosis of polymyositis was based on electromyography and muscle biopsy. After treatment with prednisone plus methotrexate and increase of the levothyroxine dose, there were a clinical improvement and a normalization of CK values.Pacientes com hipotiroidismo freqüentemente apresentam sintomas músculo-esqueléticos e aumento de enzimas musculares, como a creatinaquinase (CK). Com o tratamento hormonal adequado é incomum ocorrer grandes aumentos de CK e manutenção de queixas musculares. Este trabalho relata o caso de uma paciente com hipotiroidismo, queixa de fraqueza muscular proximal e aumento de CK, sem melhora clínica com uso de levotiroxina. O diagnóstico de polimiosite foi confirmado por eletroneuromiografia e biópsia muscular. Após tratamento com prednisona e metotrexato, além de adequação da dose de levotiroxina, houve melhora clínica significativa e normalização da CK.33033

Low prevalence of renal, cardiac, pulmonary, and neurological extra-articular clinical manifestations in spondyloarthritis: analysis of the Brazilian Registry of Spondyloarthritis

OBJECTIVE: To describe the extra-articular manifestations (cardiac, renal, pulmonary, and neurological), usually not related to spondyloarthritis (SpA), in a large cohort of Brazilian patients. MATERIALS AND METHODS: This retrospective study analyzed 1,472 patients diagnosed with SpA and cared for at 29 health care centers distributed in the five major geographic regions in the country, participating in the Brazilian Registry of Spondyloarthritis (BRS). All patients were assessed for the prevalence of major extra-articular manifestations (cardiac, renal, pulmonary, and neurological), classified according to the diagnosis [ankylosing spondylitis (AS), psoriatic arthritis (PsA), reactive arthritis (ReA), arthritis associated with inflammatory bowel disease (IBD), undifferentiated spondyloarthritis (uSpA), and juvenile SpA], and according to the clinical presentation (axial, peripheral, mixed, and enthesitis). RESULTS: Of the patients with SpA assessed, 963 had AS, 271 PsA, 49 ReA, 48 arthritis associated with IBD, 98 uSpA, and 43 juvenile SpA. Cardiac involvement was reported in 44 patients (3.0%), pulmonary involvement in 19 (1.3%), renal involvement in 17 (1.2%), and neurological involvement in 13 patients (0.9%). Most patients with visceral involvement had AS or PsA, and the mixed (axial + peripheral) and/or predominantly axial clinical form. CONCLUSION: Cardiac, renal, pulmonary, and neurological extra-articular manifestations are quite infrequent in SpA, ranging from 0.9% to 3% in this large Brazilian cohort, and affected predominantly patients with AS and PsA.OBJETIVO: Descrever as manifestações extra-articulares (cardíacas, renais, pulmonares e neurológicas) geralmente não relacionadas às espondiloartrites (EpA) em uma grande coorte de pacientes brasileiros. MÉTODOS: Este estudo retrospectivo analisou 1.472 pacientes com o diagnóstico de EpA atendidos em 29 centros distribuídos pelas cinco principais regiões geográficas do Brasil, integrantes do Registro Brasileiro de Espondiloartrites. Todos os pacientes foram avaliados para a prevalência das principais manifestações extra-articulares (cardíacas, renais, pulmonares e neurológicas), divididas por diagnóstico [espondilite anquilosante (EA), artrite psoriásica (AP), artrite reativa (ARe), artrite associada a doença inflamatória intestinal (DII), EpA indiferenciada (EI) e EpA juvenil] e por forma clínica (axial, periférica, mista e entesítica). RESULTADOS: Dentre os pacientes avaliados com EpA, 963 apresentavam EA, 271 AP, 49 ARe, 48 artrite associada a DII, 98 EI e 43 EpA juvenil. Acometimento cardíaco foi observado em 44 pacientes (3,0%), seguido por acometimento pulmonar em 19 (1,3%), renal em 17 (1,2%) e neurológico em 13 pacientes (0,9%). A maioria dos casos de acometimento visceral ocorreu nos pacientes com EA ou AP e naqueles com forma clínica mista (axial e periférica) e/ou predominantemente axial. CONCLUSÃO: As manifestações extra-articulares cardíacas, renais, pulmonares e neurológicas são muito pouco frequentes nas EpA, variando de 0,9%-3% nesta grande coorte brasileira, estando mais associadas a EA e AP.37938

Quality of life in spondyloarthritis : analysis of a large Brazilian cohort

Objetivo: analisar as variáveis demográficas e clínicas associadas à diminuição da qualidade de vida em uma grande coorte brasileira de pacientes com espondiloartrite (EpA). Métodos: Foi aplicado um protocolo de pesquisa único a 1.465 pacientes brasileiros classificados como tendo EpA de acordo com os critérios do European Spondyloarthropaties Study Group (ESSG), atendidos em 29 centros de referência em reumatologia do Brasil. Foram registradas as variáveis clínicas e demográficas. A qualidade de vida foi analisada por meio do questionário Ankylosing Spondylitis Quality of Life (ASQoL). Resultados: A pontuação média do ASQoL foi de 7,74 (+ 5,39). Ao analisar doenças específicas no grupo de EpA, as pontuações do ASQoL não apresentaram diferença estatisticamente significativa. Os dados demográficos mostraram piores escores de ASQoL associados ao gênero feminino (p = 0,014) e etnia negra (p < 0,001). Quanto aos sintomas clínicos, a dor na região glútea (p = 0,032), a dor cervical (p < 0,001) e a dor no quadril (p = 0,001), estiveram estatisticamente associadas a piores escores no ASQoL. O uso contínuo de fármacos anti-inflamatórios não esteroides (p < 0,001) e agentes biológicos (p = 0,044) esteve associado a escores mais elevados de ASQoL, enquanto outros medicamentos não interferiram nos escores do ASQoL. Conclusão: Nesta grande série de pacientes com EpA, o sexo feminino e a etnia negra, bem como sintomas predominantemente axiais, estiveram associados a uma qualidade de vida reduzida.Objective: to analyze quality of life and demographic and clinical variables associated to its impairment in a large Brazilian cohort of patients with spondyloarthritis (SpA). Methods: A common protocol of investigation was applied to 1465 Brazilian patients classified as SpA according to the European Spondyloarthropaties Study Group (ESSG) criteria, attended at 29 reference centers for Rheumatology in Brazil. Clinical and demographic variables were recorded. Quality of life was analyzed through the Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire. Results: The mean ASQoL score was 7.74 (± 5.39). When analyzing the specific diseases in the SpA group, the ASQoL scores did not present statistical significance. Demographic data showed worse scores of ASQoL associated with female gender (p = 0.014) and African-Brazilian ethnicity (p < 0.001). Regarding clinical symptoms, buttock pain (p = 0.032), cervical pain (p < 0.001) and hip pain (p = 0.001), were statistically associated with worse scores of ASQoL. Continuous use of nonsteroidal anti-inflammatory drugs (p < 0.001) and biologic agents (p = 0.044) were associated with higher scores of ASQoL, while the other medications did not interfere with the ASQoL scores. Conclusion: In this large series of patients with SpA, female gender and African-Brazilian ethnicity, as well as predominant axial symptoms, were associated with impaired quality of life