It's time to treat systemic sclerosis

20120

Paul Klee, um artista esclerodérmico

707

Study about different clinical variants and skin scores in systemic sclerosis

OBJECTIVE: To report the experience using the Total Skin Score (TSS), in the original (OR-TSS) and modified (MR-TSS) Rodnan methods in systemic sclerosis (SSc), analysing its clinical significance in the model of two (diffuse and limited) and three (diffuse, intermediate and limited) clinical variants. METHODS: Prospective study analysing skin thickness (assessed by original and modified Rodnan TSS methods) in 56 SSc patients. TSS results were compared with SSc clinical variants (subdivided in the model of two and three subgroups) as well as with cutaneous (calcinosis, telangiectasias, pigmentary abnormalities), systemic (articular, vascular, esophageal, pulmonary, cardiac, renal) and laboratory (antinuclear antibody, anticentromere antibody, anti-topoisomerase I antibody) manifestations. Data were analysed based on the presence of a low (= 20) or a high TSS (> 20) by both methods. Logistic regression analysis was performed to determine the independent variables that influenced TSS (in or by both methods) and death (after a 5-year follow-up). RESULTS: There was statistical association between TSS = 20 and limited SSc (both methods in the SSc model of two subgroups), calcinosis (MR-TSS) and anticentromere antibody (OR-TSS, trend). TSS > 20 was associated with diffuse SSc (both methods in the SSc models of two and three subgroups), pigmentary abnormalities (both methods), articular involvement (both methods), pulmonary restrictive disease (MR-TSS, trend) and anti-topoisomerase I antibody (OR-TSS, trend). Multivariate logistic regression analysis indicated that the SSc clinical variant was the most important factor that influenced significantly TSS (in both methods). After a 5-year follow-up, there were 20 deaths (35.7%); multivariate logistic regression analysis indicated that vascular involvement influenced death significantly, despite the TSS score. CONCLUSIONS: TSS, by both original and modified Rodnan methods, permits to differentiate distinct patterns in the clinical spectrum of SSc. In the model of two subgroups, there is a clear distinction between diffuse and limited disease, whereas in the model of three subgroups this distinction is not so clearly observed between the variants.OBJETIVO: Descrever a experiência com o uso do escore cutâneo total (ECT), utilizando-se os métodos de Rodnan original (ECTRO) e modificado (ECT-RM) na esclerose sistêmica (ES), analisando seu significado clínico nos modelos de duas (difusa e limitada) e três (difusa, intermediária e limitada) formas clínicas. MÉTODOS: Estudo prospectivo analisando o espessamento cutâneo (obtido através do ECT pelos métodos de Rodnan original e modificado) em 56 pacientes com ES. Os resultados do ECT foram comparados com as formas clínicas da ES (nos modelos de duas e três formas clínicas), bem como com manifestações cutâneas (calcinose, telangiectasias, distúrbios de pigmentação), sistêmicas (articular, vascular, esofágica, pulmonar, cardíaca, renal) e alterações laboratoriais (fator antinuclear, anticorpo anticentrômero, anticorpo anti-topoisomerase I). Os resultados do ECT também foram subdivididos em ECT baixo (< 20) e ECT elevado (&gt; 20), em ambos os métodos. Análise de regressão logística foi realizada para se determinar quais as variáveis independentes que mais influenciaram ECT e óbito (após cinco anos de seguimento). RESULTADOS: Houve associação estatística entre ECT < 20 e ES limitada (ambos os métodos de ECT no modelo com duas formas clínicas), calcinose (ECT-RM) e anticorpo anticentrômero (ECT-RO, tendência). ECT &gt; 20 esteve associado com ES difusa (ambos os métodos de ECT nos modelos de duas e três formas clínicas), distúrbios de pigmentação (ambos os métodos de ECT), acometimento articular (ambos os métodos de ECT), doença pulmonar restritiva (ECT-RM, tendência) e anticorpo anti-topoisomerase I (ECT-RO, tendência). Análise de regressão logística multivariada indicou que a forma clínica foi a variável que influenciou de maneira mais significativa o ECT (em ambos os métodos de ECT). Após seguimento de cinco anos, ocorreram 20 óbitos (35.7%); análise de regressão logística multivariada indicou que o acometimento vascular foi a variável que mais influenciou o óbito, independente do ECT. CONCLUSÕES: O ECT, nos métodos de Rodnan original e modificado, permitiu diferenciar distintos padrões de doença no espectro clínico da ES. No modelo de duas formas clínicas existem claras distinções entre a ES limitada e a ES difusa, enquanto que no modelo de três formas clínicas esta distinção não é tão claramente observada.010

Erectile dysfunction in systemic sclerosis

The aim of this report is to analyze the incidence of erectile dysfunction (ED) and the usefulness of the International Index of Erectile Function (IIEF) on the investigation of ED in men with systemic sclerosis (SSc). Fifteen male SSc patients were asked about the presence of ED symptoms and were submitted to the IIEF questionnaire, after obtaining informed consent. Their medical data were reviewed in the search of other possible causes of ED. The studied patients were predominantly Caucasian and had diffuse SSc. ED was referred by 67% of them, and it was first noted after an average of 29 months of SSc onset; four patients referred concomitant onset of ED and SSc. All men with ED detected by IIEF questionnaire also reported ED when directly asked, and all men who affirmed having ED were also identified by IIEF. There were no clear association between ED and extent of cutaneous involvement, visceral involvement, drugs, total skin score and disease duration. Five patients with ED also had other causes of impotence. ED affects a high percentage of male SSc patients, and the IIEF may be a valid instrument in its evaluation. The high incidence of ED in young SSc patients and the occasional concomitance of the onset of SSc and ED warrants further evaluation of a causal association.O objetivo deste trabalho é analisar a prevalência da disfunção erétil (DE) e a utilidade do Índice Internacional de Função Erétil (IIFE) na investigação da DE em pacientes com esclerose sistêmica (ES). Quinze homens com ES foram questionados acerca dos sintomas da DE e submetidos ao questionário de IIFE. Seus prontuários foram revisados para identificar outras possíveis causas da DE. Os pacientes estudados eram predominantemente caucasóides e tinham ES difusa. DE foi referida por 67% dos pacientes, iniciando-se, em média, 29 meses após o início da ES; quatro pacientes relataram início concomitante da DE e da ES. O diagnóstico da DE pelo IIFE e por interrogação direta foi concordante em todos os pacientes. Não foi encontrada associação nítida entre DE e extensão do envolvimento cutâneo, envolvimento visceral, drogas, escore cutâneo total e duração da doença. Cinco pacientes com DE também tinham outras causas de impotência. A DE afeta um grande porcentual de homens acometidos por esclerose sistêmica e o IIFE pode ser um instrumento válido em sua avaliação. A alta incidência da DE em jovens com ES e a eventual concomitância do início da ES e da DE enfatizam a necessidade de estudos posteriores sobre uma provável relação causal.949

Surgical treatment of gastroesophageal reflux disease in systemic sclerosis

OBJECTIVE: The authors describe the experience with the surgical treatment of gastroesophageal reflux disease (GERD) in 10 patients with systemic sclerosis (SSc). METHODS: Criteria for surgery included GERD with severe chronic esophagitis and stricture, treated previously with recommended doses of antisecretory (ranitidine and/or omeprazole) and prokinetic (cisapride) drugs for more than six months, without significant improvement. All patients were female and 8 were Caucasian, with 7 of them presenting limited SSc and 3, diffuse SSc. RESULTS: Surgical treatment was performed through videolaparoscopy in 9 patients and open surgery in 1 patient. Seven patients underwent modified Nissen technique and 3 patients, Lind technique. Significant esophageal strictures needed preoperative dilatations in 6 patients. Postoperative assessment three months after surgery revealed that 70% of the patients had a favourable outcome, with significant improvement in the heartburn and dysphagia; 1 patient needed a reoperation due to a paraesophageal hernia (Roux-en-Y gastrectomy) in the postoperative period. A good outcome was referred by 80% of the patients one year after surgery and by 70% of them two years after surgery, occurring two deaths. CONCLUSIONS: The authors conclude that the surgical treatment of GERD represents an efficient therapeutic option in SSc patients with severe esophagitis and stricture.OBJETIVO: Os autores descrevem sua experiência com o tratamento cirúrgico da doença de refluxo gastroesofágico (DRGE) em pacientes com esclerose sistêmica (ES). MÉTODOS: Foram selecionados 10 pacientes com DRGE que apresentavam esofagite grave e estenose esofágica, tratados previamente com doses recomendadas de drogas anti-secretórias (ranitidina e/ou omeprazol) e pró-cinéticas (cisapride) por mais de seis meses, sem melhora significativa. Todos os pacientes eram do sexo feminino e 8 eram caucasóides, sendo 7 com ES limitada e 3 com ES difusa. RESULTADOS: O tratamento cirúrgico foi realizado através de videolaparoscopia em 9 pacientes e por cirurgia aberta no outro paciente. Sete pacientes foram submetidos à técnica de Nissen modificada e 3 à técnica de Lind. Seis pacientes com estenose esofágica significativa necessitaram de dilatações endoscópicas no período pré-operatório. Avaliação pós-operatória três meses após a cirurgia revelou que 70% dos pacientes apresentaram resultado favorável, com melhora significativa da azia e da disfagia; 1 paciente necessitou de nova intervenção cirúrgica em conseqüência de uma hérnia paraesofágica no período pós-operatório, sendo realizada uma gastrectomia em Y de Roux. Uma boa evolução foi referida por 80% dos pacientes um ano após cirurgia e por 70% dois anos após cirurgia, observando-se dois óbitos. CONCLUSÕES: Os autores concluem que o tratamento cirúrgico da DRGE representa uma eficiente opção terapêutica em pacientes com ES e esofagite grave com estenose.9810

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

2016 update of the ASAS-EULAR management recommendations for axial spondyloarthritis

To update and integrate the recommendations for ankylosing spondylitis and the recommendations for the use of tumour necrosis factor inhibitors (TNFi) in axial spondyloarthritis (axSpA) into one set applicable to the full spectrum of patients with axSpA. Following the latest version of the European League Against Rheumatism (EULAR) Standardised Operating Procedures, two systematic literature reviews first collected the evidence regarding all treatment options (pharmacological and non-pharmacological) that were published since 2009. After a discussion of the results in the steering group and presentation to the task force, overarching principles and recommendations were formulated, and consensus was obtained by informal voting. A total of 5 overarching principles and 13 recommendations were agreed on. The first three recommendations deal with personalised medicine including treatment target and monitoring. Recommendation 4 covers non-pharmacological management. Recommendation 5 describes the central role of non-steroidal anti-inflammatory drugs (NSAIDs) as first-choice drug treatment. Recommendations 6-8 define the rather modest role of analgesics, and disprove glucocorticoids and conventional synthetic disease-modifying antirheumatic drugs (DMARDs) for axSpA patents with predominant axial involvement. Recommendation 9 refers to biological DMARDs (bDMARDs) including TNFi and IL-17 inhibitors (IL-17i) for patients with high disease activity despite the use (or intolerance/contraindication) of at least two NSAIDs. In addition, they should either have an elevated C reactive protein and/or definite inflammation on MRI and/or radiographic evidence of sacroiliitis. Current practice is to start with a TNFi. Switching to another TNFi or an IL-17i is recommended in case TNFi fails (recommendation 10). Tapering, but not stopping a bDMARD, can be considered in patients in sustained remission (recommendation 11). The final two recommendations (12, 13) deal with surgery and spinal fractures. The 2016 Assessment of SpondyloArthritis international Society-EULAR recommendations provide up-to-date guidance on the management of patients with axSpA

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