Carolina de Souza Müller

Mapping and predicting mortality from systemic sclerosis

Objectives To determine the causes of death and risk factors in systemic sclerosis (SSc). Methods Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres. The bootstrapping technique was used for internal validation. Results We identified 2719 French certificates of deaths related to SSc, mainly from cardiac (31%) and respiratory (18%) causes, and an increase in SSc-specific mortality over time. Over a median follow-up of 2.3 years, 1072 (9.6%) of 11 193 patients from the EUSTAR sample died, from cardiac disease in 27% and respiratory causes in 17%. By multiple variable analysis, a risk score was developed, which accurately predicted the 3-year mortality, with an area under the curve of 0.82. The 3-year survival of patients in the upper quartile was 53%, in contrast with 98% in the first quartile. Conclusion Combining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients’ survival.

Perfil de autoanticorpos e correlação clínica em um grupo de pacientes com esclerose sistêmica na região sul do Brasil

OBJECTIVES: To assess the manifestations of systemic sclerosis (SSc), with an emphasis on the analysis of autoantibodies and their clinical correlations, in a population of patients followed up at the SSc Outpatient Clinics of the Hospital de Clinicas of the Universidade Federal do Parana. METHODOLOGY: Cross-sectional study with 96 patients followed up at the SSc Outpatient Clinics of the hospital between September 2007 and September 2009. RESULTS: Most patients were of the female sex, in their forties or fifties, and the median time of disease was ten years. The limited cutaneous form of SSc was more prevalent. The analysis of the autoantibodies showed the association of anticentromere antibody (ACA) with the following: the limited form of SSc; more advanced age at the time of diagnosis; longer disease time; longer interval between the appearance of the Raynauds phenomenon (RyP) and the first non-RyP symptom; systemic arterial hypertension (SAH); and cardiac conduction blocks. The antitopoisomerase-1 antibody (ATA-1, previously called anti-Scl-70) was more common in the presence of the diffuse form of SSc, active disease, and digital ulcers. The anti-RNA polymerase III antibody (anti-Pol III) correlated with the diffuse form of SSc, disease activity, and synovitis. CONCLUSIONS: This study emphasizes and confirms the important role of autoantibodies in assessing patients with SSc, allowing the correlation between the autoimmune profile of patients with SSc and specific manifestations of the disease

Stiff skin syndrome versus scleroderma: a report of two cases

Stiff skin syndrome is a rare cutaneous disease, scleroderma-like disorder that presents in infancy or early childhood with rock-hard skin, limited joint mobility, and mild hypertrichosis. Normally, it occurs in the absence of visceral or muscle involvement. Patients do not present immunologic abnormalities or vascular hyperactivity. We describe two adults who initially were diagnosed suffering from scleroderma but fit criteria for stiff skin syndrome. A review of the clinical range of this disorder and discussion of the differential diagnosis with scleroderma is presented.

Manejo da dor

Aproximadamente, 98% dos pacientes que sao avaliados pelo reumatologista apresentam a dor como queixa principal. Porem, somente em 50% dos casos a dor e bem explicada pelo medico. Ate pouco tempo, a maioria dos livros-texto de reumatologia nao tratava da fisiopatologia e do tratamento da dor nas doencas reumaticas. Esta tendencia esta mudando, com cada vez mais artigos, conferencias e capitulos de livros analisando a dor como sintoma, dissecando os seus aspectos fisiopatologicos e estabelecendo estrategias de tratamento. Esta serie de artigos inclui um editorial chamando a atencao dos reumatologistas para o problema da dor, um artigo da dor na artrite reumatoide (AR), uma serie de artigos no uso de opioides na reumatologia e estudos sobre uma terapeutica nao-farmacologica (acupuntura) e uma nova terapeutica farmacologica.

Recomendações sobre diagnóstico e tratamento da esclerose sistêmica

Descricao do metodo de elaboracao das evidencias Os integrantes da Comissao de Esclerose Sistemica da Sociedade Brasileira de Reumatologia (bienio 2010-2012) participaram do Curso de Elaboracao de Evidencias da Associacao Medica Brasileira, em Sao Paulo, durante o primeiro semestre de 2011. As questoes foram propostas e discutidas pela internet, no segundo semestre de 2011. As 15 questoes clinicas consideradas relevantes foram estruturadas por meio da estrategia do P.I.C.O. (Paciente; Intervencao ou Indicador; Comparacao; Outcome). As estrategias de busca avaliaram as bases de dados MEDLINE, EMBASE, Scielo/Lilacs, Cochrane Library ate setembro de 2012 (Apendice). Os artigos selecionados na primeira estrategia de busca foram submetidos a avaliacao critica das evidencias, utilizando-se o escore de Jadad. Foram considerados tambem estudos observacionais e series de casos na ausencia de ensaios clinicos randomizados. Foi realizada insercao de estudos relevantes obtidos por busca manual. Posteriormente, foram elaboradas as respostas as perguntas das Recomendacoes, em que cada referencia bibliografica selecionada apresentava o correspondente grau de recomendacao e forca de evidencia cientifica. Para as Recomendacoes finais, as referencias bibliograficas foram atualizadas ate dezembro de 2012, redigidas em texto unico pelo coordenador, e submetidas aos coautores em quatro turnos, para elaboracao do texto final. Grau de recomendacao e forca de evidencia A: Estudos experimentais e observacionais de melhor consistencia. B: Estudos experimentais e observacionais de menor consistencia. C: Relatos de casos (estudos nao controlados). D: Opiniao desprovida de avaliacao critica, baseada em consensos, estudos fisiologicos ou modelos animais. Objetivo Estabelecer as recomendacoes para o manejo e para o tratamento da esclerose sistemica.

Recommendations for the management and treatment of systemic sclerosis.

Description of the method to elaborate the evidence The members of the Comissao de Esclerose Sistemica da Sociedade Brasileira de Reumatologia (Systemic Sclerosis Commission of the Brazilian Society of Rheumatology, SBR) 2010- 2012 took part in the Evidence Preparation Course given by the Associacao Medica Brasileira (Brazilian Medical Association, AMB) in Sao Paulo in the first semester of 2011. The questions were formulated and discussed via internet in the second semester of 2011. The 15 clinical questions considered to be relevant were structured using the P.I.C.O. method (patient; intervention or indicator; comparison; outcome). The literature search was conducted by searching the databases MEDLINE, EMBASE, SciElo/Lilacs, and the Cochrane Library through February, 2012 (Appendix). Critical assessment of the evidence in the selected articles was performed using the Jadad score. The observational studies and case series were considered for analysis when randomised clinical trials could not be located. A manual search located the relevant studies that were included in the analysis. The answers to the questions included in the Recommendations were refined and elaborated, and all of the selected references exhibited the corresponding grade of recommendation and the strength of scientific evidence. The references were updated through December, 2012, entered into a single file by the coordinator, and sent to the co-authors in four successive rounds for preparation of the final version. Grades of recommendation and strength of evidence A. Most consistent experimental and observational studies. B. Less consistent experimental and observational studies. C. Case reports (uncontrolled studies). D. Opinion that is not substantiated by critical evaluation, based on consensus, physiological studies or animal models. Objective To establish recommendations for the management and treatment of systemic sclerosis.

Envolvimento das musculaturas esquelética e cardíaca na esclerose sistêmica

Patients with systemic sclerosis (SSc) can have muscle involvement in the form of myositis or non-infl ammatory myopathy. The muscle involvement can be associated with left ventricular dysfunction (LVD) in patients with SSc, resulting in worse prognosis. Eighty-seven patients of the Hospital de Clinicas of the Universidade Federal do Parana, diagnosed with SSc, were assessed regarding the presence of skeletal muscle manifestations and their relation with LVD. A 42.5% prevalence of muscle involvement was observed in the patients studied, as well as a positive correlation with the diffuse form of the disease. Excluding other causes of LVD, three of the four patients with ejection fraction below the normal reference value had alteration of the muscle strength, atrophy and/or serum creatine phosphokinase (CPK) elevation.

Uso da capilaroscopia ungueal como método diagnóstico e prognóstico de rosácea

Abstract: B ACKGROUND : There is no appropriate and reliable method of evaluating and monitoring severityin rosacea. O BJECTIVE : To determine the importance of nailfold capillaroscopy as a diagnostic and prognostic methodfor patients with rosacea. M ETHODS : A cross-sectional study where eight patients with rosacea and 8 control subjects were submittedto nailfold capillaroscopy from May to July 2009. We collected clinical data related to gender, age, skin pho-totype, and rosacea stage according to Plewig and Kligman classification and the classification of theNational Rosacea Society. Additionally, we evaluated the progression of the disease and treatment therapiespreviously used. R ESULTS : The majority of the patients evaluated (6 out of 8 patients) had rosacea subtype I (vascular) or ery-thematotelangiectatic rosacea. The mean duration of the disorder was 5.96 years, and 87.5% of the patientswere under treatment with topical metronidazole. Nailfold cappilaroscopy showed that evidence of devas-cularization was absent in both groups. C

Pulmonary magnetic resonance imaging is similar to chest tomography in detecting inflammation in patients with systemic sclerosis

Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are prevalent complications of systemic sclerosis (SSc) and are currently the leading causes of death related to the disease. The accurate recognition of these conditions is therefore of utmost importance for patient management. A study was carried out with 24 SSc patients being followed at the Rheumatology Department of the Hospital de Clínicas of Universidade Federal do Paraná (UFPR) and 14 healthy volunteers, with the objective of evaluating the usefulness of lung magnetic resonance imaging (MRI) when assessing ILD in SS patients. The results obtained with lung MRI were compared to those obtained by computed tomography (CT) of the chest, currently considered the examination of choice when investigating ILD in SS patients. The assessed population was predominantly composed of women with a mean age of 50 years, limited cutaneous SS, and a disease duration of approximately 7 years. In most cases, there was agreement between the findings on chest CT and lung MRI. Considering it is a radiation-free examination and capable of accurately identifying areas of lung tissue inflammatory involvement, lung MRI showed to be a useful examination, and further studies are needed to assess whether there is an advantage in using lung MRI instead of chest CT when assessing ILD activity in SS patients.

Nailfold capillaroscopy in leprosy

Due to mounting evidences of interaction between Hansens bacilli with endothelial cells and the paucity of studies addressing the presence of nailfold capillaroscopic alterations in patients with Hansens disease, a study was carried out in order to verify the presence of capillaroscopic alterations in patients with leprosy in its various forms and its correlation with clinical parameters. Ten patients were evaluated at a specialized university service. Sixty percent of those had some capillaroscopic change, such as micro-hemorrhages, ectatic, bushy and corkscrew capillaries. Such changes were unspecific, which suggests there is not a specific pattern for this disease.