Connective tissue disease-associated interstitial lung disease.

Connective tissue disease-associated interstitial lung disease (CTD-ILD) represents a group of systemic autoimmune disorders characterized by immune-mediated organ dysfunction. Systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myositis, and Sjögren's syndrome are the most common CTDs that present with pulmonary involvement, as well as with interstitial pneumonia with autoimmune features. The frequency of CTD-ILD varies according to the type of CTD, but the overall incidence is 15%, causing an important impact on morbidity and mortality. The decision of which CTD patient should be investigated for ILD is unclear for many CTDs. Besides that, the clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. A significant proportion of patients will present with a more severe and progressive disease, and, for those, immunosuppression with corticosteroids and cytotoxic medications are the mainstay of pharmacological treatment. In this review, we summarized the approach to diagnosis and treatment of CTD-ILD, highlighting recent advances in therapeutics for the various forms of CTD.

Connective tissue disease-associated interstitial lung disease / Doença pulmonar intersticial associada a doença do tecido conjuntivo

ABSTRACT Connective tissue disease-associated interstitial lung disease (CTD-ILD) represents a group of systemic autoimmune disorders characterized by immune-mediated organ dysfunction. Systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myositis, and Sjögren's syndrome are the most common CTDs that present with pulmonary involvement, as well as with interstitial pneumonia with autoimmune features. The frequency of CTD-ILD varies according to the type of CTD, but the overall incidence is 15%, causing an important impact on morbidity and mortality. The decision of which CTD patient should be investigated for ILD is unclear for many CTDs. Besides that, the clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. A significant proportion of patients will present with a more severe and progressive disease, and, for those, immunosuppression with corticosteroids and cytotoxic medications are the mainstay of pharmacological treatment. In this review, we summarized the approach to diagnosis and treatment of CTD-ILD, highlighting recent advances in therapeutics for the various forms of CTD.

RESUMO Doença pulmonar intersticial associada à doença do tecido conjuntivo (DPI-DTC) representa um grupo de distúrbios autoimunes sistêmicos caracterizados por disfunção de órgãos imunomediada. Esclerose sistêmica, artrite reumatoide, miosite inflamatória idiopática e síndrome de Sjögren são as DTC mais comuns que apresentam acometimento pulmonar, bem como pneumonia intersticial com achados autoimunes. A frequência de DPI-DTC varia de acordo com o tipo de DTC, mas a incidência total é de 15%, causando um impacto importante na morbidade e mortalidade. A decisão sobre qual paciente com DTC deve ser investigado para DPI não é clara para muitas DTC. Além disso, o espectro clínico pode variar desde achados assintomáticos em exames de imagem até insuficiência respiratória e morte. Parte significativa dos pacientes apresentará doença mais grave e progressiva, e, para esses pacientes, imunossupressão com corticosteroides e medicamentos citotóxicos são a base do tratamento farmacológico. Nesta revisão, resumimos a abordagem do diagnóstico e tratamento de DPI-DTC, destacando os recentes avanços na terapêutica para as diversas formas de DTC.

Position article and guidelines 2018 recommendations of the Brazilian Society of Rheumatology for the indication, interpretation and performance of nailfold capillaroscopy

Abstract Nailfold capillaroscopy (NFC) is a reproducible, simple, low-cost, and safe imaging technique used for morphological analysis of nail bed capillaries. It is considered to be extremely useful for the investigation of Raynaud's phenomenon and for the early diagnosis of systemic sclerosis (SSc). The capillaroscopic pattern typically associated with SSc, scleroderma ("SD") pattern, is characterized by dilated capillaries, microhemorrhages, avascular areas and/or capillary loss, and distortion of the capillary architecture. The aim of these recommendations is to provide orientation regarding the relevance of NFC, and to establish a consensus on the indications, nomenclature, the interpretation of NFC findings and the technical equipments that should be used. These recommendations were formulated based on a systematic literature review of studies included in the database MEDLINE (PubMed) without any time restriction.

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.

Pulmonary magnetic resonance imaging is similar to chest tomography in detecting inflammation in patients with systemic sclerosis / Ressonância magnética pulmonar é semelhante à tomografia de tórax para detectar inflamação em pacientes com esclerose sistêmica

Abstract 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.

Resumo A doença intersticial pulmonar (DIP) e a hipertensão arterial pulmonar (HAP) são complicações prevalentes na esclerose sistêmica (ES) e constituem atualmente as principais causas de morte relacionadas à doença. O reconhecimento preciso dessas condições é, portanto, de fundamental importância no manejo dos pacientes. Fez-se um estudo com 24 pacientes com ES em acompanhamento no serviço de reumatologia do Hospital de Clínicas da Universidade Federal do Paraná (UFPR) e 14 voluntários sadios com objetivo de avaliar a utilidade do exame de ressonância magnética (RM) do pulmão na avaliação da DIP em pacientes com ES. Os resultados obtidos com a RM pulmonar foram comparados com os obtidos na tomografia computadorizada (TC) de tórax, exame atualmente considerado de eleição na investigação da DIP em pacientes com ES. A população avaliada era predominantemente composta por mulheres com idade média de 50 anos, ES cutânea limitada e tempo de doença de aproximadamente sete anos. Na maioria dos casos, houve concordância entre os achados na TC de tórax e RM do pulmão. Em se tratando de um exame isento de radiação e capaz de identificar com adequada precisão áreas de acometimento inflamatório do tecido pulmonar, a RM do pulmão de revelou um exame útil. São necessários mais estudos para avaliar se há vantagem da RM do pulmão sobre a TC de tórax na avaliação da atividade da DIP em pacientes com ES.

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.

Nailfold capillaroscopy in leprosy.

Due to mounting evidences of interaction between Hansen's bacilli with endothelial cells and the paucity of studies addressing the presence of nailfold capillaroscopic alterations in patients with Hansen's 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.

Autoantibodies coexistence in systemic sclerosis: how to interpret it?

Autoantibodies possibly influence clinical manifestations of systemic sclerosis (SSc). This clinical-serological correlation, associated with the paucity of autoantibodies concomitance, gave rise to the historical paradigm of autoantibodies mutual exclusivity. However, one can question this assumption. Does autoantibodies concomitance mean coexistence of two different entities? On the other hand, if considered a unique disease, is this phenomenon a random event or does it represent a distinct subgroup of patients, with peculiar clinical, pathogenic, and immunogenetic characteristics? The autoantibodies' prevalence in early SSc is high. However, anti-centromere antibody (ACA) and antitopoisomerase 1 antibody (ATA) duplicity is a rare event. Similarly, the ACA, ATA, and anti-RNA polymerase (anti-RNA-P) III coexistence have not been described yet in single patient. In the reported case, with ACA, ATA, and anti-RNA-P III positivity, we have noted early vascular manifestations and late limited cutaneous involvement. This is, to our knowledge, the first report of three concomitant specific autoantibodies in a patient with SSc. We do believe this coexistence represents a rare serologic subgroup of a unique disease, with possible clinical and prognostic value, although this remains to be confirmed.

Concomitância de autoanticorpos na esclerose sistêmica: como interpretar? / Autoantibodies coexistence in systemic sclerosis: how to interpret it?

Os autoanticorpos possivelmente influenciam as manifestações clínicas da esclerose sistêmica (ES). Essa correlação clínico-sorológica, associada à insuficiência de casos de concomitância de autoanticorpos, originou o paradigma histórico de que seriam mutuamente excludentes. Porém, pode-se questionar essa tese. Poderia a multiplicidade de autoanticorpos significar a coexistência de duas patologias distintas? Por outro lado, se assumidos como anticorpos específicos de uma doença única, essa multiplicidade seria um evento aleatório ou representaria um subgrupo distinto de pacientes, com características clínicas, patogênicas e imunogenéticas próprias? A prevalência de autoanticorpos na ES precoce é elevada. Entretanto, a duplicidade do anticorpo anticentrômero (AAC) e do anticorpo antitopoisomerase 1 (AAT) é um evento raro. Já a coexistência de AAC, AAT e anticorpo anti-RNA polimerase (anti-RNA-P) III ainda não foi descrita em um paciente isolado. Neste relato, com positividade para AAC, AAT e anti-RNA-P III, notamos manifestações vasculares precoces e posterior comprometimento cutâneo limitado. Este parece ser o primeiro relato de concomitância de três autoanticorpos específicos em um paciente com ES. Acreditamos que essa coexistência representa um subgrupo sorológico raro de uma única doença, com possível valor clínico e prognóstico - porém, ainda há necessidade de confirmação.

Autoantibodies possibly influence clinical manifestations of systemic sclerosis (SSc). This clinical-serological correlation, associated with the paucity of autoantibodies concomitance, gave rise to the historical paradigm of autoantibodies mutual exclusivity. However, one can question this assumption. Does autoantibodies concomitance mean coexistence of two different entities? On the other hand, if considered a unique disease, is this phenomenon a random event or does it represent a distinct subgroup of patients, with peculiar clinical, pathogenic, and immunogenetic characteristics? The autoantibodies' prevalence in early SSc is high. However, anti-centromere antibody (ACA) and antitopoisomerase 1 antibody (ATA) duplicity is a rare event. Similarly, the ACA, ATA, and anti-RNA polymerase (anti-RNA-P) III coexistence have not been described yet in single patient. In the reported case, with ACA, ATA, and anti-RNA-P III positivity, we have noted early vascular manifestations and late limited cutaneous involvement. This is, to our knowledge, the first report of three concomitant specific autoantibodies in a patient with SSc. We do believe this coexistence represents a rare serologic subgroup of a unique disease, with possible clinical and prognostic value, although this remains to be confirmed.

Envolvimento das musculaturas esquelética e cardíaca na esclerose sistêmica / Skeletal and cardiac muscles involvement in systemic sclerosis

Pacientes com Esclerose Sistêmica (ES) podem apresentar envolvimento muscular na forma de miosite ou miopatianão inflamatória. É verificada também associação entre acometimento muscular e disfunção ventricular esquerda (DVE) em pacientes com ES, o que lhes confere pior prognóstico. Avaliamos 87 pacientes do Hospital de Clínicas da Universidade Federal do Paraná, com diagnóstico de ES, quanto à presença de manifestações da musculatura esquelética e a relação destas com DVE. Verificamos uma prevalência de 42,5 por cento de acometimento muscular nos pacientes avaliados, observando uma correlação positiva com a forma difusa da doença. Afastadas outras causas de DVE, três dos quatro pacientes com fração de ejeção abaixo do valor de normalidade apresentaram alteração de força muscular, atrofia e/ou elevação de enzima creatinofosfoquinase sérica (CPK).

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 Clínicas of the Universidade Federal do Paraná, diagnosed with SSc, were assessed regarding the presence of skeletal muscle manifestations and their relation with LVD. A 42.5 percent 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.

Perfil de autoanticorpos e correlação clínica em um grupo de pacientes com esclerose sistêmica na região sul do Brasil / Autoantibody profile and clinical correlation in a group of patients with systemic sclerosis in southern Brazil

OBJETIVOS: Este estudo visou à análise das manifestações da esclerose sistêmica (ES), com ênfase na pesquisa dos autoanticorpos e de suas correlações clínicas, na população de pacientes em acompanhamento no ambulatório de ES do Hospital de Clínicas da Universidade Federal do Paraná. METODOLOGIA: Realizou-se um estudo transversal com 96 pacientes em acompanhamento no ambulatório de ES do hospital, entre setembro de 2007 e setembro de 2009. RESULTADOS: A maioria dos pacientes era do sexo feminino, com idade entre a quarta e quinta décadas e tempo de doença com mediana de 10 anos. A forma cutânea limitada de ES foi a mais prevalente. Na análise dos autoanticorpos, o anticorpo anticentromérico (ACA) associou-se a ES forma limitada, idade mais avançada ao diagnóstico, maior tempo de doença, intervalo maior entre o surgimento do fenômeno de Raynaud (FRy) e o primeiro sintoma não FRy, maior prevalência de hipertensão arterial sistêmica (HAS) e de bloqueios de condução cardíaca. O anticorpo antitopoisomerase-1 (antitopo-1, previamente denominado anti-Scl-70) foi mais comum na forma difusa da ES, na presença de doença ativa e de úlceras digitais. O anticorpo anti-RNA polimerase III (antipol III) correlacionou-se com a forma difusa de ES, presença de doença ativa e sinovite. CONCLUSÕES: Este estudo vem ressaltar e ratificar o papel relevante dos autoanticorpos na avaliação dos pacientes com ES, sendo possível correlacionar o perfil autoimune dessa população com manifestações específicas da doença.

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 Clínicas of the Universidade Federal do Paraná. 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 Raynaud's 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.

Autoantibody profile and clinical correlation in a group of patients with systemic sclerosis in southern Brazil.

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 Clínicas of the Universidade Federal do Paraná. 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 Raynaud's 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.

Skeletal and cardiac muscles involvement in systemic sclerosis.

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 Clínicas of the Universidade Federal do Paraná, 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.

Nailfold capillaroscopy as a diagnostic and prognostic method in rosacea.

BACKGROUND: There is no appropriate and reliable method of evaluating and monitoring severity in rosacea. OBJECTIVE: To determine the importance of nailfold capillaroscopy as a diagnostic and prognostic method for patients with rosacea. METHODS: A cross-sectional study where eight patients with rosacea and 8 control subjects were submitted to nailfold capillaroscopy from May to July 2009. We collected clinical data related to gender, age, skin phototype, and rosacea stage according to Plewig and Kligman classification and the classification of the National Rosacea Society. Additionally, we evaluated the progression of the disease and treatment therapies previously used. RESULTS: The majority of the patients evaluated (6 out of 8 patients) had rosacea subtype I (vascular) or erythematotelangiectatic rosacea. The mean duration of the disorder was 5.96 years, and 87.5% of the patients were under treatment with topical metronidazole. Nailfold cappilaroscopy showed that evidence of devascularization was absent in both groups. CONCLUSION: Nailfold capillaroscopy presents a nonspecific pattern and does not seem to help in the diagnosis or prognosis of rosacea.

Uso da capilaroscopia ungueal como método diagnóstico e prognóstico de rosácea / Nailfold capillaroscopy as a diagnostic and prognostic method in rosacea

FUNDAMENTOS: Não há um método adequado e fidedigno de avaliação e seguimento da severidade na rosácea. OBJETIVO: Determinar a importância da capilaroscopia periungueal como método diagnóstico e prognóstico em pacientes portadores de rosácea. MÉTODOS: Estudo transversal onde foram submetidos ao exame da capilaroscopia periungueal 8 pacientes com rosácea e 8 controles no período entre maio e julho de 2009. Foram coletados dados clínicos relacionados ao sexo, idade, fototipo, classificação da rosácea de acordo com a classificação de Plewig e Kligman e a classificação da National Rosacea Society. Adicionalmente, avaliamos o tempo de evolução da doença e tratamentos previamente utilizados. RESULTADOS: A grande maioria das pacientes avaliadas (6 das 8 pacientes) apresentavam rosácea grau I (vascular) ou eritêmato-teleangiectásica. A idade média de duração da rosácea foi de 5,96 anos, sendo que 87,5 por cento faziam tratamento com metronidazol tópico. Nenhum paciente tanto do grupo rosácea como controle demonstrou evidência de desvascularização ao exame capilaroscópico. CONCLUSÃO: A capilaroscopia periungueal apresenta um padrão inespecífico e não parece auxiliar no diagnóstico ou prognóstico da rosácea.

BACKGROUND: There is no appropriate and reliable method of evaluating and monitoring severity in rosacea. OBJECTIVE: To determine the importance of nailfold capillaroscopy as a diagnostic and prognostic method for patients with rosacea. METHODS: A cross-sectional study where eight patients with rosacea and 8 control subjects were submitted to nailfold capillaroscopy from May to July 2009. We collected clinical data related to gender, age, skin phototype, and rosacea stage according to Plewig and Kligman classification and the classification of the National Rosacea Society. Additionally, we evaluated the progression of the disease and treatment therapies previously used. RESULTS: The majority of the patients evaluated (6 out of 8 patients) had rosacea subtype I (vascular) or erythematotelangiectatic rosacea. The mean duration of the disorder was 5.96 years, and 87.5 percent of the patients were under treatment with topical metronidazole. Nailfold cappilaroscopy showed that evidence of devascularization was absent in both groups. CONCLUSION: Nailfold capillaroscopy presents a nonspecific pattern and does not seem to help in the diagnosis or prognosis of rosacea.

[Nutritional evaluation and physical functional ability in scleroderma patients]. / Avaliação nutricional e da capacidade funcional em doentes com esclerose sistémica progressiva.

INTRODUCTION: Systemic Sclerosis (SSc) is an inflammatory disease that decreases functional capacity through muscular atrophy, skin sclerosis and loss of joint function. Scleroderma patients suffer from movement's restriction. In addition, the disease affects the nutritional status, compromising the quality of life in varying degrees. The gastrointestinal involvement appears to be the main responsible for the nutritional impairment. OBJECTIVE: To evaluate the physical and nutritional status of patients with SSc. METHODS: We conducted a cross-sectional and descriptive study in 20 patients with SSc. All patients were evaluated in Physioteraphy Clinic of the Catholic University of Paraná, from July 2003 to April 2004. The evaluation was performed by Bioimpedance Body (BIA), the questionnaires Nutritional Risk Assessment (Determine), Mini Nutritional Assessment of Aging and Health Assessment Questionnaire (HAQ) and Visual Analogue Scale (VAS) for pain. The work muscle ability was assessed by measuring the peak torque of flexor and extensor muscles of the elbow in the isokinetic dynamometer Cybex Norm model 7000. We have calculated the mean and standard deviation for each variable analyzed, in addition to the percentage of peak torque deficit. RESULTS: The values of the VAS ranged from zero to 97.8 mm (mean: 48.9 +/- 32.9 mm). The HAQ scores ranged from zero to 2.75 (average: 0.95 +/- 0.8). The average BMI was 22.4 +/- 3.9 kg / m2. The average deficit of mass was: 1.3 +/- 2.1 kg. Ten patients had high nutritional risk. 1 patient was malnourished and 15 were at high risk for malnutrition. The average peak torque to the muscle groups of elbows was 19.2 +/- 5 N / m to the flexor and 21.9 +/-6.6 N/m for the extensors, with an average deficit of 17% and 13% for the both groups. CONCLUSIONS: We found that SSc patients were in poor nutritional status and had decreased functional and physical capacity by the weakening of the muscles of the elbow demonstrated by the isokinetic evaluation. We concluded that our SSc patients were at high risk for malnutrition and this may indicate that scleroderma patients need a better nutritional orientation. We do consider that SSc patients also must be included in physical activity programs in order to achieve better physical performance.

Manifestações clínicas e laboratoriais de polimiosite em um caso de leptospirose / Clinical and laboratorial features of polymyositis in a case of leptospirosis

Nem sempre é possível estabelecer uma clara distinção entre as doenças musculares de causa infecciosa e as miosites idiopáticas. Há situações, inclusive, em que se considera existir uma relação causal entre ambas as doenças. Descrevemos o caso de uma paciente com manifestações clínicas e laboratoriais de polimiosite que apresentou infecção precedente de leptospirose, em que se considera a hipótese de ação de "gatilho" do agente infeccioso no quadro muscular inflamatório.

Sometimes, it is not possible to establish an obvious distinction between the muscular manifestations due to infectious diseases and the idiopathic myositis. In fact, in some situations it is believed there is a causal relationship between those diseases. We describe the case of a patient with clinical and laboratorial features of polymyositis who had developed leptospirosis previously. The hypothesis of the infectious agent acting as a "trigger" to the muscular disease is discussed.

Hipertensão arterial pulmonar e acidente vascular encefálico em paciente com Arterite de Takayasu / Pulmonary arterial hypertension and cerebrovascular disease in patient with Takayasu Arteritis

Arterite de Takayasu (AT) é uma doença rara caracterizada por vasculite dos grandes vasos, principalmente aorta e seus ramos. A inflamação vascular leva a irregularidades na parede do vaso, causando estenoses e aneurismas. O envolvimento da artéria pulmonar (AP) é freqüente na AT. Apesar disso, o desenvolvimento de hipertensão arterial pulmonar (HAP) é menos comum, alterando o tratamento e o prognóstico dos pacientes. Descrevemos um caso de uma paciente com AT com estenoses múltiplas em artérias pulmonares, HAP e doença cerebrovascular, além de revisão de literatura sobre o assunto.

Takayasu arteritis (TA) is a rare illness characterized by vasculitis of great vessels, mainly of aorta and its branches. The vascular inflammation leads to irregularities of the vessel wall causing stenosis and aneurysms. The pulmonary artery (PA) involvement is frequent in TA. Despite this, the development of pulmonary arterial hypertension is less common, modifying the treatment and the prognosis of the patients. We describe a case of a patient with TA with multiple stenosis in pulmonary arteries, pulmonary arterial hypertension and cerebrovascular disease, as well as review of the literature on the subject.