Dermatomiosite amiopática: relato de caso / Amyopathic dermatomyositis: case report / Dermatomiositis amiopática: reporte de caso

Introdução: Dermatomiosite amiopática pode ser definida como a presença de manifestações cutâneas, porém sem acometimento muscular. A forma amiopática se apresenta com ausência de fraqueza muscular e definida através de testes diagnósticos que incluem dosagem de enzimas musculares séricas, eletromiografia e biópsia muscular, que normalmente apresentam resultados normais ou minimamente alterados. Objetivo: Relatar um caso de dermatomiosite amiopática em seguimento em um hospital-escola de Catanduva-SP. Método: Paciente do sexo feminino de 60 com heliotropo em região de pálpebras bilateralmente, alterações nos capilares sugestivos do padrão esclerodermia, artralgia difusa com rigidez articular menor que 10 minutos com sensação de parestesia em membros superiores e inferiores, câimbras e doença pulmonar intersticial. Resultados: Os exames laboratoriais demonstraram discreta microcitose e anisocitose no hemograma, aumento da proteína C reativa (PCR), fator antinuclear (FAN) não reagente, aldolase dentro dos valores de referência e dislipidemia. A eletromiografia não demonstrou envolvimento muscular, corroborando para o diagnóstico da dermatomiosite amiopática

Introduction: Amyopathic dermatomyositis can be defined as the presence of cutaneous manifestations, but without muscle involvement. The amyopathic form presents with the absence of muscle weakness and is defined through diagnostic tests that include measurement of serum muscle enzymes, electromyography and muscle biopsy, which normally present normal or minimally altered results. Objective: To report a case of amyopathic dermatomyositis followed at a teaching hospital in Catanduva-SP. Method: A 60-year-old female patient with heliotrope in the eyelid region bilaterally, capillary changes suggestive of a scleroderma pattern, diffuse arthralgia with joint stiffness lasting less than 10 minutes, with a sensation of paresthesia in the upper and lower limbs, cramps, and interstitial lung disease. Results: Laboratory tests showed mild microcytosis and anisocytosis in the blood count, increased C-reactive protein (CRP), nonreactive antinuclear antibody (ANA), aldolase within the reference values, and dyslipidemia. Electromyography showed no muscle involvement, corroborating the diagnosis of amyopathic dermatomyositis

Introducción: La dermatomiositis amiopática se puede definir como la presencia de manifestaciones cutáneas, pero sin afectación muscular. La forma amiopática se presenta con ausencia de debilidad muscular y se define mediante pruebas diagnósticas que incluyen medición de enzimas musculares séricas, electromiografía y biopsia muscular, que normalmente presentan resultados normales o mínimamente alterados. Objetivo: Comunicar un caso de dermatomiositis amiopática seguido en un hospital universitario de Catanduva-SP. Método: Paciente femenina de 60 años con heliotropo en región palpebral bilateral, cambios en los capilares sugestivos de patrón de esclerodermia, artralgias difusas con rigidez articular de menos de 10 minutos con sensación de parestesia en miembros superiores e inferiores, calambres y pulmón intersticial enfermedad. Resultados: Los exámenes de laboratorio demostraron microcitosis y anisocitosis leve en el hemograma, aumento de proteína C reactiva (PCR), factor antinuclear (ANA) no reactivo, aldolasa dentro de valores de referencia y dislipidemia. La electromiografía no demostró afectación muscular, lo que apoya el diagnóstico de dermatomiositis amiopática

Analysis of sexual function of patients with dermatomyositis and polymyositis through self-administered questionnaires: a cross-sectional study.

INTRODUCTION: To date, there are no descriptions in the literature on gynecologic and sexual function evaluation in female patients with dermatomyositis (DM) and polymyositis (PM). OBJECTIVE: To assess sexual function in female patients with DM/PM. PATIENTS AND METHODS: This is a monocentric, cross-sectional study in which 23 patients (16 DM and 7 PM), with ages between 18 and 40 years, were compared to 23 healthy women of the same age group. Characteristics on sexual function were obtained by applying the questionnaires Female Sexual Quotient (FSQ) and Female Sexual Function Index (FSFI) validated for the Brazilian Portuguese language. RESULTS: The mean age of patients was comparable to controls (32.7±5.3 vs. 31.7±6.7 years), as well as the distribution of ethnicity and socioeconomic class. As for gynecological characteristics, patients and healthy controls did not differ with respect to age at menarche and percentages of dysmenorrhea, menorrhagia, premenstrual syndrome, pain at mid-cycle, mucocervical secretion, and vaginal discharge. The FSQ score, as well as all domains of the FSFI questionnaire (desire, arousal, lubrication, orgasm and satisfaction), were significantly decreased in patients vs. controls, with 60.9% of patients showing some degree of sexual dysfunction. CONCLUSIONS: This was the first study to identify sexual dysfunction in patients with DM/PM. Therefore, a multidisciplinary approach is essential for patients with idiopathic inflammatory myopathies, in order to provide prevention and care for their sexual life, providing a better quality of life, both for patients and their partners.

Inflammatory myopathies: an update for neurologists / Miopatias inflamatórias: uma atualização para neurologistas

ABSTRACT Idiopathic inflammatory myopathies (IIM) are a heterogenous group of treatable myopathies. Patients present mainly to the rheumatologist and neurologists, complaining of acute or subacute onset of proximal weakness. Extramuscular manifestations may occur, including involvement of the lungs, skin, and joints. Classically, the diagnosis used to be made based on the creatine kinase level increase, abnormalities in electroneuromyography and presence of inflammatory infiltrates in the muscle biopsy. Recently, the importance of autoantibodies has increased, and now they may be identified in more than half of IIM patients. The continuous clinicoseropathological improvement in IIM knowledge has changed the way we see these patients and how we classify them. In the past, only polymyositis, dermatomyositis and inclusion body myopathy were described. Currently, immune-mediated necrotizing myopathy, overlap myositis and antisynthetase syndrome have been considered the most common forms of IIM in clinical practice, increasing the spectrum of classification. Patients previously considered to have polymyositis, in fact have these other forms of seropositive IIM. In this article, we reviewed the new concepts of classification, a practical way to make the diagnosis and how to plan the treatment of patients suffering from IIM.

RESUMO As miopatias inflamatórias idiopáticas (MII) são um grupo heterogêneo de miopatias tratáveis. Os pacientes procuram principalmente o reumatologista e o neurologista, queixando-se de início agudo ou subagudo de fraqueza proximal. Manifestações extramusculares podem ocorrer, incluindo envolvimento dos pulmões, pele e articulações. Classicamente, o diagnóstico era feito com base na elevação dos níveis de creatina quinase, anormalidades na eletroneuromiografia e presença de infiltrados inflamatórios na biópsia muscular. Recentemente, a importância dos autoanticorpos aumentou, e agora eles podem ser identificados em mais da metade dos pacientes com MII. A contínua melhora clínico-soropatológica no conhecimento do MII mudou a forma como vemos esses pacientes e como os classificamos. No passado, apenas polimiosite, dermatomiosite e miopatia por corpos de inclusão eram descritas. Atualmente, a miopatia necrosante imunomediada, a miosite de sobreposição e a síndrome antissintetase têm sido consideradas as formas mais comuns de MII na prática clínica, aumentando o espectro de classificação. Pacientes previamente considerados como portadores de polimiosite, na verdade, têm uma dessas outras formas de MII soropositivas. Neste artigo, revisamos os novos conceitos de classificação, uma forma prática de fazer o diagnóstico e como planejar o tratamento de pacientes que sofrem de MII.

Tiossulfato de sódio 10% intralesional para tratamento da calcinose em pacientes com esclerose sistêmica e dermatomiosite: série de casos / 10% intralesional sodium thiosulphate for calcinosis treatment in patients with systemic sclerosis and dermatomyositis: case series

INTRODUÇÃO: A calcinose é uma complicação frequente da esclerose sistêmica e da dermatomiosite, resultando em dor local, contraturas, ulcerações, infecções secundárias e limitação funcional. Não há tratamento eficaz para calcinose mas o tiossulfato de sódio tem se mostrado promissor. OBJETIVOS: Analisar a resposta terapêutica ao tiossulfato de sódio 10% intralesional nas calcinoses de pacientes com dermatomiosite e esclerose sistêmica. MÉTODOS: Estudo prospectivo, não controlado, incluindo 7 pacientes, totalizando 10 calcinoses, sendo um paciente com dermatomiosite e 6 com esclerose sistêmica. Desfechos primários: melhora da dor pela escala visual analógica e diminuição do maior e menor diâmetros das calcinoses ao raio-x. Desfechos secundários: melhora da qualidade de vida pelo SF12 (Medical Outcomes Study Short Form 12) e da função pelo HAQ (Health Assesment Questionnaire). Resultados foram expressos em médias e as variáveis contínuas comparadas pelo teste t-Student ou pelo teste de Wilcoxon. RESULTADOS: Foram realizadas 2 a 8 aplicações intralesionais (média de 3,7), com média de 8,9mg de tiossulfato por injeção a intervalos de 13 a 56 dias, com intervalo médio de 19 dias. Todos os pacientes relataram melhora da dor, porém esse resultado não foi significativamente estatístico. Não houve redução do diâmetro das calcinoses, nem melhora da qualidade de vida ou funcional. CONCLUSÃO: Baixas doses de tiossulfato de sódio administradas por injeções intralesionais, em intervalos médios de 19 dias, em número limitado de aplicações, não foram efetivas para o tratamento da calcinose

BACKGROUND: Calcinosis is a frequent complication of systemic sclerosis and dermatomyositis, resulting in local pain, contractures, ulcerations, secondary infections and functional limitations. There is no effective treatment for calcinosis, but sodium thiosulphate has shown to be promising. OBJECTIVE: To analyze the therapeutic response to 10% intralesional sodium thiosulphate on calcinosis of patients with dermatomyositis and systemic sclerosis. METHODS: Non-controlled prospective study, with 7 patients and 10 calcinoses, of which one had dermatomyositis and six had scleroderma. Primary outcomes: improvement in the pain by the visual analogue scale, and a decrease in both diameters (biggest and smallest) of calcinoses on X-ray. Secondary outcomes: improvement in quality of life by SF12 (Medical Outcomes Study Short Form 12), and of function by HAQ (Health Assessment Questionnaire). Results were expressed as means; continuous variables were compared by the t-Sudent test or by the Wilcoxon test. RESULTS: Two to 8 intralesional applications were performed (mean of 3.7), with mean of 8.9mg of thiosulphate per injection, at 13-56 days intervals (mean of 19 days). All patients reported improvement in pain, albeit such result was not statistically significant. There was no reduction in the diameters of calcinoses, nor there were quality of life or functional improvements. CONCLUSION: Low doses of sodium thiosulphate, administered through intralesional injections, in mean intervals of 19 days, in a limited number of applications, were not effective for calcinosis treatment

Efetividade do treinamento físico sobre o desempenho físico em pacientes com dermatomiosite e polimiosite: revisão sistemática e metanálise / Eficacia del entrenamiento físico sobre el rendimiento físico en pacientes con dermatomiositis y polimiositis: una revisión sistemática y metaanálisis / Effectiveness of physical training on physical performance in patients with dermatomyositis and polymyositis: systematic review and meta-analysis

RESUMO Este estudo teve por objetivo avaliar o impacto do treinamento físico sobre o desempenho físico em pacientes com dermatomiosite e polimiosite. Para tanto, uma revisão sistemática e metanálise foi conduzida de acordo com as diretrizes do PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses). A pesquisa bibliográfica foi realizada nas seguintes bases de dados: PubMed/MEDLINE e Web of Science, utilizando combinações das seguintes palavras-chave em inglês: dermatomyositis OR polymyositis OR myositis AND exercise OR physical exercise OR physical therapy OR aerobic exercise OR endurance exercise OR resistance exercise. Foram incluídos estudos que atenderam aos seguintes critérios: (1) os participantes apresentavam diagnóstico de dermatomiosite ou polimiosite; (2) os pacientes foram submetidos a um protocolo de treinamento físico; (3) o desempenho físico foi mensurado antes e após o protocolo de treinamento físico. Um total de 14 artigos foram selecionados para inclusão na revisão sistemática e 10 artigos foram selecionados para inclusão na metanálise. Os resultados demonstram que o treinamento físico é eficaz em aumentar o desempenho físico global nos pacientes com dermatomiosite e polimiosite (tamanho do efeito: 0,72; IC 95% 0,55; 0,89). Além disso, foi demonstrado também que tanto as variáveis de desempenho aeróbio (tamanho do efeito: 0,88; IC 95% 0,54; 1,21), quanto as variáveis de desempenho resistido (tamanho do efeito: 0,64; IC 95% 0,43; 0,85) são beneficiadas com o treinamento físico nesses pacientes. Conclui-se que o treinamento físico apresentou um efeito benéfico significativo sobre o desempenho físico global, aeróbio e resistido em pacientes com dermatomiosite e polimiosite.

RESUMEN Este estudio tuvo como objetivo evaluar el impacto del entrenamiento físico sobre el rendimiento físico en pacientes con dermatomiositis y polimiositis. Para ello, se realizó una revisión sistemática y metaanálisis siguiendo las guías PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses). Se hizo una búsqueda bibliográfica en las siguientes bases de datos: PubMed/MEDLINE y Web of Science, utilizando las siguientes palabras clave combinadas en inglés: dermatomyositis OR polymyositis OR myositis AND exercise OR physical exercise OR physical therapy OR aerobic exercise OR endurance exercise OR resistance exercise. Se incluyeron los estudios que cumplieron los siguientes criterios: (1) los participantes tenían un diagnóstico de dermatomiositis o polimiositis; (2) los pacientes se sometieron a un protocolo de entrenamiento físico; y (3) el rendimiento físico se midió antes y después del protocolo de entrenamiento físico. Al total se seleccionaron 14 artículos para incluir en la revisión sistemática y 10 artículos en el metaanálisis. Los resultados demuestran que el entrenamiento físico es eficaz para aumentar el rendimiento físico general en pacientes con dermatomiositis y polimiositis (tamaño del efecto: 0,72; IC 95% 0,55; 0,89). Además, tanto las variables de rendimiento aeróbico (tamaño del efecto: 0,88; IC 95% 0,54; 1,21) como las variables de rendimiento de resistencia (tamaño del efecto: 0,64; IC 95% 0,43; 0,85) mejoraron con la actividad física en estos pacientes. Se concluye que el entrenamiento físico tuvo un efecto significativo sobre el rendimiento físico global, aeróbico y de resistencia en pacientes con dermatomiositis y polimiositis.

ABSTRACT This study aimed to evaluate the impact of physical training on physical performance in patients with dermatomyositis and polymyositis. For this purpose, we conducted a systematic review and meta-analysis according to the guidelines of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses). The literature search was conducted in the following databases: PubMed/MEDLINE and Web of Science, using combinations of the following keywords in English: dermatomyositis OR polymyositis OR myositis AND exercise OR physical exercise OR physical therapy OR aerobic exercise OR endurance exercise OR resistance exercise. Studies that met the following criteria were included: (1) participants diagnosed with dermatomyositis or polymyositis; (2) patients that undergone a physical training protocol; (3) physical performance measured before and after the physical training protocol. A total of 14 articles were selected for inclusion in the systematic review and 10 articles were selected for inclusion in the meta-analysis. The outcomes demonstrate that physical training is effective in increasing overall physical performance in patients with dermatomyositis and polymyositis (effect size: 0.72; 95% CI 0.55; 0.89). Also, our study demonstrated that both the aerobic performance (effect size: 0.88; 95% CI 0.54; 1.21) and resistance performance variables (effect size: 0.64; CI 95% 0.43; 0.85) benefit from physical training in these patients. We concluded that physical training had a significant beneficial effect on the overall, aerobic and resistance physical performance in patients with dermatomyositis and polymyositis.

Electrocardiographic changes in dermatomyositis and polymyositis.

INTRODUCTION: Cardiac involvement is frequent in inflammatory myopathies. Electrocardiogram (ECG) may show evidence of this involvement and its changes should be well-known and described. OBJECTIVES: Due to the lack of studies in the literature, we conducted an analysis of the ECG findings in patients with dermatomyositis (DM) and polymyositis (PM), comparing them with a control group. METHODS: This cross-sectional study compared the ECG of 86 individuals with no rheumatic disorders (controls) with 112 patients (78 DM and 34 PM), during 2010-2013. The ECG findings between DM and PM were also compared. RESULTS: Demographic characteristics, comorbidities and ECG abnormalities were similar between controls and patients (p>0.05), except for a higher frequency of left ventricular hypertrophy (LVH) in patients (10.7% vs. 1.2%, p=0.008). Demographic characteristics, comorbidities, clinical and laboratory manifestations, were also similar between the groups PM and DM, except for the presence of cutaneous lesions only in DM. One-third of the patients had ECG abnormalities, which were more prevalent in PM than DM (50% vs. 24.4%, p=0.008). LVH, left atrial enlargement, rhythm and conduction abnormalities were more frequent in PM than DM (p<0.05 for all), especially the left anterior fascicular block. CONCLUSIONS: We showed distinct ECG changes between DM and PM and a higher frequency of LVH in patients compared to controls. Investigation of cardiac involvement should be considered even in asymptomatic patients, especially PM. Further studies are necessary in order to determine the correlation of ECG findings with other complementary tests, clinical manifestations, disease activity and progression to other cardiac diseases.

Hyperbaric oxygen therapy in the adjuvant treatment of cutaneous manifestations in dermatomyositis: a case report / Oxigenioterapia hiperbárica adjuvante ao tratamento da manifestação cutânea da dermatomiosite: relato de caso / La oxigenoterapia hiperbárica en el tratamiento adyuvante de las manifestaciones cutáneas en la dermatomiositis: reporte de un caso

Cutaneous manifestations of dermatomyositis are unusual and difficult to treat. This study aimed to report a case of cutaneous manifestations of dermatomyositis treated with hyperbaric oxygen. We present a case of dermatomyositis in a 44-year-old female with pain ulcers in her left leg for 17 months, refractory to an exclusive clinical treatment, who underwent a hyperbaric oxygen therapy (HBOT) breathing O2 100%, 90 minute sessions, six days a week, at 2.4 ATA. HBOT therapy proved to be highly efficacious in wound healing in this case and HBOT should be considered as a treatment in the assistance given to such patients.

Manifestações cutâneas da dermatomiosite são raras e difíceis de ser tratadas. O objetivo deste estudo é relatar um caso de manifestações cutâneas da dermatomiosite tratadas com oxigenioterapia hiperbárica, ocorrido em uma paciente de 44 anos, sexo feminino, com úlceras dolorosas em seu membro inferior esquerdo por 17 meses. O caso foi refratário ao tratamento clínico exclusivo e a paciente submetida a tratamento de oxigenioterapia hiperbárica (HBOT), respirando oxigênio a 100%, em sessões de noventa minutos, seis dias por semana, a 2,4 ATA. O HBOT mostrou ser altamente eficaz na cicatrização da lesão neste caso e deve ser um tratamento considerado no cuidado desses pacientes.

Las manifestaciones cutáneas de la dermatomiositis son inusuales y difíciles de tratar. El objetivo de este estudio fue reportar un caso de manifestaciones cutáneas de dermatomiositis tratadas con oxigenoterapia hiperbárica. Presentamos un caso de dermatomiositis en una mujer de 44 años con úlceras dolorosas en su pierna izquierda durante 17 meses, refractaria a un tratamiento clínico exclusivo, que se sometió a oxigenoterapia hiperbárica (HBOT) respirando O2 100%, sesiones de 90 minutos, seis días a la semana, a 2,4 ATA. La terapia con HBOT demostró ser muy eficaz en la cicatrización de heridas en este caso y la HBOT debe considerarse como un tratamiento en la asistencia brindada a estos pacientes.

[Devic's disease in an adolescent girl with juvenile dermatomyositis]. / Neuromielite óptica em uma adolescente com dermatomiosite juvenil.

Devic's disease, also known as neuromyelitis optica, is an autoimmune inflammatory demyelinating disorder of the central nervous system that mainly affects the optic nerve and spinal cord. Recently, Devic's disease was demonstrated to be a channelopathy due to the presence of antibodies against the water channel aquaporin-4 in the blood-brain barrier. There have been reports of Devic's disease in infancy, but there are few reported associations of Devic's disease with other diseases. The association of Devic's disease with dermatomyositis has not yet been described in the literature. The aim of this paper is to describe the first case of Devic's disease in an adolescent with juvenile dermatomyositis.

Pregnancy outcomes in dermatomyositis and polymyositis patients.

BACKGROUND: Currently, there are few studies that describe pregnancy in dermatomyositis/polymyositis patients, and they are largely limited to case reports or studies with few samples. OBJECTIVES: Therefore, we describe the pregnancy in a large sample of patients with dermatomyositis/polymyositis and to analyze the outcomes in those who became pregnant during or after disease onset. METHODS: The present single-center study analyzed 98 female patients with idiopathic inflammatory myopathies (60 dermatomyositis and 38 polymyositis patients). They were interviewed to obtain obstetric antecedent and demographic data from June 2011 to June 2012. RESULTS: Seventy-eight (79.6%) of the 98 patients had obstetric histories. Six polymyositis and 9 dermatomyositis patients became pregnant after disease onset. The pregnancy outcomes in these cases were good, except in the following cases: 1 disease reactivation, 1 intrauterine growth retardation, 1 diabetes mellitus, 1 hypertension, 1 hypothyroidism, and 2 fetal losses (same patient). Moreover, 2 patients developed dermatomyositis during pregnancy and 4 (2 polymyositis and 2 dermatomyositis) during the postpartum period with good control after glucocorticoid and immunosuppressant therapy. CONCLUSIONS: The adverse obstetric events were related to clinical intercurrences and the pregnancy does not seem to carry a worse prognosis specifically in disease (for example: disease relapsing). Moreover, dermatomyositis or polymyositis onset during pregnancy or the postpartum period had good outcome after drug therapy.

Lipid profile of pediatric patients with chronic rheumatic diseases - a retrospective analysis

SUMMARY AIM To describe the prevalence of dyslipidemia in children and adolescents with autoimmune rheumatic diseases (ARDs), particularly juvenile idiopathic arthritis (JIA), juvenile systemic lupus erythematosus (jSLE), and juvenile dermatomyositis (JDM). METHODS Retrospective cross-sectional study conducted in the pediatric rheumatology outpatient clinic. We evaluated 186 children and adolescents between the ages of 5 and 19 years. The medical records were reviewed for the following data: demographic and clinical features, disease activity, and lipid profile (triglycerides (TG), total cholesterol (TC), low density lipoprotein (LDL-C), high density lipoprotein (HDL-C) and very low density lipoprotein (VLDL-C)). In addition, non-HDL cholesterol was calculated as TC minus HDL-C. The cut-off points proposed by the American Academy of Pediatrics were used to classify the lipid profile. RESULTS Dyslipidemia was observed in 128 patients (68.8%), the most common being decreased HDL-C (74 patients, 39.8%). In the JIA group there was an association between the systemic subtype and altered LDL-C and NHDL-C, which demonstrated a more atherogenic profile in this subtype (p=0.027 and p=0.017, respectively). Among patients with jSLE, the cumulative corticosteroid dose was associated with an increase in LDL-C (p=0.013) and with a decrease in HDL-C (p=0.022). CONCLUSION Dyslipidemia is common in children and adolescents with ARDs, especially JIA, jSLE, and JDM, and the main alteration in the lipid profile of these patients was decreased HDL-C.

RESUMO OBJETIVO Descrever a prevalência de dislipidemias em crianças e adolescentes com doenças reumáticas autoimunes (Drai), em particular artrite idiopática juvenil (AIJ), lúpus eritematoso sistêmico juvenil (Lesj) e dermatomiosite juvenil (DMJ). MÉTODOS Estudo transversal retrospectivo realizado no ambulatório de reumatologia pediátrica. Foram avaliados 186 crianças e adolescentes com idades entre 5 e 19 anos. Foram coletados dos prontuários dados demográficos, clínicos, atividade de doença e perfil lipídico (triglicérides (TG), colesterol total (CT) e frações LDL-c (low density lipoprotein); HDL-c (high density lipoprotein) e VLDL-c (very low density lipoprotein). Foi também calculada a fração não HDL do colesterol (CT-NHDL -c). Para classificação do perfil lipídico, foram adotados os pontos de corte propostos pela American Academy of Pediatrics. RESULTADOS A dislipidemia foi observada em 128 pacientes (68,8%), sendo a mais comum a diminuição do HDL-c em 74 (39,8%). No grupo AIJ houve uma associação entre o subtipo sistêmico com alteração de LDL-c e NHDL-c, mostrando um perfil mais aterogênico neste subtipo (p=0,027 e 0,017, respectivamente). Em relação aos pacientes com Lesj, podemos observar que a dose cumulativa de CTC teve associação com o aumento do LDL-c (p=0,013) e com a diminuição do HDL-c (p=0,022). CONCLUSÃO A dislipidemia é frequente em crianças e adolescentes com Drai, em especial, AIJ, Lesj e DMJ, e a principal alteração no perfil lipídico desses pacientes foi a diminuição do HDL-c.

[Different aspects of magnetic resonance imaging of muscles between dermatomyositis and polymyositis]. / Aspectos distintos de ressonância magnética de músculos entre dermatomiosite e polimiosite.

INTRODUCTION: Although dermatomyositis (DM) and polymyositis (PM) share many clinical features in common, they have distinct pathophysiological and histological features. It is possible that these distinctions reflect also macroscopically, for example, in muscle alterations seen in magnetic resonance images (MRI). OBJECTIVES: To compare simultaneously the MRI of various muscle compartments of the thighs of adult DM and PM. MATERIALS: The present study is a cross-sectional that included, between 2010 and 2013, 11 newly diagnosed DM and 11 PM patients (Bohan and Peter's criteria, 1975), with clinical and laboratory activity. They were valued at RM thighs, T1 and T2 with fat suppression, 1.5 T MRI scanner sequences. RESULTS: The mean age at the time of MRI, the time between onset of symptoms and the realization of the MRI distribution of sex and drug therapy were comparable between the two groups (p>0.050). Concerning the MRI, muscle edema was significantly found in DM, and mainly in the proximal region of the muscles. The area of fat replacement was found predominantly in PM. The partial fat replacement area occurred mainly in the medial and distal region, whereas the total fat replacement area occurred mainly in the distal muscles. There was no area of muscle fibrosis. CONCLUSIONS: DM and PM have different characteristics on MRI muscles, alike pathophysiological and histological distinctions.

[Periodontal disease in pediatric rheumatic diseases]. / Doença periodontal em doenças reumáticas pediátricas.

Gingivitis and periodontitis are immunoinflammatory periodontal diseases characterized by chronic localized infections usually associated with insidious inflammation This narrative review discusses periodontal diseases and mechanisms influencing the immune response and autoimmunity in pediatric rheumatic diseases (PRD), particularly juvenile idiopathic arthritis (JIA), childhood-onset systemic lupus erythematosus (C-SLE) and juvenile dermatomyositis (JDM). Gingivitis was more frequently observed in these diseases compared to health controls, whereas periodontitis was a rare finding. In JIA patients, gingivitis and periodontitis were related to mechanical factors, chronic arthritis with functional disability, dysregulation of the immunoinflammatory response, diet and drugs, mainly corticosteroids and cyclosporine. In C-SLE, gingivitis was associated with longer disease period, high doses of corticosteroids, B-cell hyperactivation and immunoglobulin G elevation. There are scarce data on periodontal diseases in JDM population, and a unique gingival pattern, characterized by gingival erythema, capillary dilation and bush-loop formation, was observed in active patients. In conclusion, gingivitis was the most common periodontal disease in PRD. The observed association with disease activity reinforces the need for future studies to determine if resolution of this complication will influence disease course or severity.

Dermatomyositis as the first manifestation of a lung tumor.

Dermatomyositis (DM) is a rare disease characterised by proximal muscle weakness and a typical cutaneous rash. The muscle biopsy shows inflammatory lesions consistent with myositis, being related to an increased risk of cancer, often considered as a paraneoplastic syndrome. The authors present a case of a 63-year-old man, with progressive proximal muscle weakness and cutaneous rash, appearing in two months. The muscle and skin biopsies were consistent with DM. Chest tomography showed a nodular image in the lingular region and bronchy biopsy confirmed the diagnosis of small cell lung carcinoma (SCLC). This clinical case intends to enhance the importance of a thorough diagnostic study in patients with DM, as it is often a paraneoplastic syndrome.

Spontaneous pneumomediastinum in dermatomyositis: a case series and literature review / Pneumomediastino espontâneo na dermatomiosite: uma série de casos e revisão de literatura

OBJECTIVES: To describe a case series of spontaneous pneumomediastinum in dermatomyositis and to review the literature. METHODS: This was a retrospective single-center case series, reporting 9 patients with pneumomediastinum and defined dermatomyositis, followed from 2005 to 2017. RESULTS: Median age of patients: 33 years; cutaneous and pulmonary involvement in all cases; constitutional symptoms in 88.8% of patients; involvement of the joints in 11.1%, gastrointestinal tract in 44.4%, and muscles in 77.7%; subcutaneous emphysema was observed in 55.5% and pneumothorax in 11.1%, respectively. Muscle weakness was observed in 77.7% of cases and with a median level of serum creatine phosphokinase of 124U/L. Drawing on results for our literature review, the overall analysis showed that the risk factors associated with spontaneous pneumomediastinum were: (a) a history of interstitial pneumopathy; (b) normal or low levels of muscle enzymes; (c) previous use of systemic glucocorticoid; (d) over 50% of patients had subcutaneous emphysema; (e) high mortality as a consequence of severity of the interstitial lung disease. CONCLUSIONS: Our case series revealed that pneumomediastinum is a rare complication in dermatomyositis that occurs in patients with a history of interstitial pneumopathy and may be accompanied by subcutaneous emphysema and pneumothorax.

OBJETIVOS: Descrever série de casos de pneumomediastino espontâneo em portadores de dermatomiosite e revisar a literatura. MÉTODOS: Trata-se de série de casos, único centro, relatando 9 pacientes com pneumomediastino e dermatomiosite definida, acompanhados de 2005 a 2017. RESULTADOS: A mediana da idade dos pacientes foi de 33 anos. Sintomas constitucionais estavam presentes em 88,8% dos pacientes. Houve acometimento cutâneo e pulmonar em todos os casos, acometimento das articulações em 11,1%, trato gastrointestinal em 44,4% e musculatura em 77,7% dos pacientes. Enfisema subcutâneo foi observado em 55,5% e pneumotórax em 11,1%, respectivamente. A fraqueza muscular foi observada em 77,7% dos casos, com um nível médio de creatinofosfoquinase sérica de 124U/L. Com base nos resultados da revisão da literatura, a análise geral mostrou que: os fatores de risco associados ao pneumomediastino espontâneo foram: história de pneumopatia intersticial, níveis normais ou baixos de enzimas musculares, uso prévio de glicocorticoide sistêmico; >50% dos pacientes tiveram enfisema subcutâneo; houve alta mortalidade como consequência da gravidade da doença pulmonar intersticial. CONCLUSÕES: Nossa série de casos revelou que o pneumomediastino é uma complicação rara na dermatomiosite e que ocorre em pacientes com história de pneumopatia intersticial e pode ser acompanhada por enfisema subcutâneo e pneumotórax.

Patients with pure dermatomyositis/polymyositis and anti-PM/Scl autoantibody resembling anti-synthetase syndrome / Pacientes com dermatomiosite/polimiosite pura e autoanticorpo anti-pm/scl sememlhante à síndrome anti-sintetase

OBJECTIVE: The anti-PM/Scl autoantibody has been described in patients with scleromyositis. However, there are scant studies evaluating its prevalence and reactivity in dermatomyositis and polymyositis. METHOD: A cross-sectional, single center study evaluating the anti-PM/Scl autoantibody in 85 dermatomyositis and 32 polymyositis patients, without overlapping syndrome, was conducted between 2000 and 2016. Clinical data and complementary examinations were reviewed from electronic medical records with pre-parameterized information. RESULTS: The mean age of dermatomyositis and polymyositis patients was 41.1 and 42.8 years, respectively. The presence of anti-PM/Scl was observed in 5 (5.9%) dermatomyositis and 2 (6.3%) polymyositis patients. Two of these patients also had the anti-Ku antibody. The relevant clinical manifestations of these 7 patients were constitutional symptoms (100% of cases), muscular (100%), pulmonary (85.7%) and joint (71.4%) involvement, "mechanic hands" (85.7%), Raynaud phenomenon (85.7%) and plantar hyperkeratosis (85.7%). The 7 patients had relapses of disease activity, but at conclusion of the present study, 5 had complete clinical response and 2 complete remission of the disease. CONCLUSION: There is a low frequency of the anti-PM/Scl autoantibody in dermatomyositis and polymyositis patients. In addition, patients with this autoantibody exhibit a similar pattern of manifestations to that of antisynthetase syndrome.

OBJETIVO: O autoanticorpo anti-PM/Scl foi descrito em pacientes com escleromiosite. No entanto, há escassos estudos avaliando sua prevalência e reatividade em dermatomiosite (DM) e polimiosite (PM). MÉTODOS: Estudo transversal, num único centro, que avaliou o autoanticorpo anti-PM/Scl em 85 DM e 32 PM, sem síndrome de sobreposição, no período entre 2000 e 2016. Os dados clínicos e os exames complementares foram revisados a partir de registros médicos eletrônicos com informações pré-parametrizadas. RESULTADOS: A média de idade dos pacientes com DM e PM foi, respectivamente, de 41,1 e 42,8 anos. A presença de anti-PM/Scl foi observada em 5 (5,9%) DM e 2 (6,3%) pacientes com PM. Dois desses pacientes também possuíam o anticorpo anti-Ku. As manifestações clínicas relevantes desses 7 pacientes foram sintomas constitucionais (100% dos casos), envolvimento muscular (100%), pulmonar (85,7%) e articular (71,4%), "mãos mecânicas" (85,7%), fenômeno de Raynaud (85,7 %) e hiperqueratose plantar (85,7%). Os 7 pacientes apresentaram recidivas da atividade da doença, mas, no final do presente estudo, 5 apresentaram resposta clínica completa e 2 remissões completas da doença. CONCLUSÃO: Há uma baixa freqüência do autoanticorpo anti-PM/Scl em pacientes com DM e PM. Além disso, os pacientes com este autoanticorpo apresentam um padrão semelhante de manifestações para a síndrome da antisintetase.

Insulin resistance is increased in adult patients with dermatomyositis / Resistência insulínica aumentada em pacientes com dermatomiosite

OBJECTIVE: To evaluate insulinemia in glucocorticoid naïve patients with dermatomyositis and to evaluate insulin resistance using the homeostatic model assessment of insulin resistance (HOMA2-IR). METHODS: This cross-sectional study included 25 dermatomyositis, non-diabetic glucocorticoid naïve patients. The control group consisted of 50 volunteers matched for age, gender, ethnicity, weight and height. The HOMA2-IR index was calculated from baseline insulin and glucose data. The International Myositis Assessment & Clinical Studies Group (IMACS) parameters were used to evaluate disease status. RESULTS: Mean age of the patients was 43.5 years and these were predominantly females. Patients had low disease activity according to IMACS parameters. Higher body mass index and waist circumference were observed in the dermatomyositis group compared to the control group. Insulin level and HOMA2-IR were also higher in patients with dermatomyositis. Moreover, analyzing dermatomyositis alone, the HOMA2-IR index correlated positively with weight, body mass index and waist circumference and was independent on disease status parameters. CONCLUSIONS: Patients with dermatomyositis had higher values for basal insulinemia, insulin resistance, body mass index and waist circumference. Moreover, HOMA2-IR moderately correlated with these anthropometric parameters. These metabolic abnormalities are related to the development of metabolic syndrome, one of the main comorbidities observed in dermatomyositis.

OBJETIVO: Avaliar a insulinemia em pacientes com dermatomiosite virgens de glicocorticoide e avaliar a resistência insulínica, utilizando o modelo de avaliação da homeostase de resistência insulínica (HOMA2-IR). MÉTODOS: Este estudo transversal incluiu 25 pacientes com dermatomiosites, não-diabéticos e sem uso prévio de glicocorticoides. Para o grupo de controle, 50 voluntários foram pareados por idade, gênero, etnia, peso e estatura. O índice HOMA2-IR foi calculado a partir de dados basais de insulina e glicose. Os parâmetros do International Myositis Assessment & Clinical Studies Group (IMACS) foram utilizados para avaliar o status da doença. RESULTADOS: A méda de idade dos pacientes foi de 43,5 anos, predominantemente do sexo feminino. Os pacientes apresentaram baixa atividade de doença de acordo com os parâmetros do IMACS. O índice de massa corporal e a circunferência da cintura foram maiores no grupo da dermatomiosite em comparação com o grupo controle. O nível de insulina e o HOMA2-IR também foram maiores em pacientes com dermatomiosite. Além disso, analisando a dermatomiosite isoladamente, o índice HOMA2-IR correlacionou-se positivamente com o peso, o índice de massa corporal e a circunferência da cintura e foi independente dos parâmetros de status da doença. CONCLUSÕES: Pacientes com dermatomiosite apresentam valores mais elevados de insulinemia basal, resistência à insulina, índice de massa corporal e circunferência da cintura. Além disso, o HOMA2-IR está moderadamente correlacionado com esses parâmetros antropométricos. Essas anormalidades metabólicas estão relacionadas ao desenvolvimento da síndrome metabólica, uma das principais comorbidades observadas na dermatomiosite.

Dermatomiositis juvenil: experiencia de 13 años en un hospital de atención terciaria. Análisis de 17 casos clínicos / Juvenile dermatomyositis: 13 years of experience in a tertiary care hospital. Analysis of 17 clinical cases / Dermatomiosite juvenil: 13 anos de experiência em hospital de nível terciário de atenção. Analise de 17 casos clinicos

La dermatomiositis juvenil (DMJ) es una miopatía inflamatoria adquirida de base inmunológica acompañada por alteraciones cutáneas características. El objetivo de este artículo es describir las características clínicas y exámenes complementarios de un grupo de 17 pacientes con diagnóstico de DMJ, su evolución y tratamiento. Material y método: se estudiaron los pacientes que se asistieron en la Policlínica de enfermedades de tejido conectivo del Centro Hospitalario Pereira Rossell (CHPR) en el período del 1 de octubre de 2003 al 1 de abril de 2017. Resultados: los rasgos clínicos más frecuentes de presentación fueron las manifestaciones cutáneas características, debilidad muscular, síntomas constitucionales, manifestaciones gastrointestinales y respiratorias. Las enzimas musculares estuvieron aumentadas en todos los casos. La resonancia nuclear magnética, electromiograma y la biopsia muscular fueron patológicos en todos los casos realizados. El tratamiento se realizó fundamentalmente en base a corticoides y fármacos inmunosupresores, siendo el metotrexate la droga de elección. En los casos graves o refractarios se asoció gamaglobulina, ciclofosfamida o ciclosporina. La duración del tratamiento tuvo una mediana de 3 años 10 meses. Se logró remisión en 47% de los pacientes. La evolución fue monofásica en 15,4%, de los casos, polifásica en 8% y crónica en 77%. No hubo fallecimientos registrados, ni enfermedad maligna asociada. Conclusiones: la DMJ es una enfermedad de baja incidencia. La mayoría de los pacientes tuvieron una evolución crónica. Esto determina la necesidad de un tratamiento inmunosupresor prolongado con los efectos adversos de la misma. Se logró la remisión en 47% de los pacientes. No se registraron fallecimientos en la serie estudiada.

Juvenile dermatomyositis (JDM) is an acquired inflammatory myopathy with an immunologic basis and characteristic cutaneous rash. The aim of this article is to describe clinical features and most important exams of a group of 17 patients with JDM their evolution and treatment. Methods: children with JDM recruited from Connective tissue Diseases Office of Pereira Rossell Hospital from 1/10/2003 through 1/4/2017 were studied. Results: the most frequent features were: characteristic cutaneous rash, muscle weakness, systemic symptoms, gastrointestinal and respiratory manifestations. The diagnostic investigations showed an increase serum muscle enzymes in all patients. The nuclear magnetic resonance, electromyogram and muscle biopsy resulted abnormal in all the investigated cases. Treatment was based on corticosteroids and immunosuppressive drugs being methotrexate the preferred drug. In severe or refractory cases cyclophosphamide, human gammaglobulin or cyclosporine were associated. Median treatment length was 3 years 10 months Remission was achieved in 47 percent. The evolution was monophasic in 15.4 percent, polyphasic in 7.7 and chronic in 77 percent. No deaths were registered neither malignant associated diseases Conclusions: JDM is an infrequent illness. Most of the patients had chronic evolution. This obliges to prolonged immunosuppression with its adverse effects. Remission was achieved in 47% of the cases. No deaths were registered in this population.

A dermatomiosite juvenil (DMJ) é uma miopatia inflamatória imunológica adquirida acompanhada de alterações cutâneas características. O objetivo deste artigo é descrever as características clínicas e os exames complementares de um grupo de 17 pacientes diagnosticados com DMJ, sua evolução e tratamento. Materiais e métodos: foram estudados os pacientes que compareceram à Policlínica das Doenças do Tecido Conjuntivo no Centro Hospitalar Pereira Rossell (CHPR) no período de 01/10/2003 a 04/01/2017. Resultados: as características clínicas mais frequentes foram manifestações cutâneas características, fraqueza muscular, sintomas constitucionais, manifestações gastrointestinais e respiratórias. As enzimas musculares estiveram aumentadas em todos os casos. A Ressonância nuclear magnética, o eletromiograma e a biópsia muscular foram patológicos em todos os casos. O tratamento foi baseado principalmente em corticosteroides e drogas imunossupressoras, e o metotrexato foi a droga de escolha. Em casos graves ou refratários, também se administrou gamaglobulina, ciclofosfamida ou ciclosporina. A duração do tratamento teve uma mediana de 3 anos e 10 meses. A remissão foi alcançada em 47% dos pacientes. A evolução foi monofásica em 15,4% dos casos, polifásica em 8% e crônica em 77%. Não houve mortes registradas, nem doença maligna associada. Conclusões: a DMJ é uma doença de baixa incidência. A maioria dos pacientes teve evolução crônica. Isso determina a necessidade de um tratamento imunossupressor prolongado com os seus conseguintes efeitos adversos. A remissão foi alcançada em 47% dos pacientes. Nenhuma morte foi registrada na série estudada.

Terapia celular combinada com membranas de biopolímeros melhora a cicatrização de úlceras em paciente com dermatomiosite juvenil / Combining cell therapy with biopolymer films improves wound healing in a juvenile dermatomyositis patient

Introdução: Dermatomiosite juvenil (DMJ) é doença sistêmica que afeta a musculatura proximal e a pele de crianças. A doença ulcerada é um desafio terapêutico. Objetivo: Avaliar a melhora da doença ulcerada na DMJ, pelo uso de terapia celular. Métodos: Realização de cocultura de fibroblastos e queratinócitos autólogos e aplicação dessas células nas úlceras juntamente com cola de fibrina e colocação de membrana de quitosana-alginato ou quitosana-xantana sobre as lesões. Resultados: Menos de 12 horas após a terapia, o paciente referiu completa eliminação da dor e, dentro de dois dias, estava presente tecido de cicatrização. Algumas das úlceras estavam quase completamente cicatrizadas no final da primeira semana, e algumas das calcinoses desapareceram. Essa técnica não cura a doença, mas melhora a qualidade de vida, sendo possível criopreservar as células saudáveis do paciente para tratar novas lesões. Sendo as células de origem autóloga, elimina-se o risco de rejeição. Além disso, esse procedimento não necessita de debridamento das lesões nem hospitalização. Conclusões: A aplicação de culturas autólogas de fibroblastos e queratinócitos em úlceras já é considerada tratamento efetivo em pacientes com queimaduras e outras feridas cutâneas e, agora mostrou-se também eficaz no tratamento de feridas na DMJ.

Introduction: Juvenile dermatomyositis (JDM) is a systemic disease that affects children's proximal musculature and skin. The ulcerated stage of the disease is a therapeutic challenge. Objective: To evaluate the improvement of ulcerated stage of JDM caused by the use of cell therapy. Methods: Co-culture of autologous fibroblasts and keratinocytes, application of these cells in ulcers in conjunction with fibrin glue, and placement of chitosan-alginate or chitosan-xanthan membrane on the lesions. Results: Less than 12 hours after therapy, the patient reported complete cessation of pain and, within 2 days, healing tissue emerged. Some of the ulcers were almost completely healed by the end of the 1st week, and some of the calcinoses disappeared. This technique does not cure the disease, however it improves the patient's quality of life, and it is possible to cryopreserve healthy cells to treat new lesions. Given the fact that the cells are of autologous origin, the risk of rejection is eliminated. Furthermore, this procedure does not require debridement of the lesions or hospitalization. Conclusions: The application of autologous cultures of fibroblasts and keratinocytes in ulcers is already considered an effective treatment in patients with burns and other skin wounds, and has now also been proven effective in the treatment of wounds in JDM.

Morphological alterations of upper gastrointestinal tract in patients with new onset-dermatomyositis: correlation with demographic, clinical and laboratory features / Alterações morfológicas do trato intestinal superior em portadores de dermatomiosite de instalação recente: correlações com características demográficas, clínicas e laboratoriais

OBJECTIVE: To endoscopically assess the upper digestive tract of adult patients with newly diagnosed dermatomyositis; to correlate possible changes in the gastrointestinal tract with demographic, clinical and laboratory features in this population. METHOD: A cross-sectional study evaluating 65 newly diagnosed dermatomyositis cases from 2004 to 2015 was carried out. We excluded patients with clinically amyopathic dermatomyositis, overlap dermatomyositis, polymyositis, liver diseases, prior gastric surgery, upper gastrointestinal tract symptoms (except for upper dysphagia), systemic infections, alcohol consumption and smoking. RESULTS: Mean age of patients was 44.9 years, with disease duration of four months. Endoscopic findings were observed in 70.8% of patients. (1) Esophageal disease/gastric distress was documented in 18.5% of patients: erosive distal esophagitis (16.9%) and non-erosive distal esophagitis distal (1.5%); (2) gastric distress in 63.1% of cases: antral gastritis (42.3%) and pangastritis (27.8%); (3) duodenal involvement in 15.4% of patients: bulbar duodenitis (10.9%) and duodenal ulcers (7.7%). There were no neoplasic lesions. On multivariate analysis, erosive distal esophagitis was less associated with older patients. Males had a higher prevalence of erosive gastritis. Enanthematous pangastritis was less associated with lesions with "V-neck" sign lesions. CONCLUSIONS: This study provides the first estimates of the prevalence of high endoscopic findings in adult patients with newly diagnosed dermatomyositis. The results may be relevant to guide conduct in digestive disorders with upper digestive endoscopy, and point to the need for pharmacological prevention of digestive tract lesions in these patients. Further studies are needed to validate this data and evaluate patients with dyspeptic symptoms.

OBJETIVOS: Avaliar os exames de endoscopia digestiva alta (EDA) de pacientes adultos com DM (dermatomiosite) recém-diagnosticados; correlacionar eventuais alterações do trato gastrintestinal com dados demográficos, clínicos, e medicamentosos desta população. MÉTODO: Estudo transversal, em que foram avaliados 65 casos de DM recém-diagnosticados, no período entre 2004 a 2015. Foram excluídos casos de DM clinicamente amiopática, sobreposição com DM, hepatopatias, cirurgia gástrica prévia, sintomas do trato gastrointestinal (exceto disfagia alta), quadros infecciosos sistêmicos, etilismo e tabagismo. RESULTADOS: A média idade dos pacientes foi de 44,9 anos, com um tempo de sintomas atribuídos a DM de quatro meses. Alterações endoscópicas foram encontradas em 70,8% dos pacientes. O acometimento esofágico/gástrico foi documentado em 18,5% dos pacientes: esofagite distal erosiva (16,9%) e esofagite distal não-erosiva (1,5%); alterações gástricas em 63,1% dos casos: gastrite antral (42,3%) e pangastrite (27,8%); o acometimento duodenal em 15,4% dos pacientes: bulboduodenite (10,9%) e úlcera duodenal (7,7%). Não foram detectadas lesões malignas. Em análise multivariada, a esofagite distal erosiva esteve menos associada a indivíduos de idade maior. Sexo masculino apresentava mais diagnóstico de gastrite erosiva. A pangastrite enantemática esteve menos associada a lesões em "V" do decote. CONCLUSÕES: O presente estudo estima, pela primeira vez, a prevalência de alterações endoscópicas altas em pacientes adultos com DM recém-diagnosticada. Os resultados podem ser relevantes para guiar potenciais alterações digestivas com exame de EDA, bem como apontar para necessidade de prevenção medicamentosa de lesões do trato digestivo nestes pacientes.

Muscle biopsies in dermatomyositis and polymyositis: practical relevance of analyzing different levels of histological sections of the same muscular compartment / Biópsias musculares em dermatomiosite e polimiosite: relevância prática da análise de diferentes níveis de cortes histológicos de um mesmo compartimento muscular

ABSTRACT Introdution: It is frequent in medical practice to have findings with normal aspects in histological muscle biopsies from patients with dermatomyositis (DM) or polymyositis (PM). This happens because, for example, the inflammatory infiltrate occurs in foci. Objectives: To evaluate the morphological and histological inflammatory infiltrate in various histological section levels. In addition, to correlate these findings with patients' clinical, laboratory and therapeutic data. Methods: Cross-sectional study in which muscle biopsies from 34 patients were evaluated (DM and PM). From each muscle/patient biopsy block, three levels of histological sections were made (I, II, III) with 400-µm interval between adjacent levels (I × II, and II × III). Semi-quantitative analyses were performed in the following parameters between the adjacent levels: muscle fiber features, conjunctive tissue, vessels, presence of inflammatory cell infiltration. Results: Time spans between muscle biopsy and symptom onset of DM and PM patients were 5.5 and 3.5 months, respectively. All histological parameters analyzed varied between levels and did not correlate with the demographic, clinical, laboratory and therapeutic data before muscle biopsy (p > 0.05). Conclusion: Our results stress the importance of evaluating different levels of histological sections from the same muscle biopsy block, in order to minimize possible false-negative results. In addition, the data reinforce that besides the inflammatory infiltrate, the other histological parameters analyzed also occur in foci, justifying the dissociation between these parameters and clinical patients.

RESUMO Introdução: É frequente na prática médica encontrar achados histológicos com aspectos dentro da normalidade em biópsias musculares de pacientes com dermatomiosite (DM) ou polimiosite (PM). Isso se deve ao fato de, por exemplo, o infiltrado inflamatório ocorrer em focos. Objetivos: Avaliar os aspectos morfológicos e o infiltrado inflamatório em diversos níveis histológicos, bem como correlacionar esses achados com os dados clínicos, laboratoriais e terapêuticos dos pacientes. Métodos: Estudo transversal no qual foram avaliadas biópsias musculares de 34 pacientes (DM e PM). Para cada bloco de biópsia muscular/paciente, foram realizados três níveis de cortes histológicos (I, II e III), com intervalos de 400 µm entre os níveis adjacentes (I × II e II × III). Foram analisados semiquantitativamente os seguintes parâmetros entre os níveis adjacentes: características das fibras musculares, tecido conjuntivo, vasos e presença de infiltrado de células inflamatórias. Resultados: O tempo entre a realização da biópsia muscular e o início de sintomas dos pacientes com DM e PM foi, respectivamente, de 5,5 e 3,5 meses. Todos os parâmetros histológicos analisados variaram entre os níveis e não se correlacionaram com os dados demográficos, clínicos, laboratoriais e terapêuticos pré-biópsia muscular (p > 0,05). Conclusão: Nossos resultados reforçam a importância de avaliar diferentes níveis de cortes histológicos de um mesmo bloco de biópsia muscular com o objetivo de minimizar eventuais resultados falso negativos. Além disso, os dados evidenciam que, além do infiltrado inflamatório, os demais parâmetros histológicos analisados também ocorrem em focos, justificando a dissociação entre esses parâmetros e a clínica dos pacientes.