Características clínicas, inmunológicas y daño de órganos en pacientes con miopatías inflamatorias idiopáticas / Organ damage clinical and immunological characteristics in patients with idiopathic inflammatory myopathies

Introducción: Las miopatías inflamatorias idiopáticas constituyen un grupo de enfermedades musculares caracterizadas por debilidad muscular crónica e inflamación muscular de etiología desconocida. Objetivo: Identificar las características clínicas e inmunológicas y su relación con el daño de órganos en los pacientes con miopatías inflamatorias idiopáticas. Métodos: Se realizó estudio observacional, descriptivo, transversal, en 52 pacientes con diagnóstico de miopatía inflamatoria idiopática, seguidos en la consulta protocolizada de Reumatología del Hospital Clínico Quirúrgico Hermanos Ameijeiras entre enero 2016 y enero 2017. Para las variables cualitativas se calcularon los porcentajes de cada grupo. Se utilizó Chi-cuadrado de Pearson (estadístico exacto de Fisher). Nivel de significación del 95 por ciento (α = 0,05) para relacionar la presencia de anticuerpos y el tipo de miopatía así como la presencia de manifestaciones clínicas de MII. Resultados: El 80,8 por ciento fueron mujeres y 86,5 por ciento de procedencia urbana. La edad media al comienzo fue 42,8 ± 13,2 años, tiempo de demora al diagnóstico de 8,8 ± 7,0 meses, tiempo medio de evolución de la enfermedad de 7,5 ± 7,1 años. El 80,8 por ciento estaba en remisión, 50 por ciento tenía anticuerpos específicos. La hipertensión arterial se encontró en 28,8 por ciento de los pacientes y 23,1 por ciento presentó neumonía intersticial. La artritis estuvo presente en 96,2 por ciento. El 26,9 por ciento presentaron anticuerpos específicos Jo-1 y 21,2 por ciento Ro 52. Conclusiones: Predominaron los pacientes del sexo femenino en la cuarta década de la vida de procedencia urbana, los anticuerpos específicos encontrados más frecuentes fue el anti Jo-1, asociado a la presencia de neumopatía intersticial(AU)

Introduction: Idiopathic inflammatory myopathies constitute a group of muscle diseases characterized by chronic muscle weakness and muscle inflammation of unknown etiology. Objective: To identify the clinical and immunological characteristics and their relationship with organ damage in patients with idiopathic inflammatory myopathies. Methods: An observational, descriptive, cross-sectional study was carried out in 52 patients with diagnosis of idiopathic inflammatory myopathy, followed in the protocolized consultation of Rheumatology at Hermanos Ameijeiras Clinical and Surgical Hospital from January 2016 to January 2017. For the qualitative variables, the percentages of each group were calculated. Pearson's Chi-square (Fisher's exact statistic) was used. 95percent significance level (α = 0.05) was used to relate the presence of antibodies and the type of myopathy as well as the presence of clinical manifestations of MII. Results: 80.8percent were women and 86.5percent of urban origin. The mean age at the beginning was 42.8 ± 13.2 years, time delay to diagnosis was 8.8 ± 7.0 months, mean time of evolution of the disease of 7.5 ± 7.1 years. 80.8percent were in remission, 50percent had specific antibodies. Hypertension was found in 28.8percent of the patients and 23.1percent had interstitial pneumonia. Arthritis was present in 96.2percent. 26.9percent had specific Jo1 antibodies and 21.2percent had Ro 52. Conclusions: Urban female patients in the fourth decade of life predominated, the most frequent specific antibodies found was anti-Jo-1, associated with the presence of interstitial lung disease(AU)

Clinical and immunological characteristics of 88 cases of overlap myositis / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinical and immunological characteristics of overlap myositis (OM) patients.@*METHODS@#The data of 368 patients with idiopathic inflammatory myopathies (IIMs) admitted to Peking University People's Hospital from January 2004 to August 2020 were analyzed retrospectively, including demographic characteristics, clinical characteristics (including fever, Gottron' s sign/papules, Heliotrope rash, V-sign, Shawl sign, Mechanic' s hands, skin ulceration, periungual erythema, subcutaneous calcinosis, dysphagia, myalgia, myasthenia, arthritis, Raynaud' s phenomenon, interstitial lung disease, pulmonary hypertension and myocardial involvement), laboratory characteristics, immunological characteristics [including antinuclear antibodies, rheumatoid factors, myositis-associated autoantibodies (MAAs) and myositis-specific autoantibodies (MSAs)] and survival. The clinical and immunological characteristics and prognostic differences of OM and non-OM were compared. The Kaplan-Meier and Log Rank methods were used to analyze the survival.@*RESULTS@#A total of 368 patients were included. 23.9% (88/368) of IIMs patients were OM patients. Among the 88 OM patients, 85.2% (75/88) of them were female, and the median interval between disease onset and diagnosis was 13.5 months. The incidence of overlapped connective tissue diseases in the OM patients was dermatomyositis (DM) in 60.2%, polymyositis (PM) in 3.4%, immune-mediated necrotizing myopathy (IMNM) in 2.3% and anti-synthetase syndrome (ASS) in 34.1%. Compared with the non-OM patients, the proportion of the females in the OM patients was higher (85.2% vs. 72.1%, P=0.016), the OM patients had longer disease duration [13.5(4.5, 48.0) months vs. 4.0(2.0, 12.0) months, P < 0.001]. As for clinical characteristics, compared with the non-OM patients, the incidence of V-sign (25.0% vs. 44.6%, P=0.001) and periungual erythema (8.0% vs. 19.6%, P=0.013) were lower; the incidence of Raynaud's phenomenon (14.8% vs. 1.8%, P < 0.001), interstitial pneumonia (88.6% vs. 72.1%, P=0.001), pulmonary hypertension (22.7% vs. 7.5%, P < 0.001) and myocardial involvement (18.2% vs. 9.3%, P=0.033) were higher. As for immunological characteristics, compared with the non-OM patients, the incidence of elevated aspartate aminotransferase (AST) (31.8% vs. 45.0%, P=0.035) was lower and elevated C-reactive protein (CRP) (58.0% vs. 44.6%, P=0.037) was higher; the positive rates of antinuclear antibodies (ANA) (85.1% vs. 63.4%, P=0.001) and rheumatoid factors (RF) (40.2% vs. 17.8%, P < 0.001) and anti-Ro-52 (71.6% vs. 56.1%, P=0.038) in serum were higher. There was no significant difference in the survival between the OM patients and non-OM patients.@*CONCLUSION@#Pulmonary hypertension and myocardial involvement were frequently observed in OM.

Clasificación y manejo actual en dermatomiositis juvenil / Classification and current management in juvenile dermatomyositis

La Dermatomiositis Juvenil representa el 75-80% de las miopatías inflamatorias juveniles. Si bien, tiene baja incidencia y prevalencia, presenta importante morbilidad dada por sus manifestaciones cutáneas, musculares, pulmonares, gastrointestinales, cardiacas, entre otras. Corresponde a un desorden poligénico con múltiples factores gatillantes, que determina el desarrollo de una vasculopatía que lleva a atrofia muscular, inflamación y activación de vías del IFN-1. Actualmente su diagnóstico se basa en las guias EULAR/ACR (2017). En los últimos años, se han descubiertos distintos subtipos de la enfermedad, basados en el perfil de autoanticuerpos específicos de miositis, lo que ha permitido establecer pronóstico y estrategias terapéuticas personalizadas. El manejo farmacológico continúa basándose principalmente en el uso de corticoesteroides y DMARDs, así como también terapia biológica; en los últimos años, los inhibidores JAK han mostrado resultados promisorios, convirtiéndose en la más nueva alternativa terapéutica para el control de la enfermedad.

Juvenile Dermatomyositis represents 75-80% of juvenile inflammatory myopathies. Although it has a low incidence and prevalence, it presents significant morbidity due to its cutaneous, muscular, pulmonary, gastrointestinal and cardiac manifestations, among others. It corresponds to a polygenic disorder with multiple triggering factors, which determines the development of a vasculopathy that leads to muscle atrophy, inflammation and activation of IFN-1 pathways. Currently its diagnosis is based on the EULAR/ACR guidelines (2017). In recent years, different subtypes of the disease have been discovered, based on the profile of myositis-specific autoantibodies, which has made it possible to establish prognosis and personalized therapeutic strategies. Pharmacological management continues to be based mainly on the use of corticosteroids and DMARDs, as well as biological therapy; In recent years, JAK inhibitors have shown promising results, becoming the newest therapeutic alternative for disease control.

Dermatomiositis juvenil. Sistematización de casos / Juvenile dermatomyositis. Case systematization

Introducción: La búsqueda bibliográfica realizada estableció una problemática a atender: la escasez de estudios de caso de dermatomiositis juvenil, por lo que la presente investigación pretende arrojar luz sobre esta patología, poco reflejada en la literatura médica. Nótese, además, que la sistematización puede servir de reservorio bibliográfico para estudios de posgrados de especialistas médicos. Dermatomiositis juvenil. Sistematización de casos: sistematizar y comparar 10 casos de dermatomiositis juvenil, publicados en las principales revistas médicas en cuanto a la edad del paciente, antecedentes de salud, cuadro clínico, resultados de complementarios, diagnóstico diferencial, manejo. Dermatomiositis juvenil. Sistematización de casos: hasta un 30 por ciento de los pacientes con dermatomiositis juvenil puede presentar calcinosis, especialmente en puntos de presión como codos, rodillas, dedos y glúteos. La calcinosis puede estar presente en el momento del diagnóstico, pero corrientemente se establece luego de 1 a 3 años y puede provocar la aparición de úlceras cutáneas, mengua de los rangos articulares, dolor e inflamación local. Alrededor del 10 por ciento de los pacientes con dermatomiositis juvenil puede presentar úlceras cutáneas. El estudio de su evolución suele anunciar un curso severo de la enfermedad con debilidad constante, calcinosis extensa y mala respuesta al tratamiento. Conclusiones: resulta importante sistematizar los estudios relacionados con casos de alteraciones dermatológicas de la dermatomiositis juvenil, ya que la enfermedad constituye una manifestación notable, tanto como marcador de actividad como de su daño derivado. Así también, pueden coadyuvar a lograr una percepción estadística más clara de la tasa de morbilidad y su consecuente relación con los pronósticos(AU)

Introduction: Literature search established a problem to be addressed: the scarcity of case studies of juvenile dermatomyositis, which is why this research aims to shed light on this pathology, little reflected in the medical literature. Note also that systematization can serve as a bibliographic reservoir for postgraduate studies of medical specialists. Dermatomiositis juvenil. Sistematización de casos: to systematize and compare 10 cases of juvenile dermatomyositis, published in the main medical journals regarding patient's age, health history, clinical picture, complementary results, differential diagnosis, management. Dermatomiositis juvenil. Sistematización de casos: up to 30 percent of patients with juvenile dermatomyositis can present calcinosis, especially in pressure points such as elbows, knees, fingers and buttocks. Calcinosis may be present at the time of diagnosis but is usually established after 1 to 3 years and may cause the appearance of skin ulcers, decreased joint ranges, pain and local inflammation. About 10 percent of patients with juvenile dermatomyositis may have skin ulcers. The study of its evolution usually announces a severe course of the disease with constant weakness, extensive calcinosis and poor response to treatment. Conclusions: it is important to systematize the studies related to cases of dermatological alterations of juvenile dermatomyositis, since the disease constitutes a remarkable manifestation, both as a marker of activity and of its derived damage. Likewise, they can help to achieve a clearer statistical perception of the morbidity rate and its consequent relationship with prognosis(AU)

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.

Analysis of sexual function of patients with dermatomyositis and polymyositis through self-administered questionnaires: a cross-sectional study / Análise da função sexual de pacientes com dermatomiosite e polimiosite através de questionários autoaplicados: um estudo transversal

Abstract 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.

Resumo Introdução: Até o presente momento, não há descrições na literatura da avaliação ginecológica e da função sexual em pacientes do sexo feminino com dermatomiosite (DM) e polimiosite (PM). Objetivos: Avaliar a função sexual em pacientes do sexo feminino com DM/PM. Casuística e métodos: Estudo transversal unicêntrico em que 23 pacientes (16 DM e sete PM), entre 18 e 40 anos, foram comparadas com 23 mulheres saudáveis, com a mesma faixa etária. As características sobre a função sexual foram obtidas por meio da aplicação dos questionários Female Sexual Quotient (FSQ) e Female Sexual Function Index (FSFI) validados para a língua portuguesa do Brasil. Resultados: A média de idade das pacientes foi comparável à dos controles (32,7 ± 5,3 vs. 31,7 ± 6,7 anos), assim como a distribuição de etnia e da classe socioeconômica. Quanto às características ginecológicas, pacientes e controles saudáveis não apresentaram diferenças em relação à idade na menarca e às porcentagens de dismenorreia, menorragia, síndrome pré-menstrual, dor no meio do ciclo, secreção mucocervical e corrimento vaginal. O escore de pontuação do FSQ, assim como todos os domínios do questionário do FSFI (desejo, excitação, lubrificação, orgasmo e satisfação), estavam significantemente diminuídos nas pacientes comparativamente com os controles, 60,9% das pacientes apresentavam algum grau de disfunção sexual. Conclusões: Este foi o primeiro estudo que identificou disfunção sexual nas pacientes com DM/PM. Assim, uma abordagem multidisciplinar é essencial para pacientes com miopatias inflamatórias idiopáticas para fornecer medidas de prevenção e cuidados para sua vida sexual e propiciar uma melhor qualidade de vida das pacientes e de seus parceiros.

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.

Atualização em dermatomiosite / Updating in dermatomyositis

JUSTIFICATIVA E OBJETIVOS: A dermatomiosite é umadoença sistêmica crônica, de etiologia desconhecida, que se caracterizapor acometimento inflamatório da pele e dos músculos.O diagnóstico baseia-se nos achados clínicos e laboratoriais. Os corticoides são a terapia mais utilizada. As causas de óbito maisfrequentes são neoplasia maligna, septicemia e fibrose pulmonar.O objetivo deste estudo foi efetuar uma ampla revisão de literatura com foco no reconhecimento dos principais achados clínicos e no tratamento desta doença.CONTEÚDO: A dermatomiosite é uma doença sistêmica crônica que se caracteriza por acometimento inflamatório da pele e dos músculos. Possui duas formas principais: miopática, maisfrequente, onde se encontram lesões musculares e cutâneas; e amiopática, somente com lesões cutâneas. O sexo feminino é o mais afetado, e a idade média do diagnóstico é 40 anos. Manifestações cutâneas são observadas em todos os pacientes. Das alterações sistêmicas, a manifestação muscular mais frequente é a perda de força proximal, e a manifestação pulmonar mais comum é a pneumopatia intersticial. Podem ser observadas neoplasias durante o seguimento da doença, sendo mais frequentes nos pacientes acima de 60 anos. A desidrogenase lática é a enzima muscular alterada na maioria dos casos. O diagnóstico da dermatomiositepode ser realizado por exame anatomopatológico de biópsia muscular,além de eletroneuromiografia. Os corticoides são os mais utilizados. As causas de óbito mais frequentes são neoplasia maligna,septicemia e fibrose pulmonar. Não há causa conhecida. Odiagnóstico baseia-se nos achados clínicos e laboratoriais.CONCLUSÃO: Através da analise dos dados bibliográficos foi possível concluir que a dermatomiosite é uma doença de diagnóstico predominantemente clínico. Os exames laboratoriais e de imagem constituem importantes confirmadores do quadro, mas nunca são identificadores isolados, sendo sempre a clínica, soberana no diagnóstico desta doença.(...)

BACKGROUND AND OBJECTIVES: The dermatomyosits is a systemic chronic disease, of unknown etiology, which is characterized for inflammatory attack of the skin and muscles. The diagnosis is based on clinical and laboratory finds. The corticosteroids are the most used therapy. The most frequent causes of death are: the malignant neoplasia, the septicemia and the pulmonary fibrosis. A bibliographical revision was carried out, with the objective of attracting attention of the doctors for the recognition of the principal clinical finds of this illness. CONTENTS: The dermatomyositis is a systemic chronic disease that is characterized by the inflammatory attack of the skin and muscles. It has two principal forms: miopatic that is more frequent, and presents muscular and skin injuries; and the amiopatic that presents skin injuries only. The feminine sex is more affected mainly at the middle age of 40s. Skin manifestations are observed in all the patients. Among the systemic alterations,the most frequent muscular demonstration is the loss of strength proximal, and the commonest pulmonary demonstration is interstitial pneumopathy. Neoplasis can be observed during the continuation of the disease, and is more frequent in the patients above 60 years old. The Lactic dehydrogenase means that the muscular enzyme is altered in most of the cases. The diagnosis of the dermatomyositis can be done for the muscular biopsy applyinganatomopatologic exam, besides electroneuromiography examination. The corticosteroids are the most used therapy. The most frequent causes of death are the malignant neoplasis, sepsis, and the pulmonary fibrosis and there is no known cause. The diagnosis is based on the clinical and laboratories finds.CONCLUSION: Through the analyzes of the bibliographical facts it is possible to conclude that dermatomiositis is a disease of predominantly clinical diagnosis.(...)

Association d'un lupus erythemateux systemique et d'une dermatomyosite

Systemic lupus erythematosus [SLE] associated with dermatopolymyositis [OM]. This association is rare. Diagnosis may be difficult because of their common clinical findings. We report here a case. A 22-year-old man was admitted for arthritis with fever, diffuse myalgia and periorbital skin heliotrope rash. Electromyogram and muscular biopsy were suggestive of DM. The patient was treated with oral prednisone. Two months and a half later, he was admitted for impure nephrotic syndrome in relation with diffuse proliferative glomerulonephritis. Antibodies against native double- stranded- DNA were positive, and normal skin biopsy showed immune complex deposit on dermo-epidermic junction, suggestive of SLE. The patient was treated with high doses of prednisone and 6 monthly intravenous pulses of cyclophosphamide. Skin lesions and nephrotic syndrome improved. Presently, the patient remains asymptomatic. While being of different pathogenesis, SLE and DM may coexist in the same patient

Clinical spectrum of inflammatory myositis in South India--a ten year study.

OBJECTIVE: To describe the clinical spectrum of inflammatory myopathies at a referral hospital in South India. METHODS: Patients were assessed for the pattern of muscle involvement, for the presence of arthritis, Raynaud's phenomenon, interstitial lung disease (ILD) and cardiac involvement. Muscle enzymes, electromyogram (EMG) and muscle biopsies were done. RESULTS: Eighty seven patients with inflammatory myopathies were encountered over 10 years. These included 24 with adult polymyositis, 26 with adult dermatomyositis, one with amyopathic dermatomyositis, five with juvenile myositis, one with dermatomysitis following carcinoma breast and 30 with overlap with other connective tissue diseases. There was a female preponderance (M:F = 1:2.35) except in juvenile myosits group (M:F = 1.5:1). The mean age of onset in years was 33.26 in adult polymyositis, 35.03 in adult dermatomyositis, 7.4 in juvenile dermatomyositis, 42 in malignancy-associated dermatomyositis and 25.51 in the overlap group. Proximal muscle weakness was seen in 98.8% patients, dysphagia in 33.3%, distal muscle weakness in 12.5%, respiratory muscle weakness in 9.2% and dysphonia in 4.6%. Other features included arthritis 35.63%, interstitial lung disease (ILD) 9.2%, Raynaud's 5.7%, myocarditis 4.6% and conduction disturbances 1.15%. Eleveated muscle enzymes were seen in 85.1% patients. Eletromyogram was positive in 66.6%. Muscle biopsy was positive in 85.29%. Anti-nuclear antibody was positive in 67.24%. All received steroids, non-responders needed methotrexate (13 patients) or azathioprine (11 patients). Death occurred in 10 (seven with dermatomyositis predominantly due to respiratory involvement and three with overlap). CONCLUSION: There was female preponderance except in juvenile myositis group. Proximal muscle weakness was the commonest feature. ILD was the commonest respiratory problem, while myocarditis was the commonest cardiac problem seen. Response to therapy and prognosis in polymyositis were good with no mortality during the study period. Death in the dermatomyositis group was mainly due to respiratory involvement.

Caracteríticas de las miopatías inflamatorias idiopáticas en la población mexicana / Idiopathic inflammatory myophaty: characteristics in the Mexican population

La mayoría de las descripciones epidemiológicas y clínicas respecto a las miopatías inflamatorias idiopáticas (MII) corresponden a población anglosajona y existen muy pocos estudios sobre las características de este grupo de padecimientos en la población mestiza mexicana. En este estudio se analizaron las características de los pacientes con diagnóstico de MII atendidos en el Servicio de Reumatología del Centro Médico Nacional 20 de Noviembre del Instituto de Seguridad y Servicio Social para Trabajadores del Estado de enero 1996 a julio de 1997. Fueron incluidos 42 pacientes con diagnóstico de MII, 34 del sexo femenino y ocho del masculino, todos de raza mestiza. La MII se clasificó como polimiositis en 12 casos, dermatomiositis en 22, juvenil en seis y asociada a otra enfermedad de tejido conectivo en dos. La edad promedio de los grupos adultos fue 33.3 años y en el grupo juvenil fue de siete años. Un gran porcentaje de pacientes (84 por ciento) refirió síntomas inespecíficos previos a la aparición de debilidad muscular o alteraciones cutáneas típicas. La evolución del padecimiento fue limitada en 15 enfermos, con exacerbaciones y remisiones en 23 y progresiva en cuatro. Se encontró mayor frecuencia de MII tipo II que lo reportado en población anglosajona, que se caracterizó además por inicio a edad temprana, evolución más agresiva y peor pronóstico en relación con los otros tipos. Del total de pacientes, sólo el 19 por ciento remitió con esteroides, el resto requirió de la adición de uno o más inmunosupresores. Se observó relación estadísticamente significativa entre el menor tiempo de evolución al iniciar el tratamiento y la obtención de remisión

Dermatomiosite e câncer de mama / Dermatomyositis and breast cancer

Os autores relatam um caso de dermatomiosite em mulher jovem com câncer de mama. A incidência de malignidade nas pacientes com dermatomiosite parece ser cinco a sete vezes maior que na populaçao geral. Se presente, a malignidade pode preceder ou ocorrer concomitantemente ou após o diagnóstico de dermatomiosite. O tratamento do câncer associado pode nao alterar o curso da miosite ou da doença cutânea. Essa associaçao parece ser resultado de uma funçao imunológica inadequada. Alguns desses tumores poderiam ser detectados em fase mais precoce, proporcionando melhor prognóstico.

Asociación colitis ulcerativa idiopática y dermatomiositis

Se informa un caso de dermatomiositis en un paciente de 46 años asociado a colitis ulcerativa idiopática (CUI). Es el quinto caso informado en la literatura universal. En los cuatro casos reportados previamente, la CUI precede a la dermatomiositis hasta por 17 años en un caso. Dos pacientes son de origen caucásico y los otros dos de origen japones. En nuestro paciente de origen mestizo, la dermatomiositis precedio a la CUI por siete meses. Es posible que esta asociación sea por azar; desafortunadamente , no se han estudiado las familias desde el punto de vista inmunogénetico en los reportes de la literatura.

Complejo dermatomiositis-polimiositis: presentación de un caso / Dermatomyositis-polymyosistis'complex: report of a case

Se presenta un caso de poliomiositis en un niño con manifestaciones dermatológicas y se comenta la fecuencia de esta asociación en una población general. Se revisó la sintomatología en los casos típicos y se mencionan algunas enfermedades que deben entrar en el diagóstico diferencial. También se comentaon los exámenes de laboratorio y se puso énfasis en la importancia de la biopsia de músculo para confirmar el diagnóstico y finalmente revisó brevemente el tratamiento de la misma enfermedad