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.(...)

Juvenile idiopathic inflammatory myopathies: the value of magnetic resonance imaging in the detection of muscle involvement

CONTEXT: One of the major current challenges related to juvenile idiopathic inflammatory myopathy is the search for highly sensitive and specific non-invasive methods for diagnosis as well as for follow-up. OBJECTIVES: The aim of our study was to describe typical magnetic resonance imaging findings and to investigate the usefulness of this method in detecting active muscle disease in juvenile dermatomyositis and juvenile systemic lupus erythematosus patients. DESIGN: Transverse study, blinded assessment. SETTING: University referral unit (Pediatric Rheumatology section, Department of Pediatrics, Universidade Federal de Sao Paulo / Escola Paulista de Medicina). SAMPLE: Thirteen patients (9 girls) with dermatomyositis, as well as 13 patients (12 girls) with juvenile systemic lupus erythematosus and 10 normal children (5 girls), were enrolled in the study. MAIN MEASUREMENTS: Qualitative and quantitative analyses of gluteus maximus, quadriceps, adductors and flexors were performed and evaluated by two radiologists, blinded to all clinical information. Spin-echo in T1, DP, T2 and IR was used in all MRI images. RESULTS: The different muscle groups presented non-uniform involvement in the patients. The patients with dermatomyositis presented acute and chronic muscular alterations, while those with lupus presented only chronic myopathy, especially atrophy. In the dermatomyositis group, the major alterations were found in the gluteus and flexor regions (signal intensity and fat replacement). The signal intensity was increased in all acute myopathies. CONCLUSION: The qualitative and quantitative resonance analyses are useful in detecting clinically active disease in patients with dermatomyositis

Dermatomiositis en el Hospital Militar "Dr. Carlos Arvelo" / Dermatomyositis in the Military Hospital \"Dr. Carlos Arvelo\"

Se revisaron en forma retrospectiva todos los casos con el diagnóstico de dermatomiositis ingresados en el hospital "Dr. Carlos Arvelo". De un total de 12 pacientes, el sexo más afectado fue el femenino con relación 2:1, la edad promedio para el diagnóstico fue de 39,33 años, la clínica más frecuente consistió en artritis o artralgias en el 50 por ciento, debilidad muscular 83,33 por ciento, lesiones eritematosas 75 por ciento, disfagia 50 por ciento. Los hallazgos paraclínicos más comúnmente alterados fueron la electromiografía y las enzimas musculares en el 100 por ciento de los casos, con biopsia de músculo compatible en el 70 por ciento. Las patologías asociadas más frecuentes fueron gastritis 33,3 por ciento, síndrome de Sjögren 25 por ciento, esclerodermia y tiroiditis crónica 16,66 por ciento cada uno. No se observó patología neoplásica asociada. El tratamiento más usado, fue el esteroideo