RESUMO

Las Miopatías Inflamatorias Autoinmunes (MI) comprenden un grupo de enfermedades heterogéneas con presentación y características clínicas variables. Se distinguen subtipos clínicos como Polimiositis (PM), Dermatomiositis (DM), Miositis por cuerpos de Inclusión (MCI), Miopatía Necrotizante Inmunomediada (MNIM), Miositis de los Síndromes de Superposición, formas juveniles de MI (DMJ), Síndrome Antisintetasa (SAS) y Miopatía Asociada a Cáncer (MAC). La presencia de anticuerpos séricos y el infiltrado inflamatorio en la biopsia de músculo sugiere que se trata de una condición autoinmune. Realizar el diagnóstico de las MI suele ser un desafío y las herramientas diagnósticas no siempre están disponibles en la práctica diaria. Se obtuvo información sobre la disponibilidad de estos métodos del Registro Argentino de Miopatías Inflamatorias. El estudio de enzimas musculares, Anticuerpos Antinucleares (ANA), anticuerpo anti-Jo-1 y la tomografía computada de tórax, estuvieron disponibles para la mayoría de los pacientes mientras que la Resonancia Magnética de musculo (RM), el estudio de difusión de monóxido de carbono (DLco) y la biopsia muscular se realizaron en menos del 50% de los casos. La determinación de otros anticuerpos específicos de miositis, de importancia en el diagnóstico y pronóstico de la enfermedad se realizó, en mayor parte, a través de un subsidio de la SAR.

The Idiopathic Inflammatory Myopathies (IIM) comprise a heterogeneous group of acquired muscle diseases classified as polymyositis (PM), dermatomyositis (DM), Inclusion Body Myositis (IBM), Immuno Mediated Necrotizing Myopathies (IMNM), Overlap Myositis (OM), juvenile myositis, Antisynthethase Syndrome (ASS) and cancer related myositis (CAM). The presence of myositis specific antibodies in the serum and autoantibodies against target antigens and inflammatory infiltrates in muscle tissue suggests the autoimmune condition of the disease. The diagnosis of inflammatory myopathies is often a challenge and the disposal of diagnostic tools are not always available in daily practice. Information on the accessibility of these methods was obtained from the Argentine Register of Myopathies. The study of muscle enzymes, ANA, anti-Jo-1 antibodies and chest tomography were easy to get to most patients while muscle MRI, lung diffusion capacity for carbon monoxide (DLco) and muscle biopsy were performed in less than 50% of cases. Other myositis specific antibodies, necessary for disease diagnosis and prognosis, were mostly done through a subsidy from the Argentine Rheumatology Society.

Assuntos

RESUMO

O Instituto Lauro de Souza Lima (ILSL) é um centro de referência dermatológica e a dermatomiosite é uma doença frequente em seu ambulatório. A dermatomiosite é identificada por suas lesões de pele específicas e pela presença de miopatia inflamatória. Sua incidência é baixa. A confirmação diagnóstica é realizada através da análise da apresentação dermatológica, dosagem sérica de enzimas musculares, eletroneuromiografia e biópsias de pele e músculo. Objetivo: analisar a incidência, características clínicas e procedimentos diagnósticos realizados bem como o tempo para o diagnóstico, levando em conta a sua efetividade. Método: estudo retrospectivo dos prontuários de pacientes diagnosticados com dermatomiosite de janeiro de 2000 a abril de 2013 no ILSL. Conclusão: aconselha-se que se dê prioridade ao atendimento de pacientes com os sintomas neurológicos mais sugestivos, tais como: fraqueza muscular e disfagia associadas a lesões de pele, tendo em vista a possibilidade de dermatomiosite.

As the Instituto Lauro de Souza Lima is a Dermatology Reference Center, dermatomyositis is prevalent in its outpatient clinic. Dermatomyositis is identified by its specific skin lesions and inflammatory myopathy and has low incidence. Diagnostic confirmation is made by the dermatological presentation, serum muscle enzymes, electroneuromyography and skin and muscle biopsies. Objective: to analyze the incidence, clinical features, diagnostic procedures and time to diagnosis of dermatomyositis patients, according to the algorithm rationality and effectiveness Methods: it is a retrospective surveillance of Instituto Lauro de Souza Lima (ILSL) patients from January 2000 until April 2013. Conclusion: in a dermatology outpatient department it is advisable to prioritize dermatological cases with neurological symptoms suggestive of dysphagia and muscular weakness with skin lesions, referring for neurological examination due to the possibility of derrmatomyositis.

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RESUMO

Se presentan los casos de 3 pacientes atendidos en el Hospital Pediátrico Juan Manuel Márquez, 2 de las cuales presentaron como primeras manifestaciones debilidad en los miembros inferiores y artritis que dificultaba la marcha de forma progresiva. El tercer paciente presentaba lesiones escamosas en la piel, hipopigmentadas en la cara y los miembros, acompañadas de astenia y artritis de las rodillas. Se realizaron estudios para concluir el diagnóstico, incluida la biopsia de músculo. Se aplicaron los criterios para confirmar el diagnóstico de dermatomiositis y se excluyeron otras enfermedades según el cuadro clínico y los exámenes complementarios realizados. A los 2 primeros pacientes se les indicó tratamiento inmunomodulador con prednisona y metotrexato, y al tercer paciente se le indicó prednisona y azathioprina. Se observó una buena respuesta al tratamiento y control de la enfermedad. El primer paciente falleció a los 4 meses de tratamiento a causa de complicaciones (vasculitis intestinal y sepsis asociada al tratamiento quirúrgico). Los restantes pacientes se siguen actualmente por consulta

Authors present three patients seen in the Juan Manuel Márquez Children Hospital where two of them had as first manifestations weakness in lower extremities and arthritis provoking a progressive difficulty to walk. The third patient had skin squamous lesions with hypopigmentation in the case and limbs as well as asthenia and knee arthritis. To made the diagnosis studies were conducted including thigh biopsy Criteria were applied to confirm diagnosis of dermatomyositis with exclusion of other diseases according to clinical picture and complementary examinations performed. In the two first patients a imunomodulation treatment was prescribed including Prednisone and Methotrexate, and the other patient received Prednisone and Azathioprine. There was a good response to treatment and a disease control. The first patient dye at 4 months of treatment due to complications (intestinal vasculitis and surgical treatment associated-sepsis). Remainder patients are followed up in consultation