Miopatía necrotizante autoinmune: Presentación de un caso clínico / Autoimmune necrotizing myositis: Presentation of a clinical case

Las miopatías inflamatorias (MI) son un grupo heterogéneo de enfermedades musculares de rara ocurrencia, caracterizadas por inflamación de los distintos componentes del tejido muscular, ya sea de forma aislada o, más comúnmente, en el contexto de una afección sistémica. Las miopatías necrotizantes inmunomediadas (MNIM) constituyen un subtipo de miopatía inflamatoria caracterizada por debilidad muscular proximal, necrosis de miofibrillas con mínimo infiltrado celular inflamatorio en la biopsia muscular e infrecuente compromiso extramuscular asociado1. Si bien existen similitudes clínicas e histopatológicas, el espectro de las miopatías inflamatorias es considerablemente variable. Por este motivo, es fundamental realizar estudios complementarios para la identificación correcta del subtipo de MI a fin de determinar su pronóstico e implementar un adecuado tratamiento. Se presenta el caso de una paciente de 29 años, sin antecedentes personales y heredofamiliares de enfermedad autoinmune ni antecedentes patológicos relevantes, que consulta a la Guardia Médica de nuestra Institución por un cuadro de dolor e impotencia funcional en los cuatro miembros, con debilidad muscular a predominio de cintura escapular y en menor medida pelviana, acompañado de astenia, tendencia al sueño e hiporreactividad.

Inflammatory myopathies (IM) or myositis are a heterogeneous group of muscle diseases of rare occurrence. Such diseases are characterized by inflammation of the different components of muscle tissue, which can occur either in isolation or, more commonly, as part of a systemic disorder. Immune-mediated necrotizing myopathies (IMNM) are a type of autoimmune myopathy characterized by proximal muscle weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy and infrequent extramuscular involvement1. Even though there are clinical and histopathological similarities. The spectrum of inflammatory myopathies is considerably variable. Therefore, the performance of complementary studies is essential for the proper identification of the IM subtype to contribute accurately on treatment so determine the better prognosis. The present article shows the case of a young 29 years old, with no personal and family history background of autoimmune disease and no relevant pathological background. The patient consulted the medical ward of the Institution with pain, functional impairment of upper and lower extremities, muscle weakness mainly located in the pectoral girdle area and, although to a lesser degree, in the pelvic girdle area. It was also associated with asthenia, tendency to drowsiness and hyporeactivity.

Motivos de consulta de pacientes con enfermedades autoinmunes sistémicas en el servicio de urgencias de un hospital terciario / Causes for consultation in the emergency room among patients with systemic autoimmune diseases

Background: The causes of acute decompensations of patients with systemic autoimmune diseases are not well known. Aim: To describe the causes for consultation in an emergency room of patients with systemic autoimmune diseases. Material and Methods: Review of medical records of patients with systemic autoimmune diseases, aged over 14 years, consulting in an emergency room of a general hospital during three months. Results: In the study period, 166 patients with systemic autoimmune diseases consulted in the emergency room, of a total of 18,153 consultations (0.9%). Patients with rheumatoid arthritis were those that consulted with higher frequency (37%) followed by patients with systemic lupus erythematosus (21%). The most common causes for consultation were cardiovascular diseases in 25%, followed by digestive disorders in 15%. The most common diagnosis was chest pain with suspected ischemic heart disease in 36%. No differences in cardiovascular risk factors were observed between those patients consulting for cardiovascular diseases and those consulting for other causes. Conclusions: The most common cause of consultation in the emergency room of patients with systemic autoimmune diseases is cardiovascular.

Validation of International Autoimmune Hepatitis Group Scoring System for Diagnosis of Type 1 Autoimmune Hepatitis in Korea / 대한간학회지

BACKGROUND/AIMS: There are no pathognomonic features of autoimmune hepatitis (AIH). Its diagnosis requires the exclusion of various other conditions. The aim of this study was to validate indirectly the International Autoimmune Hepatitis Group (IAHG) scoring system in diagnosing AIH. METHODS: Twenty-six patients with Type 1 AIH and female patients with chronic hepatitis B (n=34), chronic hepatitis C (n=25), or toxic hepatitis (n=13) were evaluated according to 9 categories of pretreatment minimum required parameters proposed by IAHG. Aggregate scores of AIH to those of non-AIH groups, which were assessed before and after extracting the proportions of etiologic factors, were also compared and evaluated. RESULTS: While aggregate scores of non-AIH groups, before extracting the proportions of etiologic factors, were 5.2+/-1.8, 5.6+/-1.1, and 7.4+/-1.2 in that order, those of AIH groups were 12.8+/-1.7. These were significantly higher than those of non-AIH groups (p<0.01). All patients in AIH groups and only 1 patient in a non-AIH group showed aggregate scores of more than 10. Aggregate scores after extracting the proportions of etiologic factors were more than 4 in all, except 2, patients. These should have been consistent with 10 if there were no etiologic factors in non-AIH groups. CONCLUSION: The IAHG scoring system might have a relatively excessive importance to the scores of categories excluding distinct etiologies from AIH. It might be difficult to differentiate AIH from chronic liver diseases of indistinct cause based on the IAHG scoring system.

Hepatite auto-imune / Autoimmune hepatitis

Os autores fazem revisäo da literatura médica a respeito da hepatite auto-imune, enfatizando os aspectos laboratoriais, clínicos e terapêuticos desta entidade. Discutem a importância dos auto-anticorpos, mostrando a proliferaçäo de novos marcadores imunológicos e detalhando a sua utilizaçäo no diagnóstico e terapêutica. Por fim, ressaltam a importância do diagnóstico acertado e precoce, já que se trata de uma doença em que a terapia imunossupressiva prolonga muito a sobrevida.

Asociación de enfermedades del tejido conectivo con implantes mamarios de silicona. Perspectiva actual

This review describes one of the most interesting and current controversies in rheumatology : the relationship between silicone implants and diseases of the connective tissue. This association has been consedered since 1964 and more recently scleroderma and systemic lupus have been associated to breast implants. Lately it has been demostrated that silicone is not an inert material, but exert active immunological reactions. Moreover recents reports in the literature describe multiple autoantibodies in such patients (namely anti-nucleolar and anti-centromere) as well as atypical rheumatoid symptoms. Prospective controlled studies investigating such atypical complaints in recipients of silicone implants need to be ascertained.