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      MIOPATÍA POR CUERPOS DE INCLUSIÓN: CASO CLÍNICO Y REVISIÓN DE LA LITERATURA Translated title: INCLUSION BODY MYOPATHY: CASE REPORT AND LITERATURE REVIEW Translated title: MIOPATIA POR CORPOS DE INCLUSÃO: RELATO DE CASO E REVISÃO DA LITERATURA

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          Abstract

          La miositis por cuerpos de inclusión (MCI) es una patología poco común que se presenta en individuos mayores de 50 años y con mayor frecuencia en hombres; sin embargo, los síntomas pueden empezar 20 años antes de su diagnóstico. Hasta un 30% de los casos de miopatías inflamatorias pueden ser de MCI y su prevalencia varía en los diferentes países y grupos étnicos. Clínicamente se manifiesta como debilidad lentamente progresiva proximal y distal. En este artículo se presenta un caso de una paciente de 78 años con antecedentes previos de diabetes mellitus tipo 2, hipertensión arterial, e hipotiroidismo, con cuadro de 18 meses de evolución consistente en debilidad de miembros inferiores, incapacidad para subir y bajar escaleras, limitación en la marcha, acompañado de disestesias. Se realizó la medición de la creatin quinasa (CK) y su valor inicial fue 7820, la electromiografía y neuroconducciones mostraron polineuropatia axonal motora en las cuatro extremidades y miopatía inflamatoria. Se trató con glucorticoides a dosis de 1 mg/kg y después de seis meses de tratamiento no se observó mejoría. Se decide tomar biopsia muscular que mostró miopatía por cuerpos de inclusión, lo que explicó la falla terapéutica inicial. Este caso es interesante desde el punto de vista clínico, ya que no es frecuente la elevación de la CK en los niveles que presentó la paciente y por las múltiples comorbilidades asociadas.

          Translated abstract

          Inclusion body myositis (IBM) is an infrequent disease in people over 50 years of age, affecting more frequently males; however the symptoms may begin 20 years before its diagnosis. Up to 30% of all inflammatory myopathies can be IBM and its prevalence varies among different countries and ethnical groups. IBM manifest clinically by slowly progressive proximal and distal weakness. In this article a report of case is presented: A female patient 78 years old with previous history of type 2 diabetes mellitus, hypertension and hypothyroidism; with 18 months history of weakness in lower limbs, inability to climb stairs, limited to walk, associated with dysesthesias. The initial CK was 7820 mg/dl, the electromyography and nerve conductions reported a motor axonal polyneuropathy of four limbs and inflammatory myopathy. She was treated with steroids at doses of 1mg/kg without improvement, after 6 months of treatment. It was decided to take a muscle biopsy that showed an inclusion body myopathy, explaining the initial treatment failure. This case is interesting from a clinical point of view, since it is not frequent elevated CK levels as it was presented by the patient and for the numerous associated co-morbidities.

          Translated abstract

          A miosite por corpo de inclusão (MCI) é uma doença rara que apresenta-se em indivíduos acima de 50 anos, atingindo 30% das miopatias inflamatória; orém os sintomas podem começar 20 anos antes. Sua prevalência varia entre países e grupos étnicos. Clínicamente manifesta-se como uma fraqueza lentamente progressiva proximal e distal mais frecuentemente nos homens. Nós relatamos um caso de uma paciente de 78 anos com antecedentes de diabetes mellitus tipo 2, hipertensão e hipotireoidismo. Com quadro clínico de 18 meses de evolução consistindo em fraqueza nos membros inferiores, incapacidade de subir escadas, a limitação para a marcha, acompanhados de disestesias. Foi feito a prova de CK dando um valor inicial de 7820 e com eletromiografia e neurocondução que informaram polineuropatia axonal motora de quatro membros e miopatia inflamatória. Foi tratado com corticóides em doses de 1 mg / kg sem melhora após 6 meses de tratamento, razão pela qual foi decidido fazer uma biópsia do músculo que mostrou miopatía do corpo de inclusão, explicando a falha do tratamento inicial. Este caso é interessante do ponto de vista clínico, uma vez que não é frequente a elevação da CK nos niveis apresentados pela paciente e pelas múltiplas comorbidades associadas.

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          Sporadic inclusion body myositis--diagnosis, pathogenesis and therapeutic strategies.

          Sporadic inclusion body myositis (sIBM) presents with a characteristic clinical phenotype of slow-onset weakness and atrophy, affecting proximal and distal limb muscles and facial and pharyngeal muscles. Histologically, sIBM is characterized by chronic myopathic features, lymphocytic infiltrates invading non-vacuolated fibers, vacuolar degeneration, and accumulation of amyloid-related proteins. The cause of sIBM is unclear, but two processes-one autoimmune and the other degenerative-appear to occur in parallel. In contrast to dystrophies, in sIBM the autoinvasive CD8(+) T cells are cytotoxic and antigen-driven, invading muscle fibers expressing major histocompatibility complex class I antigen and costimulatory molecules. The concurrent degenerative features include vacuolization, filamentous inclusions and intracellular accumulations of amyloid-beta-related molecules. Although viruses have not been amplified from the muscle fibers, at least 12 cases of sIBM have been seen in association with retroviral infections, indicating that a chronic persistent viral infection might be a potential triggering factor. Emerging data imply that continuous upregulation of cytokines and major histocompatibility complex class I on the muscle fibers causes an endoplasmic reticulum stress response, resulting in intracellular accumulation of misfolded glycoproteins and activation of the transcription factor NFkappaB, leading to further cytokine activation. In spite of the brisk, antigen-driven T-cell infiltrates, sIBM does not respond to immunotherapies. New therapies using monoclonal antibodies against lymphocyte signaling pathways might prove helpful in arresting disease progression.
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            Effect of Alemtuzumab (CAMPATH 1-H) in patients with inclusion-body myositis

            Sporadic inclusion-body myositis (sIBM) is the most common disabling, adult-onset, inflammatory myopathy histologically characterized by intense inflammation and vacuolar degeneration. In spite of T cell-mediated cytotoxicity and persistent, clonally expanded and antigen-driven endomysial T cells, the disease is resistant to immunotherapies. Alemtuzumab is a humanized monoclonal antibody that causes an immediate depletion or severe reduction of peripheral blood lymphocytes, lasting at least 6 months. We designed a proof-of-principle study to examine if one series of Alemtuzumab infusions in sIBM patients depletes not only peripheral blood lymphocytes but also endomysial T cells and alters the natural course of the disease. Thirteen sIBM patients with established 12-month natural history data received 0.3 mg/kg/day Alemtuzumab for 4 days. The study was powered to capture ≥10% increase strength 6 months after treatment. The primary end-point was disease stabilization compared to natural history, assessed by bi-monthly Quantitative Muscle Strength Testing and Medical Research Council strength measurements. Lymphocytes and T cell subsets were monitored concurrently in the blood and the repeated muscle biopsies. Alterations in the mRNA expression of inflammatory, stressor and degeneration-associated molecules were examined in the repeated biopsies. During a 12-month observation period, the patients’ total strength had declined by a mean of 14.9% based on Quantitative Muscle Strength Testing. Six months after therapy, the overall decline was only 1.9% (P < 0.002), corresponding to a 13% differential gain. Among those patients, four improved by a mean of 10% and six reported improved performance of daily activities. The benefit was more evident by the Medical Research Council scales, which demonstrated a decline in the total scores by 13.8% during the observation period but an improvement by 11.4% (P < 0.001) after 6 months, reaching the level of strength recorded 12 months earlier. Depletion of peripheral blood lymphocytes, including the naive and memory CD8+ cells, was noted 2 weeks after treatment and persisted up to 6 months. The effector CD45RA+CD62L­ cells, however, started to increase 2 months after therapy and peaked by the 4th month. Repeated muscle biopsies showed reduction of CD3 lymphocytes by a mean of 50% (P < 0.008), most prominent in the improved patients, and reduced mRNA expression of stressor molecules Fas, Mip-1a and αB-crystallin; the mRNA of desmin, a regeneration-associated molecule, increased. This proof-of-principle study provides insights into the pathogenesis of inclusion-body myositis and concludes that in sIBM one series of Alemtuzumab infusions can slow down disease progression up to 6 months, improve the strength of some patients, and reduce endomysial inflammation and stressor molecules. These encouraging results, the first in sIBM, warrant a future study with repeated infusions (Clinical Trials. Gov NCT00079768).
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              B cells as therapeutic targets in autoimmune neurological disorders.

              B cells have a fundamental role in the pathogenesis of various autoimmune neurological disorders, not only as precursors of antibody-producing cells, but also as important regulators of the T-cell activation process through their participation in antigen presentation, cytokine production, and formation of ectopic germinal centers in the intermeningeal spaces. Two B-cell trophic factors-BAFF (B-cell-activating factor) and APRIL (a proliferation-inducing ligand)-and their receptors are strongly upregulated in many immunological disorders of the CNS and PNS, and these molecules contribute to clonal expansion of B cells in situ. The availability of monoclonal antibodies or fusion proteins against B-cell surface molecules and trophic factors provides a rational approach to the treatment of autoimmune neurological diseases. This article reviews the role of B cells in autoimmune neurological disorders and summarizes the experience to date with rituximab, a B-cell-depleting monoclonal antibody against CD20, for the treatment of relapsing-remitting multiple sclerosis, autoimmune neuropathies, neuromyelitis optica, paraneoplastic neurological disorders, myasthenia gravis, and inflammatory myopathies. It is expected that ongoing controlled trials will establish the efficacy and long-term safety profile of anti-B-cell agents in several autoimmune neurological disorders, as well as exploring the possibility of a safe and synergistic effect with other immunosuppressants or immunomodulators.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                med
                Revista Med
                rev.fac.med
                Universidad Militar Nueva Granada. Facultad de Medicina (Bogotá )
                0121-5256
                June 2010
                : 18
                : 1
                : 93-99
                Affiliations
                [1 ] Universidad Militar Nueva Granada Colombia
                [2 ] Universidad de la Sabana Colombia
                [3 ] Hospital Militar "Carlos J. Finlay" Cuba
                Article
                S0121-52562010000100010
                a31aa301-61a5-4da7-ac1b-32b4277fac9c

                http://creativecommons.org/licenses/by/4.0/

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                Product Information: website
                Self URI (journal page): http://www.scielo.org.co/scielo.php?script=sci_serial&pid=0121-5256&lng=en
                Categories
                PUBLIC, ENVIRONMENTAL & OCCUPATIONAL HEALTH

                Public health
                myositis,myopathy,inclusion bodies,miosite,doenças musculares,corpos de inclusão,miositis,miopatía,cuerpos de inclusión

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