RESUMO
Sporadic inclusion-body myositis (sIBM) is the most frequent myopathy after 50 years of age. As the clinical presentation may often be typical, pathological confirmation by muscle biopsy appears necessary, but sometimes difficult. Further delineation of the framework of this particular disease, especially during its early-onset stage, appears to be challenging. New classification of diagnostic criteria as well as the identification of new diagnostic hallmarks appear to be the two main tools towards to achieve this purpose. sIBM pathophysiology has long been discussed and remains yet controversial. Since its initial description, there have been two major pathogenic hypotheses: inflammatory and degenerative. To date, the debate is still ongoing, as recent works support both pathophysiological mechanisms, although the inflammatory process seems to be slightly more preeminent in the recent literature. Treatment remains the most disappointing aspect of the disease as, despite various therapeutic attempts, no significant efficacy has been reported thus far. Nevertheless, advances in our pathophysiological understanding of the disease are paving the way for further therapeutic perspectives that might arise in the years to come. The objective of the present work was to summarize the most significant data published on sIBM during the past 2 years.
Assuntos
Miosite de Corpos de Inclusão/terapia , Biópsia , Humanos , Miosite de Corpos de Inclusão/diagnóstico , Miosite de Corpos de Inclusão/genética , Miosite de Corpos de Inclusão/fisiopatologiaRESUMO
Focal myositis are inflammatory muscle diseases of unknown origin. At the opposite from the other idiopathic inflammatory myopathies, they are restricted to a single muscle or to a muscle group. They are not associated with extramuscular manifestations, and they have a good prognosis without any treatment. They are characterized by a localized swelling affecting mostly lower limbs. The pseudo-tumor can be painful, but is not associated with a muscle weakness. Creatine kinase level is normal. Muscle MRI shows an inflammation restricted to a muscle or a muscle group. Muscle biopsy and pathological analysis remain necessary for the diagnosis, showing inflammatory infiltrates composed by macrophages and lymphocytes without any specific distribution within the muscle. Focal overexpression of HLA-1 by the muscle fibers is frequently observed. The muscle biopsy permits to rule out differential diagnosis such a malignancy (sarcoma). Spontaneous remission occurs within weeks or months after the first symptoms, relapse is unusual.
Assuntos
Miosite/diagnóstico , Miosite/terapia , Biópsia , Diagnóstico Diferencial , Eletromiografia , Humanos , Imageamento por Ressonância Magnética , Debilidade Muscular/diagnóstico , Debilidade Muscular/patologia , Músculo Esquelético/diagnóstico por imagem , Músculo Esquelético/patologia , Miosite/etiologia , Miosite/patologiaRESUMO
Amongst the heterogeneous group of inflammatory myopathies, focal myositis stands as a rare and benign dysimmune disease. Although it can be associated with root and/or nerve lesions, traumatic muscle lesions and autoimmune diseases, its triggering factors remain poorly understood. Defined as an isolated inflammatory pseudotumour usually restricted to one skeletal muscle, clinical presentation of focal myositis is that of a rapidly growing solitary mass within a single muscle, usually in the lower limbs. Electromyography shows spontaneous activity associated with a myopathic pattern. MRI reveals a contrast enhanced enlarged muscle appearing hyper-intense on FAT-SAT T2 weighted images. Adjacent structures are spared and there are no calcifications. Serum creatine kinase (CK) levels are usually moderately augmented and biological markers of systemic inflammation are absent in most cases. Pathological histological features include marked variation in fibre size, inflammatory infiltrates mostly composed of T CD4+ lymphocytes and macrophages, degenerating/regenerating fibres and interstitial fibrosis. Differential diagnoses are numerous and include myositis of other origin with focal onset. Steroid treatment should be reserved for patients who present with major pain, nerve lesions, associated autoimmune disease, or elevated C reactive protein or CK.
Assuntos
Miosite , Humanos , Miosite/diagnóstico , Miosite/patologia , Miosite/fisiopatologia , Miosite/terapiaRESUMO
This study attempts to quantify an overall association between CME course attendance and referrals. Attendance at formal CME courses given by the University of Michigan Medical School and referrals to the University Hospitals were examined over a two-year period. Attendance and referrals were linked to physicians in Michigan identified through the Michigan Department of Licensing and Regulation and through the American Medical Association. For physicians who are office-based and likely to be in active practice (age less than 70), those who attended at least one of the University's CME courses referred more patients than those who did not attend one (means of 1.9 referrals per physician and 1.3 referrals per physician, p less than .001). The causal direction of the relationship is not clear, but probably operates in both directions. It is reasonable for medical center marketers to consider CME as an indirect method for marketing clinical services. It is also reasonable for CME directors to identify referring physicians as high-priority groups for marketing CME. Both marketing efforts may be significantly enhanced by linking data bases for referrals and for CME attendance. CME directors must also ensure that marketing efforts do not compromise the objectivity and integrity of the content of the institution's CME program.