Sporadic inclusion-body myositis: Recent advances and the state of the art in 2016.
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.
Croix-Rousse University Hospital, Department of neurology, 103, grande rue de la Croix-Rousse, 69004 Lyon, France. Electronic address: laure.gallay@hotmail.fr.
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Gallay
Croix-Rousse University Hospital, Department of neurology, 103, grande rue de la Croix-Rousse, 69004 Lyon, France.
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Petiot
D001706:Biopsy; D006801:Humans; D018979:Myositis, Inclusion Body / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*
Classification; Inclusion-body myositis; Pathogenesis; Review; Therapeutic management