Inclusion body myositis: update.

[inclusion body myositis]

To examine new developments in sporadic inclusion body myositis (IBM ), including updated clinical and prognostic factors , novel autoantibody associations , unique histopathologic findings , proposed new clinical diagnostic criteria , and novel therapeutic agents .IBM is a slowly progressive disease , leading to wheelchair use , on average , 12 -20 years after onset of symptoms ; however , it does not appear to interfere with life expectancy . Older age at the onset of first symptoms as well as immunosuppressive therapy are likely associated with more rapid disease progression . Quantitative muscle strength of knee extensor and the IBM functional rating scale seem to be sensitive disease progression markers and may be useful clinical trial outcome measures . Newly proposed diagnostic criteria utilize data-driven approaches with very high sensitivity and specificity . A novel autoantibody , as well as unique proteins seen histopathlogically , may help hone in on diagnosis as well as to deepen our understanding of IBM pathophysiology . Novel treatments , including follistatin and bimagrumab , are directed at potential therapeutic targets .We have observed an explosion of knowledge in IBM in the recent past , which challenges traditional dogma and ushers in a new era of understanding with potential clinical implications for those who suffer with IBM .

Diseases
Validation

Diseases presenting "specificity" symptom

familial mediterranean fever

hodgkin lymphoma, classical

inclusion body myositis

pyruvate dehydrogenase deficiency

typhoid

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