What is the efficacy of medial treatment of sporadic inclusion body myositis (s-IBM)?

Updated: Jun 08, 2018
  • Author: Michael P Collins, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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A long-term observational study of a large cohort of patients in Paris and Oxford found that immunosuppressive treatments do not ameliorate sporadic inclusion body myositis and could modestly exacerbate the progression of disability. However, the results from this study are limited due to the semi-retrospective nature of data collection and possible bias in patients selected for immunosuppressive treatment. [97]

An open-label study of high-dose prednisone in 8 patients showed no improvement in strength or functional disability scores despite a decrease in creatine phosphokinase (CPK) and inflammatory cell infiltration. Posttreatment muscle biopsy samples showed increased vacuole formation and amyloid deposition, suggesting that mechanisms other than the inflammatory response play a role in disease propagation. [98]

A randomized, controlled study of oxandrolone in 19 patients reported a regional improvement in upper extremity strength, but only borderline improvement in whole-body strength. [99]

A randomized, controlled study of methotrexate in 44 patients likewise showed no improvement in strength despite a significant decrease in CPK levels. [100]

An early small, uncontrolled study reported improvement in strength in 4 patients following intravenous immunoglobulin (IVIg) treatment. [101] However, subsequent larger and placebo-controlled studies have failed to duplicate these results. [102, 23, 103] Two studies suggest some benefit in patients with severe dysphagia. [104, 105] A subsequent controlled study of IVIg in combination with prednisone likewise showed no treatment response despite a reduction in endomysial inflammation. [106]

An open-label, randomized study of anti-T-lymphocyte globulin treatment followed by 12 months of oral methotrexate (vs methotrexate alone) reported regional improvement in distal upper extremity strength, but continued deterioration of the proximal muscle groups. [107]

Beta interferon-1a at standard (30 µg IM/wk) and high-dosage (60 µg IM/wk) regimens were found to be well tolerated but produced no significant improvement in muscle strength or muscle mass. [108, 109]

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