A 67-year-old African-American woman with HIV and Sjogren’s syndrome was diagnosed with HIV-associated inclusion body myositis with proximal weakness. She had intermediate findings between PM and IBM, including MRI with increased T2 signal and enhancement in proximal leg muscles, and biopsy showing interstitial inflammation, MHC1 upregulation, and rare rimmed sarcoplasmic vacuoles. Initial serum creatine kinase (CK) of 3,407 IU/L downtrended to 265 on high dose prednisone. When steroids were weaned off, she experienced a clinical relapse and increase in CK to 3,705 U/L. When started on IV immunoglobulins and pulse dose IV steroids, her CK decreased to 1,847 U/L with some improvement in strength.
A 37-year-old African-American man with HIV presented with two years of progressive gait dysfunction. Initial examination found weakness particularly in finger and wrist flexors with proximal leg weakness resulting in being wheelchair bound. Serum CK was 5,255 U/L. Muscle biopsy had intermediate findings between PM and IBM, with multifocal endomysial inflammation with CD8+ T cells invading into viable myofibers, marked fiber size variation, rare rimmed vacuoles and mildly increased number of COX deficient fibers. With scheduled pulse IV steroids, hand strength improved and he could stand.