High-dose cyclophosphamide without stem cell rescue in immune-mediated necrotizing myopathies.

ABSTRACT:

Objective

To describe the experience managing treatment-refractory immune-mediated necrotizing myopathies (IMNM) with high-dose cyclophosphamide (HiCy) therapy.

Methods

Five patients with severe refractory IMNM who were treated with HiCy without stem cell rescue were identified. Their medical records were reviewed to assess demographic, clinical, and histologic characteristics as well as response to therapy.

Results

Three patients with anti-signal recognition particle (SRP) and 2 patients with anti-HMG-CoA reductase autoantibodies were included. The mean follow-up time after HiCy therapy was 37 ± 28 months. Two patients demonstrated substantial response, evidenced by improved muscle strength and decreased muscle enzymes after HiCy therapy; both of these patients were

SUBMITTER: Mecoli CA 

PROVIDER: S-EPMC5619913 | biostudies-literature | 2017 Sep

REPOSITORIES: biostudies-literature