gms | German Medical Science

Background: Necrotizing myopathy (NAM) is a rare autoimmune disease, characterized by subacute proximal limb muscle weakness and a high creatinine-kinase level. It can be associated with use of statins or associated with cancer [1], [2], [3]. Patients have no or minimal inflammation on muscle biopsy. The exact mechanism is not known, but many patients with NAM have autoantibodies directed against HMGCR or SRP [1]. HMGCR is the enzyme targeted by statins, and it has been suggested that statins may trigger NAM, althought many patients with NAM have never taken statin medications [1]. The presence of the antibodies and the response to immunotherapy support the autoimmune nature of NAM. Proximal muscle weakness in the limbs, as well as weakness in other muscles, including breathing and swallowing muscles, can be present [2]. Heart muscle involvement is rare.

Aim: The aim is to present clinical course and treatment of three women with NAM.

Method: Functional outcome of rehabilitation of three patients with necrotizing myopathy is presented.

Results/findings: They had weak respiratory muscles, muscles in upper and lower limbs, could not stand up or walk. NAM was confirmed after muscle biopsy. Aggressive early treatment with a combination of intravenous immunosuppressive therapy was indicated, including methylprednisolone, cyclosporine, methotrexate and intravenous immunoglobulin. Muscle strength and balance improved after rehabilitation and patients could walk alone.

Discussion and conclusions: In patients with NAM, regular visits at rheumatologist, proper treatment and complex rehabilitation are very important, including physical therapy, occupational, vocational, social and emotional support.