A German team publishes the results of the long-term follow-up of two children with autoimmune necrotizing myopathy, and highlights:
- the difficulties of this diagnosis in childhood due to its great rarity, an unspecific clinical picture (in the two children an impairment of the girdles with a sharp increase in CKemia and abnormal distribution of α-dystroglycan in muscle cells), absence of major inflammation on muscle biopsy and the fact that anti-HMGCR autoantibodies, specific for autoimmune necrotizing myopathy with anti-SRP, are not always detectable routinely;
- the complexity of the treatment, as the biological and MRI signs of active myositis are not always simultaneous from clinical escape to treatment; in the two published cases, permanent reduction in strength and muscle wasting occurred despite corticosteroid therapy combined with methotrexate, followed by the combination of rituximab – methotrexate – intravenous immunoglobulins.
