Maintenance treatment with subcutaneous immunoglobulins in the long-term management of anti-HMCGR myopathy

Neuromuscul Disord. 2021 Feb;31(2):134-138. doi: 10.1016/j.nmd.2020.12.012. Epub 2020 Dec 28.

Abstract

We describe the clinical response to long-term subcutaneous immunoglobulins (SCIg) in anti-3‑hydroxy-3-methyl-glutaryl-coenzyme-A-reductase (anti-HMCGR) myopathy previously treated with intravenous immunoglobulins (IVIg). We collected data from patients affected by anti-HMGCR myopathy, switched from IVIg to SCIg therapy, after achieving clinical stabilization. The Medical Research Council sum score, creatine kinase (CK) levels, and anti-HMGCR antibodies were used to assess the response. We identified three patients with anti-HMGCR myopathy treated with SCIg with a favourable clinical course, allowing the maintenance of clinical stability, the reduction or suspension of steroids therapy and in two of them a complete CK normalization. Finally, anti-HMGCR antibodies tested in all patients after 12 months from SCIg starting, showed a global decrease. SCIg represent an useful alternative to long-term IVIg as already well known in several autoimmune neuromuscular disorders and inflammatory myopathies with advantages of lower side effects and home self-administration.

Keywords: Anti-HMGCR myopathy; Intravenous immunoglobulins; Long-term treatment; Necrotizing myopathy; Subcutaneous immunoglobulins.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Autoimmune Diseases / drug therapy*
  • Female
  • Humans
  • Hydroxymethylglutaryl CoA Reductases
  • Immunoglobulins, Intravenous / therapeutic use*
  • Male
  • Middle Aged
  • Myositis / drug therapy*
  • Retrospective Studies

Substances

  • Immunoglobulins, Intravenous
  • HMGCR protein, human
  • Hydroxymethylglutaryl CoA Reductases