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

Angela Zuppa, Chiara De Michelis, Giuseppe Meo, Valeria Prada, Chiara Gemelli, Maria Infantino, Mariangela Manfredi, Giampaola Pesce, Alberto S. Tagliafico, Luana Benedetti, Chiara Fiorillo, Angelo Schenone, Luca Quartuccio, Marina Grandis

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
JournalNeuromuscular Disorders
DOIs
Publication statusAccepted/In press - 2021

Keywords

  • Anti-HMGCR myopathy
  • Intravenous immunoglobulins
  • Long-term treatment
  • Necrotizing myopathy
  • Subcutaneous immunoglobulins

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Clinical Neurology
  • Genetics(clinical)

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