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Maintenance treatment with subcutaneous immunoglobulins in the long-term management of anti-HMCGR myopathy
- Publication Year :
- 2021
- Publisher :
- Elsevier Ltd, 2021.
-
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.
- Subjects :
- 0301 basic medicine
medicine.medical_specialty
Necrotizing myopathy
Intravenous immunoglobulins
Gastroenterology
03 medical and health sciences
0302 clinical medicine
Internal medicine
Long term management
Medicine
Myopathy
Genetics (clinical)
biology
Anti-HMGCR myopathy
business.industry
Long-term treatment
Clinical course
030104 developmental biology
Subcutaneous immunoglobulins
Neurology
Intravenous Immunoglobulins
Pediatrics, Perinatology and Child Health
biology.protein
Creatine kinase
Neurology (clinical)
Antibody
medicine.symptom
business
Previously treated
030217 neurology & neurosurgery
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....8f083a23a243a64d16b471e0a71b6fc7