Full Papers
Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibodies: incidence using different testing criteria, and case series
V.M. Joseph1, E.C. Rose2, C.J. Edwards3, M. Lloyd4, S.K. Amar5, R.D. Wheeler6, T. Forster7, K.A. Smith8, E. Eren9, R. Hughes10, V. Hajela11, P.D. Kiely12
- Department of Rheumatology, St George’s University Hospitals NHS Foundation Trust, London, and Institute of Medical and Biomedical Education, St George’s, University of London, UK.
- Department of Rheumatology, University Hospital Southampton NHS Foundation Trust, Southampton, UK.
- NIHR Southampton Clinical Research Facility, University Hospital Southampton NHS Foundation Trust, Southampton, UK.
- Department of Rheumatology, Frimley Healthcare NHS Foundation Trust, Frimley, UK.
- Department of Immunology, Frimley Healthcare NHS Foundation Trust, Frimley, UK.
- Protein Reference Unit, South West London Pathology, UK.
- Immunology and Virology, Berkshire and Surrey Pathology Services, UK.
- Clinical Immunology, Department of Laboratory Sciences, University Hospital Southampton NHS Trust, Southampton, UK.
- Clinical Immunology, Department of Laboratory Sciences, University Hospital Southampton NHS Trust, Southampton, UK.
- Department of Rheumatology, Ashford and St Peter’s Hospitals NHS Foundation Trust, UK.
- Department of Rheumatology, University Hospitals Sussex NHS Foundation Trust, UK.
- Department of Rheumatology, St George’s University Hospitals NHS Foundation Trust, London, and Institute of Medical and Biomedical Education, St George’s, University of London, UK. patrick.kiely@nhs.net
CER15068
2022 Vol.40, N°2
PI 0298, PF 0303
Full Papers
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PMID: 35200125 [PubMed]
Received: 11/08/2021
Accepted : 15/11/2021
In Press: 28/01/2022
Published: 25/02/2022
Abstract
OBJECTIVES:
To estimate the incidence of anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies utilising different testing criteria, and review the clinical details of a series of patients with associated autoimmune myopathy.
METHODS:
The incidence of anti-HMGCR antibodies in 2019 from 3 groups, South West London, Berkshire/Surrey and Southampton, were compared in the adult population. Anti-HMGCR antibodies were measured by commercial chemiluminescent and immunodot assays. The case notes of patients with anti-HMGCR antibodies were reviewed for the case series.
RESULTS:
The estimated incidence of anti-HMGCR antibodies in the first 2 groups was 1.94 per million adults per year, and in the third group 10.3 per million adults per year. In the first 2 groups the test criteria restricted analysis to specific clinician request for anti-HMGCR. In the third group test criteria included cases with less specific clinical features or a cytoplasmic indirect immunofluorescence anti-nuclear antibody pattern. The latter strategy had a positive predictive value of 66.1% for anti-HMGCR associated myopathy. A case series of 27 patients with anti-HMGCR antibodies revealed 19 with myopathy, oesophageal involvement in 26% and median peak CK 8000 IU/L. Response to treatment, including intravenous immunoglobulin, was good with CK normalising after median 5.5 months. In 8 cases there was no evidence of autoimmune muscle disease, 7 not statin exposed.
CONCLUSIONS:
Varying criteria result in a 5-fold difference in estimated incidence of anti-HMGCR antibodies, revealing positive cases without evidence of myopathy. Patients with anti-HMGCR myopathy respond well to immune suppression, supporting wider testing for these antibodies amongst patients with myopathy.
