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[Anti-HMG-CoA reductase necrotizing myopathy: A report of 4 cases].
Rev Med Interne. 2017 Apr; 38(4):228-234.RM

Abstract

INTRODUCTION

Anti-3-hydroxy-3-méthylglutaryl-coenzyme A reductase antibody-associated necrotizing autoimmune myopathy has been recently described (2011). This myopathy is distinct from statin toxic myopathy. Our objective is to report on the clinical and para-clinical characteristics of this myopathy and to show the difficulties of therapeutic care.

CASE REPORTS

We describe 4 cases of patients followed-up in Brittany, France. All data have been analyzed retrospectively. The mean age of our patients was 59.5 years, with a sex ratio of 1. The clinical presentation was homogeneous, with a subacute painful proximal and symmetrical weakness, without extra-muscular involvement. Other presentations have been described (including pseudo-dystrophic presentation). All patients had a previous statin medication (mean duration of 3.75 years) although this criteria is not a requisite. All patients had high levels of creatine kinase and abnormal electromyographic examination. The pathological pattern on muscular biopsy was a necrotizing myopathy without significant inflammatory cells infiltration. Cardio-respiratory function was normal and no associated neoplasia was found. Over the follow-up, we observed a marked corticosteroid-dependence, not improved by immunosuppressive drugs (azathioprine and methotrexate). The benefit of intravenous immunoglobulin was clear with, sometimes, prolonged responses.

CONCLUSION

An early diagnosis of this myopathy is necessary in order to introduce an immunotherapy associated with a close monitoring. The therapeutic strategy (within which the stead of intravenous immunoglobulin seems increased) remains to be defined and long-term prospective studies are thus needed.

Authors+Show Affiliations

Service de neurologie, centre hospitalier Yves-le-Foll, 10, rue Marcel-Proust, 22027 Saint-Brieuc cedex 1, France. Electronic address: martijulien@yahoo.fr.Service de neurologie, centre hospitalier Yves-le-Foll, 10, rue Marcel-Proust, 22027 Saint-Brieuc cedex 1, France.Service de médecine interne, hôpital du Scorff, centre hospitalier de Bretagne Sud, 5, avenue du Choiseul, 56100 Lorient, France.Service de médecine interne, hôpital privé Sévigné, 3, rue du Chêne-Germain, 35576 Cesson-Sévigné cedex, France.Service de neurologie, centre hospitalier Yves-le-Foll, 10, rue Marcel-Proust, 22027 Saint-Brieuc cedex 1, France.Service d'anatomie et cytologie pathologiques, hôpital Morvan, centre hospitalier régional et universitaire de Brest, 2, avenue Foch, 29609 Brest cedex, France.

Pub Type(s)

Case Reports
Journal Article

Language

fre

PubMed ID

27876175

Citation

Marti, J, et al. "[Anti-HMG-CoA Reductase Necrotizing Myopathy: a Report of 4 Cases]." La Revue De Medecine Interne, vol. 38, no. 4, 2017, pp. 228-234.
Marti J, Kolev I, Lorleac'h A, et al. [Anti-HMG-CoA reductase necrotizing myopathy: A report of 4 cases]. Rev Med Interne. 2017;38(4):228-234.
Marti, J., Kolev, I., Lorleac'h, A., Besnard, S., Pihan, M., & Marcorelles, P. (2017). [Anti-HMG-CoA reductase necrotizing myopathy: A report of 4 cases]. La Revue De Medecine Interne, 38(4), 228-234. https://doi.org/10.1016/j.revmed.2016.10.384
Marti J, et al. [Anti-HMG-CoA Reductase Necrotizing Myopathy: a Report of 4 Cases]. Rev Med Interne. 2017;38(4):228-234. PubMed PMID: 27876175.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Anti-HMG-CoA reductase necrotizing myopathy: A report of 4 cases]. AU - Marti,J, AU - Kolev,I, AU - Lorleac'h,A, AU - Besnard,S, AU - Pihan,M, AU - Marcorelles,P, Y1 - 2016/11/18/ PY - 2016/02/23/received PY - 2016/07/08/revised PY - 2016/10/19/accepted PY - 2016/11/24/pubmed PY - 2017/9/29/medline PY - 2016/11/24/entrez KW - Anti-HMGCR KW - Immune-mediated necrotizing myopathy KW - Myopathie nécrosante auto-immune KW - Statines KW - Statins SP - 228 EP - 234 JF - La Revue de medecine interne JO - Rev Med Interne VL - 38 IS - 4 N2 - INTRODUCTION: Anti-3-hydroxy-3-méthylglutaryl-coenzyme A reductase antibody-associated necrotizing autoimmune myopathy has been recently described (2011). This myopathy is distinct from statin toxic myopathy. Our objective is to report on the clinical and para-clinical characteristics of this myopathy and to show the difficulties of therapeutic care. CASE REPORTS: We describe 4 cases of patients followed-up in Brittany, France. All data have been analyzed retrospectively. The mean age of our patients was 59.5 years, with a sex ratio of 1. The clinical presentation was homogeneous, with a subacute painful proximal and symmetrical weakness, without extra-muscular involvement. Other presentations have been described (including pseudo-dystrophic presentation). All patients had a previous statin medication (mean duration of 3.75 years) although this criteria is not a requisite. All patients had high levels of creatine kinase and abnormal electromyographic examination. The pathological pattern on muscular biopsy was a necrotizing myopathy without significant inflammatory cells infiltration. Cardio-respiratory function was normal and no associated neoplasia was found. Over the follow-up, we observed a marked corticosteroid-dependence, not improved by immunosuppressive drugs (azathioprine and methotrexate). The benefit of intravenous immunoglobulin was clear with, sometimes, prolonged responses. CONCLUSION: An early diagnosis of this myopathy is necessary in order to introduce an immunotherapy associated with a close monitoring. The therapeutic strategy (within which the stead of intravenous immunoglobulin seems increased) remains to be defined and long-term prospective studies are thus needed. SN - 1768-3122 UR - https://www.unboundmedicine.com/medline/citation/27876175/[Anti_HMG_CoA_reductase_necrotizing_myopathy:_A_report_of_4_cases]_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0248-8663(16)30965-1 DB - PRIME DP - Unbound Medicine ER -