Clinical and genetic associations of autoantibodies to 3-hydroxy-3-methyl-glutaryl-coenzyme a reductase in patients with immune-mediated myositis and necrotizing myopathy

2015 ◽  
Vol 52 (2) ◽  
pp. 196-203 ◽  
Author(s):  
Vidya Limaye ◽  
Chris Bundell ◽  
Peter Hollingsworth ◽  
Arada Rojana-Udomsart ◽  
Frank Mastaglia ◽  
...  
Keyword(s):  
Coenzyme A ◽  
Genetic Associations ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Coenzyme A Reductase

Immune-mediated myopathy related to anti 3-hydroxy-3-methylglutaryl-coenzyme A reductase antibodies as an emerging cause of necrotizing myopathy induced by statins

2014 ◽  
Vol 81 (1) ◽  
pp. 79-82 ◽  
Author(s):  
Clément Lahaye ◽  
Anne Marie Beaufrére ◽  
Olivier Boyer ◽  
Laurent Drouot ◽  
Martin Soubrier ◽  
...  
Keyword(s):  
Coenzyme A ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Coenzyme A Reductase

scholarly journals Diagnostic modelling and therapeutic monitoring of immune-mediated necrotizing myopathy: role of electrical myotonia

2020 ◽  
Vol 2 (2) ◽  
Author(s):  
James D Triplett ◽  
Shahar Shelly ◽  
Guy Livne ◽  
Margherita Milone ◽  
Charles D Kassardjian ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Odds Ratio ◽  
Coenzyme A ◽  
Signal Recognition Particle ◽  
Delayed Diagnosis ◽  
Signal Recognition ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Chronic Course ◽  
Coenzyme A Reductase

Abstract Delayed diagnosis of immune-mediated necrotizing myopathy leads to increased morbidity. Patients with the chronic course without 3-hydroxy-3-methylglutaryl-coenzyme-A reductase-IgG or signal recognition particle-IgG are often challenging to diagnose. Immunotherapy response can also be difficult to assess. We created a statistical model to assist immune-mediated necrotizing myopathy diagnosis. Electrical myotonia versus fibrillations were reviewed as biomarkers for immunotherapy treatment response. Identified were 119 immune-mediated necrotizing myopathy cases and 938 other myopathy patients. Inclusion criteria included all having electrophysiological evaluations, muscle biopsies showing inflammatory/necrotizing myopathies, comprehensively recorded neurological examinations, and creatine kinase values. Electrical myotonia was recorded in 56% (67/119) of retrospective and 67% (20/30) of our validation immune-mediated necrotizing myopathy cohorts, and significantly (P < 0.001) favoured immune-mediated necrotizing myopathy over other myopathies: sporadic inclusion body myositis (odds ratio = 4.78); dermatomyositis (odds ratio = 10.61); non-specific inflammatory myopathies (odds ratio = 8.46); limb-girdle muscular dystrophies (odds ratio = 5.34) or mitochondrial myopathies (odds ratio = 14.17). Electrical myotonia occurred in immune-mediated necrotizing myopathy seropositive (3-hydroxy-3-methylglutaryl-coenzyme-A reductase-IgG 70%, 37/53; signal recognition particle-IgG 29%, 5/17) and seronegative (51%, 25/49). Multivariate regression analysis of 20 variables identified 8 (including electrical myotonia) in combination accurately predicted immune-mediated necrotizing myopathy (97.1% area-under-curve). The model was validated in a separate cohort of 30 immune-mediated necrotizing myopathy cases. Delayed diagnosis of cases with electrical myotonia occurred in 24% (16/67, mean 8 months; range 0–194). Half (8/19) had a chronic course and were seronegative, with high model prediction (>86%) at the first visit. Inherited myopathies were commonly first suspected in them. Follow-up evaluation in patients with electrical myotonia on immunotherapy was available in 19 (median 21 months, range 2–124) which reduced from 36% (58/162) of muscles to 7% (8/121; P < 0.001). Reduced myotonia correlated with immunotherapy response in 64% (9/14) as well as with median creatine kinase reduction of 1779 U/l (range 401–9238, P < 0.001). Modelling clinical features with electrical myotonia is especially helpful in immune-mediated necrotizing myopathy diagnostic suspicion among chronic indolent and seronegative cases. Electrical myotonia favours immune-mediated necrotizing myopathy diagnosis and can serve as an adjuvant immunotherapy biomarker.

scholarly journals Case Report: Immune Mediated Necrotizing Myopathy With IgG Antibodies to 3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase (HMGCR) May Present With Acute Systolic Heart Failure

2020 ◽  
Vol 11 ◽  
Author(s):  
Malik Ghannam ◽  
Georgios Manousakis
Keyword(s):  
Heart Failure ◽  
Coenzyme A ◽  
Multiorgan Failure ◽  
Systolic Heart Failure ◽  
Symptomatic Treatment ◽  
Igg Antibodies ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Cardiac Evaluation ◽  
Coenzyme A Reductase

Involvement of cardiac muscle is felt to be very uncommon in anti-HMGCR myopathy, and therefore early cardiac evaluation is not considered a high priority for this condition. We herein present the case of a 72 year-old woman admitted due to dyspnea and orthopnea, who, in retrospect, suffered from proximal more than distal muscle weakness for 3 months prior to admission. She was found to have acute systolic heart failure. Serologic testing showed positive 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) IgG antibodies, and muscle biopsy showed necrotizing myopathy. No alternative explanation for heart failure was found. Despite immunotherapy and symptomatic treatment, she died from multiorgan failure. Our study suggests that heart failure in anti HMGCR myopathy may not be as rare as previously thought, and therefore early cardiac evaluation should be considered in patients with this diagnosis, to minimize morbidity and mortality.

scholarly journals Paraneoplastic Anti-3-hydroxy-3-methylglutary-coenzyme A Reductase Antibody-positive Immune-mediated Necrotizing Myopathy in a Patient with Uterine Cancer

2017 ◽  
Vol 56 (14) ◽  
pp. 1915-1918 ◽  
Author(s):  
Atsushi Mizuma ◽  
Maiko Kouchi ◽  
Shizuka Netsu ◽  
Sachiko Yutani ◽  
Ruriko Kitao ◽  
...  
Keyword(s):  
Coenzyme A ◽  
Uterine Cancer ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Coenzyme A Reductase

scholarly journals Pediatric necrotizing myopathy associated with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibodies

Rheumatology ◽  
2016 ◽  
Vol 56 (2) ◽  
pp. 287-293 ◽  
Author(s):  
Wen-Chen Liang ◽  
Akinori Uruha ◽  
Shigeaki Suzuki ◽  
Nobuyuki Murakami ◽  
Eri Takeshita ◽  
...  
Keyword(s):  
Coenzyme A ◽  
Necrotizing Myopathy ◽  
Coenzyme A Reductase

Anti-3-hydroxy-3-methylglutaryl-coenzyme a reductase necrotizing myopathy masquerading as a muscular dystrophy in a child

2017 ◽  
Vol 56 (6) ◽  
pp. 1177-1181 ◽  
Author(s):  
Payam Mohassel ◽  
A. Reghan Foley ◽  
Sandra Donkervoort ◽  
Pierre R. Fequiere ◽  
Katherine Pak ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Coenzyme A ◽  
Necrotizing Myopathy ◽  
Coenzyme A Reductase

scholarly journals Childhood-onset anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) necrotizing myopathy needs to be distinguished from muscular dystrophy: A case study

2017 ◽  
Vol 57 (10) ◽  
pp. 567-572 ◽  
Author(s):  
Yuka Hama ◽  
Madoka Mori-Yoshimura ◽  
Hirofumi Komaki ◽  
Shigeaki Suzuki ◽  
Hitoshi Kohsaka ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Coenzyme A ◽  
Childhood Onset ◽  
Necrotizing Myopathy ◽  
Coenzyme A Reductase
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