Treatment experience of Taiwanese patients with anti‐3‐hydroxy‐3‐methylglutaryl‐coenzyme A reductase myopathy

Liang, W.; Wang, Chen Hua; Chen, Wan Zi; Kuo, Yun Ting; Lin, Hsiu Fen; Suzuki, Shigeaki; Nishino, Ichizo; Jong, Yuh Jyh

doi:10.1002/kjm2.12240

Cited by 6 publications

(4 citation statements)

References 24 publications

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“…Among idiopathic inflammatory myopathies (IIM), immune-mediated necrotizing myopathy (IMNM) has been defined as a disease with proximal weakness, high CK level and histopathological changes including muscle fiber necrosis and regeneration with unremarkable inflammation [4,5]. Anti-HMGCR myopathy is a subgroup of IMNM and diagnosed high anti-HMGCR autoantibody level in addition to these clinical and muscle biopsy findings [1,4].Anti-HMGCR myopathy was first recognized in patients with statin exposure, but later has been determined also in statin-naive patients, particularly young adults and pediatric patients. Clinical spectrum has been expanded to typical classic type with acute/subacute progressive, proximal weakness and chronic limb-girdle muscular dystrophy (LGMD)-like phenotype [1,6].…”

Section: Discussionmentioning

confidence: 99%

“…However, our patient had alleles HLA DRB1*08 and DRB1*15. Some reports suggest that the CK level can be utilized as a biomarker for disease activity in anti-HMGCR myopathy [1,4]. Remarkably, there are sufficient data that CK is an excellent marker of disease activity in anti-HMGCR, determining the level of muscle necrosis and being strongly correlated with levels of anti-HMGCR autoantibodies.…”

Section: Discussionmentioning

confidence: 99%

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Six Years Follow-Up of an 11-Year-Old Girl with Anti-HMGCR Myopathy

Cavusoglu,

Talim,

Ekinci

et al. 2024

JND

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Anti-HMGCR myopathy is decribed as an immune-mediated necrotizing myopathy which is characterised by subacute, progressive proximal muscle weakness and elevated creatine kinase (CK) level. In pediatric population, anti-HMGCR myopathy has been reported solely as small case reports, albeit rare. Although treatment consensus has not yet been established, proper treatment with several immunomodulators to include IVIg can show remarkable improvement. We report an 11-year-old-girl diagnosed with anti-HMGCR myopathy with 6 years of follow-up.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Six Years Follow-Up of an 11-Year-Old Girl with Anti-HMGCR Myopathy

Cavusoglu,

Talim,

Ekinci

et al. 2024

JND

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“…Differentiation can be achieved by de-termining myositis-specific antibodies ( 4), which we consider standard of care at our centre. While disease onset has been reported at any age, younger and statin-naive patients have an overall poorer treatment response and prognosis (14)(15)(16)26). Of particular importance is juvenile anti-HMGCRassociated IMNM with a recurrent and chronic disease course and a higher disease burden than in adults.…”

Section: Discussionmentioning

confidence: 99%

Treatment and outcomes in anti-HMG-CoA reductase-associated immune-mediated necrotising myopathy. Comparative analysis of a single-centre cohort and published data

Rademacher¹,

Glaubitz²,

Zechel

et al. 2022

Clinical and Experimental Rheumatology

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ObjectiveAnti-hydroxy-methyl-glutaryl-coenzyme A reductase (HMGCR) antibody-associated myopathy was recognised as a new form of immune-mediated necrotising myopathy (IMNM) a decade ago. Due to the rarity of the disease, only limited data on clinical manifestations and therapeutic outcomes are available. MethodsWe retrospectively analysed a monocentric cohort of HMGCR-associated IMNM patients treated at the University Medical Centre Göttingen. Clinical, laboratory, and biopsy data, as well as treatment outcomes, were analysed. In addition, a literature search was performed on published HMGCR IMNM cohorts in Medline and Web of Science. ResultsWe identified nine patients; five were female. The median age was 68 years . Six were statin-exposed and older than statin-naive patients (71 years [65-77] vs. 51 years ). All had muscle weakness, seven myalgias. Strength (MRC sum score) was 53/65 (46-61) at baseline and increased to 63/65 (50-65) with therapy. Creatine kinase (CK) levels decreased from a median level of 12837 U/L (range 6346-25011) to 624 U/L (35-1564 U/L). All received glucocorticoids (GC) and at least one immunosuppressive therapy. The literature review identified 26 studies comprising 691 patients. 57.9% were female, 61.3% statin exposed. 95.2% had weakness, 39.1% myalgia. Dysphagia affected 28.8%. 84.9% received GC and a median of 1.5 additional immunosuppressants. Compared to published data, our patients had higher baseline CK values (12837 [6346-25011] vs. 6951 [2539-10500], p<0.001), and we used azathioprine and intravenous immunoglobulins (p<0.001) more frequently but methotrexate and rituximab less frequently (p<0.001). Conclusion HMGCR-associated IMNM is a rare subset of myositis. With systemic treatment, patients usually achieve partial or complete remission. Optimal treatment has not been established, but glucocorticoids, azathioprine, and methotrexate are generally effective with or without intravenous immunoglobulins.

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“…They underwent muscle MRI, and it was found that adductors were earlier affected, while legs were relatively spared with the highest degree of involvement of the medial head of gastrocnemius. At the upper extremities, the biceps brachii was the most severely involved, followed by the triceps [ 71 ].…”

Section: Statins and Autoimmune Illnessesmentioning

confidence: 99%

Statins Neuromuscular Adverse Effects

Attardo

Musumeci

Velardo

et al. 2022

IJMS

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Statins are drugs widely prescribed in high-risk patients for cerebrovascular or cardiovascular diseases and are, usually, safe and well tolerated. However, these drugs sometimes may cause neuromuscular side effects that represent about two-third of all adverse events. Muscle-related adverse events include cramps, myalgia, weakness, immune-mediated necrotizing myopathy and, more rarely, rhabdomyolysis. Moreover, they may lead to peripheral neuropathy and induce or unmask a preexisting neuromuscular junction dysfunction. A clinical follow up of patients assuming statins could reveal early side effects that may cause neuromuscular damage and suggest how to better modulate their use. In fact, statin dechallenge or cessation, or the alternative use of other lipid-lowering agents, can avoid adverse events. This review summarizes the current knowledge on statin-associated neuromuscular adverse effects, diagnosis, and management. It is conceivable that the incidence of neuromuscular complications will increase because, nowadays, use of statins is even more diffused than in the past. On this purpose, it is expected that pharmacogenomic and environmental studies will help to timely predict neuromuscular complications due to statin exposure, leading to a more personalized therapeutic approach.

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Treatment experience of Taiwanese patients with anti‐3‐hydroxy‐3‐methylglutaryl‐coenzyme A reductase myopathy

Cited by 6 publications

References 24 publications

Six Years Follow-Up of an 11-Year-Old Girl with Anti-HMGCR Myopathy

Six Years Follow-Up of an 11-Year-Old Girl with Anti-HMGCR Myopathy

Treatment and outcomes in anti-HMG-CoA reductase-associated immune-mediated necrotising myopathy. Comparative analysis of a single-centre cohort and published data

Statins Neuromuscular Adverse Effects

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