Immune Mediated Necrotizing Myopathy: A Rare Complication of Statin Therapy

Abusharar, Shady Piedra; Moku, Prashanth; Banks, Sharon; Khalid, Fahad M; Specht, Charles S.; Polimera, Hyma

doi:10.4081/cp.2020.1248

Cited by 5 publications

(7 citation statements)

References 20 publications

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“…Treatment of anti-HMGCR myopathy appears to be safer and more efficacious if commenced early, perhaps due to lower doses and durations of immunosuppressant required, and more minimal inflammation in the early stages of diseases [18]. Our review of the literature suggests that in general, a triple therapy of corticosteroids, IVIG, and a corticosteroid-sparing immunosuppressant appears efficacious in patients with proximal weakness [7][8][9][10][11][12][13][14]. However, a dual combination of steroids and a steroid-sparing agent may be sufficient, especially important as there is a global shortage of IVIG.…”

Section: Discussionmentioning

confidence: 90%

“…Interestingly, 85% of muscle symptoms begun within 1 month of statin initiation, or titration to a higher dose [3]. It is therefore relatively rare to only develop muscle weakness symptoms over 4 years after starting statins, as was the case and those analysed in the review [7][8][9][10][11][12][13][14]. As such, on top of risk factors that influence the development of SRMs such as IMNM, other genetic factors may be at play.…”

Section: Discussionmentioning

confidence: 94%

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Challenges in the diagnosis and management of immune-mediated necrotising myopathy (IMNM) in a patient on long-term statins

2022

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Immune-mediated necrotising myopathy (IMNM) is a severe and poorly understood complication of statin use. Prompt management with immunosuppressive treatment is often needed to control the condition, which differs from the management of the more commonly recognised statin-induced myopathy. We present a case report and brief review of the literature regarding the pathogenesis, diagnosis, and management of anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) positive IMNM (HMGCR IMNM). There are no randomised clinical trials, but several smaller studies and cases suggest a triple therapy of corticosteroids, IVIG, and a corticosteroid-sparing immunosuppressant appears efficacious in patients with IMNM and proximal weakness. The mechanism of statin-induced IMNM is uncertain, and this is further complicated by the reports of HMGCR IMNM in statin-naïve patients, including children. We present a case of biopsy-confirmed HMGCR IMNM in a woman taking daily statins for treatment of hypercholesterolaemia for 4 years. She presented with symptoms consistent with a urinary tract infection (UTI), including muscle weakness. She was treated as an isolated case of UTI. One month later, she presented again with worsening weakness in her shoulders and hips. Creatine kinase was elevated, and MRI showed increased signal with STIR sequences in both thighs. Anti-HMGCR was positive and leg biopsy-confirmed necrotising changes. Stopping her statin prescription and a short course of prednisolone did not improve her muscle weakness. Adding methotrexate resulted in eventual resolution of her symptoms. IMNM should be considered as a differential in any patient taking statins presenting with muscle weakness, and this case suggests that immunosuppressant therapy in addition to cessation of statins is effective at treating IMNM. Clinical trials are needed to further investigate the efficacy of different combinations of immunosuppressants.

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Section: Discussionmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 94%

Challenges in the diagnosis and management of immune-mediated necrotising myopathy (IMNM) in a patient on long-term statins

2022

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“…The management of the patient’s diagnosis is a high dose of IV methylprednisolone (Solu-Medrol), which is considered the first-line treatment for this condition [ 23 , 24 ]. From the literature, a typical starting dose of prednisone (or equivalent) is 1 mg/kg/day [ 25 ].…”

Section: Discussionmentioning

confidence: 99%

“…It is essential for the patients to follow up with a rheumatologist or a neurologist to develop a long-term pharmacological treatment plan. Such a plan could include the addition of azathioprine at a dose of 3 mg/kg body weight or methotrexate 20–25 mg per week orally or subcutaneously [ 23 ]. Besides the above immune-suppressing drugs, rituximab may be considered in combination with methotrexate, though there have been reports that rituximab is ineffective in the treatment of HMG-CoA reductase antibody-positive myopathy [ 11 , 23 , 30 , 31 ].…”

Section: Discussionmentioning

confidence: 99%

Unveiling the Rare Complication: Statin-Induced Immune-Mediated Necrotizing Myopathy

Chowdhury,

Mahneva,

Maharaj

et al. 2023

Am J Case Rep

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Patient: Female, 59-year-old Final Diagnosis: Anti-HMG-CoA reductase-induced myopathy Symptoms: Balance problems • fatigue • muscle weakness • myalgia • myopathy Clinical Procedure: Muscle biopsy Specialty: General and Internal Medicine • Neurology • Rheumatology Objective: Unknown etiology Background: Statin-induced necrotizing autoimmune myopathy is an exceptionally rare yet severe complication of statin therapy that may develop in individuals at any time during their exposure to statins. The development of proximal muscle weakness, muscle pain, and elevated creatine kinase (CK) levels in patients while taking statins should prompt clinical consideration of statin-induced myopathy. The pathophysiology arises from the production of auto-antibodies, which target the 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMG-CoA reductase) enzyme, leading to the aggressive breakdown of myofibrils. Case Report: Here, we present a case of a 59-year-old woman with a medical history of dyslipidemia who developed anti-HMGCoA reductase antibodies after taking atorvastatin. She came to the emergency department with complaints of severe proximal muscle weakness. The laboratory workup showed an elevated CK level up to 12 000 IU/L. Despite discontinuing atorvastatin, the patient’s elevated CK levels persisted. The patient underwent a muscle biopsy, demonstrating myofibril necrosis. Serological analysis showed anti-HMG-CoA reductase antibodies in the patient’s serum, which led to the diagnosis of immune-mediated necrotizing myopathy due to statins. The patient’s statin therapy was promptly discontinued, and she was treated with a high dose of IV corticosteroids. After the patient’s discharge, brief discontinuation of the corticosteroids resulted in CK elevation and a return of symptoms. This led to the second re-admission and restarting of corticosteroids until stabilization and discharge. Conclusions: This case represents an important reminder for clinicians to recognize the possibility of statin-induced immune-mediated necrotizing myopathy in patients presenting with proximal muscle weakness while taking a statin, notwithstanding the rarity of this condition.

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“…Ancak tedaviye cevap vermeyen hastalarda disfaji ve solunum yetmezliği gibi ölümle sonuçlanabilen ciddi klinik tablolar da görülebilmektedir. [12] Statin ilişkili otoimmün nekrotizan miyopati nadir görülse de erken tanı ve tedavi ciddi önem taşıdığından, proksimal…”

Section: Discussionunclassified

A rare side effect: Statin induced autoimmune necrotizing myopathy

Nazlıgül¹

2021

bogazicitip

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Statin induced necrotizing autoimmune myopathy is a rare side effect and requires immunosuppressive therapy. Persistence of proximal muscle weakness and elevated creatine kinase level despite statin discontinuation, muscle necrosis and elevated anti-HMGCR (3-hydroxy 3-methyl glutaryl coenzyme A reductase) autoantibodies are distinguishing features from other statin related musculoskeletal side effects. In this report, a case using statin and diagnosed with autoimmune necrotizing myopathy is presented.

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Immune Mediated Necrotizing Myopathy: A Rare Complication of Statin Therapy

Cited by 5 publications

References 20 publications

Challenges in the diagnosis and management of immune-mediated necrotising myopathy (IMNM) in a patient on long-term statins

Challenges in the diagnosis and management of immune-mediated necrotising myopathy (IMNM) in a patient on long-term statins

Unveiling the Rare Complication: Statin-Induced Immune-Mediated Necrotizing Myopathy

A rare side effect: Statin induced autoimmune necrotizing myopathy

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