Severe statin-induced autoimmune myopathy successfully treated with intravenous immunoglobulin

Güngör, Cansu; Wieshmann, Udo

doi:10.1136/bcr-2020-234805

Cited by 6 publications

(5 citation statements)

References 20 publications

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“…Similarly, a cohort study of patients demonstrated a mean of 38.8 months (range 15–84 months) from the atorvastatin exposure until the first CK elevation [ 15 ]. There is a published case in which a patient was diagnosed with immune-mediated necrotizing myopathy after taking a statin for 10 years [ 16 ]. From this, the times between exposure and symptom onset vary greatly.…”

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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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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“…The proposed mechanism of SINM involves the up-regulation of HMGCR within muscle tissue, which becomes a target for autoantibodies for inducing autoimmune necrosis [ 7 ]. SINM can occur in patients who have been receiving long-term statin therapy [ 9 ]. A large systematic review found that patients with a mean age of 65 who had been on statins for over three years subsequently developed inflammatory myositis [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

Statin-Induced Myositis with Concomitant Myocarditis

Patail¹,

Kothari²,

Nadig³

et al. 2022

Cureus

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Statins are commonly prescribed medications that provide many significant cardiovascular benefits for both primary and secondary prevention in patients with and without coronary artery disease. Known adverse effects of these medications include varying degrees of muscle toxicity, including myalgia, myopathy, and rare cases of necrotizing myositis, hepatic dysfunction, and central nervous system changes. Despite known adverse effects, statins are rarely associated with myocarditis. Statins can cause skeletal muscle myopathy and myositis by upregulating HMG-CoA reductase (HMGCR) in muscle tissue, resulting in antibodymediated inflammation. A similar proposed mechanism is likely possible within cardiac myocytes. We present a rare case of statin-induced necrotizing myositis with concomitant cardiac involvement. Severe skeletal muscle myositis was confirmed by lower extremity MRI and biopsy findings. In association, elevated and plateaued high-sensitivity troponin without evidence of cardiac ischemia warranted cardiac MRI, which further confirmed myocarditis due to inflammation within a non-vascular distribution. Given its rare presentation, the treatment for statin-induced cardiac toxicity is unclear; however, the patient in this case report was treated with pulse-dose intravenous steroids and indefinite discontinuation of statin medications.

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“…Our patient outcomes support the need for early immunosuppression in most patients. Although IVIG is often reserved for patients who have not benefitted from either steroid or oral immunosuppressive therapy, several suggestive studies have shown that IVIG could be used as a first-line monotherapy in select patients, particularly those with type 2 diabetes [ 3 , 6 ]. Indeed, 4 of 5 patients with diabetes in our series achieved marked improvement after initiation of IVIG, suggesting a potential earlier role for this therapy in diabetic patients.…”

Section: Discussionmentioning

confidence: 99%

“…Findings of a positive anti-HMG-CoA reductase (anti-HMGCR) antibody in the appropriate clinical context are highly suggestive of the diagnosis, which is further established with muscle biopsy pathology demonstrating a necrotizing myopathy with muscle-cell necrosis and regeneration [ 3 ]. Patients usually present clinically with progressive symmetric proximal weakness that may persist or worsen even after statin therapy is discontinued [ 2 – 6 ]. To date, no randomized controlled trials have been conducted to identify the most effective treatment for statin-associated autoimmune myopathy, but first-line treatment is typically a combination of steroids and immunosuppressive drugs [ 4 , 5 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

“…To date, no randomized controlled trials have been conducted to identify the most effective treatment for statin-associated autoimmune myopathy, but first-line treatment is typically a combination of steroids and immunosuppressive drugs [ 4 , 5 , 7 ]. In addition to immunosuppressive treatment, compelling data highlights the treatment benefits of IVIG [ 4 , 6 , 8 ].…”

Section: Introductionmentioning

confidence: 99%

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Challenges in Treating Statin-Associated Necrotizing Myopathy

Webster

Wiemer

Harash

et al. 2021

Case Reports in Rheumatology

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Myalgia and mild elevation in muscle enzymes are common side effects of statin therapy. While these symptoms are generally self-limited, in rare cases, statin use is associated with an immune-mediated necrotizing myopathy caused by development of autoantibodies against HMG-CoA reductase. The primary presenting symptom of this condition is progressive symmetric proximal weakness that does not abate or worsens even after cessation of statin therapy and is associated with markedly elevated creatine kinase (CK) levels. To date, no randomized controlled trials have been conducted to identify the most effective treatment for statin-associated autoimmune myopathy. Treatment recommendations involve a combination of steroids and immunosuppressive drugs. This single-center case series highlights the clinicopathologic features diagnostic for statin-associated autoimmune myopathy as well as treatment challenges for the patient population. The series highlights a range of potential presentations, from mildly symptomatic despite highly elevated CK, to severe muscle weakness including dysphagia. Multiple patients required several immunosuppressant medications as well as intravenous immunoglobulin (IVIG) to achieve disease control. In this case series, marked improvement was noted in several diabetic patients with IVIG.

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Severe statin-induced autoimmune myopathy successfully treated with intravenous immunoglobulin

Cited by 6 publications

References 20 publications

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

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

Statin-Induced Myositis with Concomitant Myocarditis

Challenges in Treating Statin-Associated Necrotizing Myopathy

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