Inflammatory Myositis Following Statin Use in a Patient With Untreated Hypothyroidism.

Inflammatory myositis (IM) presents a diagnostic challenge due to its multifaceted etiology and varying clinical presentations. This case involves a 55-year-old male with asymptomatic hypothyroidism, recent statin use, and rapidly progressing proximal muscle weakness. He presented with profound weakness in the upper and lower extremities, severely impairing his daily activities. The patient's medical history included recent hospitalizations for idiopathic interstitial lung disease, myopericarditis, and pneumonia, adding complexity to his condition. Laboratory findings revealed elevated serum muscle enzymes, notably creatine kinase, indicating muscle damage and rhabdomyolysis. Serological testing confirmed the absence of myositis-specific antibodies and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies. The patient was eventually diagnosed with IM and rhabdomyolysis, likely secondary to statin use or hypothyroidism. Treatment with methylprednisolone, levothyroxine, and discontinuation of atorvastatin led to rapid improvements in AST levels and overall muscle strength. This case highlights the challenges of managing IM and emphasizes the importance of assessing thyroid function before initiating lipid-lowering therapy.

Dysphagia: A Case Report of an Atypical Presentation of Statin-Induced Necrotizing Myositis.

Statin medications act by inhibiting the enzyme hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase (HMGCR), thus decreasing hepatic cholesterol synthesis. They are considered the mainstay treatment of hypercholesterolemia due to their tremendous efficacy and mortality benefit. Although generally well tolerated, statins may adversely affect skeletal muscle resulting in side effects ranging from mild myalgia to life-threatening necrotizing myositis. Statin-induced necrotizing autoimmune myositis is a rare yet devastating adverse effect that may occur shortly after initiation of therapy or after several years of use. Unfortunately, medication discontinuation has shown no impact on prevention or alleviation of symptoms. Though there is currently no definitive guidance for the treatment of this condition, corticosteroids are generally considered to be first line, via high-dose oral prednisone or intravenous methylprednisolone. In this case report, we discuss the case of a 72-year-old male with an unusual presentation of statin-induced necrotizing autoimmune myositis: dysphagia, weakness, and weight loss. His diagnosis was confirmed by muscle biopsy indicating necrotizing myositis and his serum was found to be strongly positive for anti-HMG-CoA reductase antibodies. This patient had a very brief history of statin use, but his primary care provider discontinued the medication a couple of months prior to symptom onset due to elevated liver function tests. He was treated with aggressive intravenous fluid hydration and intravenous corticosteroids during an extended inpatient hospital stay. He was discharged to a rehabilitation facility. This report demonstrates the importance of creating a wide differential for patients who present with fatigue, generalized weakness, and dysphagia. It is essential to always consider statin-induced necrotizing myositis if a patient has a history of statin use, even if the statin has been discontinued. Necrotizing myositis demands timely diagnosis and management to improve mortality.

Autoimmune inflammatory myopathies.

The autoimmune inflammatory myopathies constitute a heterogeneous group of acquired myopathies that have in common the presence of endomysial inflammation and moderate to severe muscle weakness. Based on currently evolved distinct clinical, histologic, immunopathologic, and autoantibody features, these disorders can be best classified as dermatomyositis, necrotizing autoimmune myositis, antisynthetase syndrome-overlap myositis, and inclusion body myositis. Although polymyositis is no longer considered a distinct subset but rather an extinct entity, it is herein described because its clinicopathologic information has provided over many years fundamental information on T-cell-mediated myocytotoxicity, especially in reference to inclusion body myositis. Each inflammatory myopathy subset has distinct immunopathogenesis, prognosis, and response to immunotherapies, necessitating the need to correctly diagnose each subtype from the outset and avoid disease mimics. The paper describes the main clinical characteristics that aid in the diagnosis of each myositis subtype, highlights the distinct features on muscle morphology and immunopathology, elaborates on the potential role of autoantibodies in pathogenesis or diagnosis , and clarifies common uncertainties in reference to putative triggering factors such as statins and viruses including the 2019-coronavirus-2 pandemic. It extensively describes the main autoimmune markers related to autoinvasive myocytotoxic T-cells, activated B-cells, complement, cytokines, and the possible role of innate immunity. The concomitant myodegenerative features seen in inclusion body myositis along with their interrelationship between inflammation and degeneration are specifically emphasized. Finally, practical guidelines on the best therapeutic approaches are summarized based on up-to-date knowledge and controlled studies, highlighting the prospects of future immunotherapies and ongoing controversies.

Immune-mediated necrotizing myopathy (NAM) related to SARS-Cov-2 infection: a case report.

BACKGROUND: There is a growing body of evidence that severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) or COVID-19 infection is associated with the development of autoimmune diseases. A recent systematic review reported that the new-onset autoimmune disorders during or after COVID-19 infection included inflammatory myopathies such as immune-mediated necrotizing myopathies. CASE PRESENTATION: We described a 60-year-old man diagnosed with COVID-19 infection and later presented with a two-week history of myalgia, progressive limb weakness, and dysphagia. He had a Creatinine Kinase (CK) level of more than 10,000 U/L, was strongly positive for anti-signal recognition particle (SRP) and anti-Ro52 antibody, and a muscle biopsy revealed a paucity-inflammation necrotizing myopathy with randomly distributed necrotic fibers, which was consistent with necrotizing autoimmune myositis (NAM). He responded well clinically and biochemically to intravenous immunoglobulin, steroids and immunosuppressant and he was able to resume to his baseline. CONCLUSION: SARS-CoV-2 may be associated with late-onset necrotizing myositis, mimicking autoimmune inflammatory myositis.

Debilidad muscular proximal progresiva de inicio subagudo en un paciente anciano: descripción de un caso / Progressive proximal muscle weakness with subacute onset in an elderly patient: a case report

Introducción: Las estatinas son de los medicamentos más recetados. Aunque las estatinas generalmente se toleran bien, pueden provocar efectos secundarios musculoesqueléticos. La miopatía autoinmune necrotizante inducida por estatinas (SINAM) es una afección rara y la prevalencia sólo es de 1 de cada 100.000 personas. Este trastorno se caracteriza por debilidad muscular simétrica progresiva y grave, elevación marcada de la creatincinasa y síntomas persistentes a pesar de la interrupción de la estatina. La electromiografía suele mostrar un patrón de miopatía irritable inespecífico, indistinguible de otras miopatías inflamatorias. La biopsia muscular muestra la presencia de fibras necróticas, fibras en regeneración sin células inflamatorias significativas y una regulación positiva difusa o focal de la expresión del complejo mayor de histocompatibilidad de clase I. Los anticuerpos anti-3-hidroxi-3-metilglutaril-coenzima A (anti-HMG-CoA) reductasa representan un rasgo serológico característico de la SINAM.Caso clínico: Presentamos a un paciente que desarrolló debilidad muscular progresiva después de tomar simvastatina durante los últimos siete años. En la presentación inicial, su nivel de creatincinasa fue de 2.954 U/L y los anticuerpos anti-HMG-CoA reductasa fueron positivos. La biopsia mostró rasgos miopáticos profundos con numerosas fibras necróticas, algunas fibras en regeneración e infiltrado de células inflamatorias perimisial, combinado con una sobreexpresión difusa del complejo mayor de histocompatibilidad de clase I. Se le diagnosticó SINAM, se suspendió la estatina y se inició una dosis alta de corticoides sistémicos, inmunoglobulina intravenosa y metotrexato. Después de tres meses de seguimiento, tuvo una mejora significativa en la fuerza muscular y el nivel de creatincinasa volvió a la normalidad.(AU)

Introduction: Statins are some of the most widely prescribed medications. Although statins are generally well tolerated, they can lead to musculoskeletal side effects. Statin-induced necrotizing autoimmune myositis (SINAM) is a rare condition and the prevalence is only 1 per 100,000 people. This disorder is characterized by progressive and severe symmetric muscle weakness, marked elevation of creatine kinase and persistent symptoms despite statin discontinuation. Electromyography commonly shows a nonspecific irritable myopathy pattern indistinguishable from other inflammatory myopathies. Muscle biopsy shows the presence of necrotic fibers, regenerating fibers without significant inflammatory cells and diffuse or focal upregulation of major histocompatibility complex class I expression. The anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibodies represent a characteristic serological feature of SINAM. Case report: We present a patient who developed progressive muscle weakness after taking simvastatin for the last seven years. At initial presentation, her creatine kinase level was 2,954 U/L and anti-HMGCR antibodies were positive. The biopsy showed a profound myopathic features with numerous necrotic fibers, some regenerating fibers and perimysial inflammatory cell infiltrate, combined with a diffuse overexpression of major histocompatibility complex class I products. She was diagnosed with SINAM, statin was suspended and a high dose of systemic corticosteroids, intravenous immunoglobulin therapy and methotrexate was started. At three-month follow-up, she had significant improvement in muscle strength and creatine kinase level returned to normal...(AU)

Statin-Induced Necrotizing Autoimmune Myositis.

Statins are the most frequently prescribed medications for primary and secondary prevention of atherosclerotic cardiovascular disease (ASCVD). The United States Preventative Services Task Force recommends that clinicians selectively offer a statin for the primary prevention of ASCVD for adults aged 40 - 75 years with one or more cardiovascular disease risk factors and an estimated 10-year risk of a cardiovascular event of 10% or greater. Despite their ubiquity, it is estimated that approximately 6-10% of patients remain intolerant due to muscle aches. Here, we present a case of a 71-year-old female that was taking atorvastatin for a year and presented to the emergency room with proximal muscle aches and weakness. Laboratory values were significant for an elevated creatinine kinase of 4,166 U/L (reference range, 20 - 180). Her magnetic resonance imaging was significant for edema in bilateral lower extremity proximal muscles. Serology revealed a high anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody, confirming the diagnosis of statin-induced necrotizing autoimmune myositis. A muscle biopsy of the right vastus lateralis revealed necrotic muscle fibers. During her hospital course, she was treated with intravenous methylprednisolone, mycophenolate mofetil, and tacrolimus. Her symptoms gradually improved, and she was discharged after 14 days with a rheumatology follow-up. This is an exceedingly rare complication of statin use and has only recently received increasing attention. Here we present our experience with this disease.

Statin-Related Necrotizing Autoimmune Myositis: More Than Myalgia.

Statins are widely prescribed for cardiovascular disease, and, in general, are well tolerated by most people. Most side effects related to statins are mild, with some side effects also considered a nocebo effect. Occasionally, statins can be associated with severe side effects. One of the more severe adverse events is immune-mediated necrotizing myositis, which is both difficult to diagnose and treat. The symptoms can be debilitating, and aggressive immunosuppressive therapy is the best-recognized method of treatment of this complication. In this case report, we discuss the clinical features, diagnosis, and treatment of this disease entity with an emphasis on the need for rapid diagnosis and aggressive treatment to help reduce morbidity.

A Rapidly Debilitating Myopathy: A Rare Case of Statin-Induced Necrotizing Myositis.

Statins are well tolerated in general but can be associated with myopathies. Statin-induced myopathies can range widely from mild myalgias to necrotizing autoimmune myopathies. We present a case of an 81-year-old man on statins for five years with no complications, who developed progressive muscle weakness, rhabdomyolysis, and dysphagia. His laboratory workup revealed elevated inflammatory markers with creatine kinase (CK) levels above 2000 U/L. The myositis panel was negative, and the anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody was positive. His muscle biopsy showed randomly scattered necrotic fibers with minimal perivascular inflammation confirming statin-induced necrotizing autoimmune myopathy (SINAM). Statins were discontinued immediately after initial suspicion. The patient was started on intravenous immunoglobulin followed by hydrocortisone and mycophenolate mofetil. The patient continued to have muscle weakness and progressive dysphagia to the point that he could not handle his secretions. His disease course was complicated by recurrent aspiration pneumonia. Percutaneous endoscopic gastrostomy tube placement was considered, but his family decided on hospice care given his overall comorbidities. Physicians should note that SINAM can occur after a few months to several years of statin use. This disease can be rapidly debilitating and progress even after discontinuation of statins, and treatment requires immunosuppressants, including steroids and steroid-sparing agents.

Delayed-onset Necrotizing Myositis following COVID-19 Infection.

As the numbers of cases of COVID-19 continue to rise, the heterogeneity of its clinical manifestation continues to increase. Here, we describe a case of delayed-onset, biopsy-proven necrotizing myositis following infection with SARS-CoV-2. LEARNING POINTS: SARS-CoV-2 may be associated with late-onset necrotizing myositis, mimicking autoimmune inflammatory myositis.We need more studies to understand the underlying pathogenesis of SARS-CoV-2-induced myositis.

Inflammatory myopathies: A new landscape.

Greater accuracy in clinical descriptions combined with advances in muscle histology and immunology have established that inflammatory myopathies (IMs), similarly to inflammatory rheumatic diseases, constitute a highly heterogeneous group of conditions. The topographic distribution, severity, and tempo of onset of the myopathy vary widely, and the histological findings distinguish at least five different profiles, which may reflect different pathophysiological processes. Most IMs are connective tissue diseases that can affect multiple organs, among which the most common targets are the skin, joints, and lungs. The extramuscular manifestations may antedate the muscular involvement and should therefore suggest a diagnosis of IM even in the absence of obvious muscle disease. About 20 different autoantibodies have been identified in patients with IM. Some are mutually exclusive and associated with specific combinations of clinical manifestations. Following the model of antisynthetase syndrome, about 10 syndromes associated with autoantibodies specific of IM have been identified. Thus, polymyositis is now emerging as a rare entity that is often mistaken for more recently described patterns of IM. No consensus exists to date about the classification of IMs. Nevertheless, the clinical manifestations, autoantibody profile, and muscle histology can be used to distinguish patient subgroups with fairly homogeneous patterns of complications, treatment responses, and outcomes. These subgroups are also characterized by specific genetic and environmental factors. The advances made in the nosology of IMs have benefited the diagnosis, personalization of treatment strategies, and understanding of pathophysiological mechanisms. They can be expected to assist in the development of specific treatments.

Subcutaneous IgG in the Myositis Spectrum Disorders.

The efficacy of subcutaneous immunoglobulin is reported in several neurological disorders and, more recently, its use has been extended to other inflammatory diseases, such as the idiopathic inflammatory myopathies, including polymyositis and dermatomyositis. Due to the rarity of these disorders, the role of immunoglobulin, administered intravenously or subcutaneously, remains unclear and poorly investigated. We report our experience about the use of subcutaneous immunoglobulin in myositis spectrum disorders, from idiopathic inflammatory myopathies to more complex conditions, such as overlap and cancer-associated myositis or pregnancy.

Anti-signal Recognition Particle Necrotizing Autoimmune Myopathy: An Atypical Presentation.

Necrotizing autoimmune myopathy (NAM), also known as necrotizing autoimmune myositis, is a heterogeneous group of diseases characterized by the presence of necrotic muscle fibers on biopsy, elevated creatine kinase (CK) levels, an abnormal electromyogram (EMG) result, and the associated antibodies. The anti-signal recognition particle (anti-SRP) and the anti-3-hydroxyl-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibodies are the two most prevalent antibodies identified with NAM. NAM is a rare disease that typically affects middle-aged Caucasian women. In this case report, we present the diagnosis of anti-SRP NAM in a middle-aged African American male. This case report displays the atypical presentation of NAM outside of the typical patient population as well as the need for additional research to determine the pathogenesis and the precise role of anti-SRP antibodies in NAM.