scholarly journals Statin-induced necrotising autoimmune myopathy and autoimmune hepatitis presenting with dysphagia

2020 ◽  
Vol 13 (2) ◽  
pp. e232391 ◽  
Author(s):  
Osama Qasim Agha ◽  
Sukhdeep Kaur ◽  
Nirmal Vijayavel
Keyword(s):  
Creatine Kinase ◽  
Autoimmune Hepatitis ◽  
Complete Resolution ◽  
Oropharyngeal Dysphagia ◽  
Liver Function Tests ◽  
Unknown Aetiology ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Autoimmune Myopathy ◽  
Smooth Muscle Antibodies

Statin-induced necrotising autoimmune myopathy (SINAM) is a rare disease characterised by proximal muscle weakness and elevated creatine kinase levels that is usually in the thousands. Anti-3-hydroxy-3-methyl glutaryl co-enzyme A reductase (HMGCR) antibodies are associated with SINAM. Autoimmune hepatitis (AIH) is an inflammatory disease of the liver that is usually of unknown aetiology but can also be associated with concurrent extrahepatic autoimmune disorders. We are reporting a case of biopsy proven AIH associated with SINAM in a patient presenting with oropharyngeal dysphagia. The patient had elevated anti-HMGCR antibodies and anti-smooth muscle antibodies. SINAM and AIH were confirmed by muscle biopsy and liver biopsy, respectively. The patient had complete resolution of his symptoms and complete normalisation of his liver function tests after 6 months of the treatment.

scholarly journals Atypical presentation of necrotising autoimmune myopathy

2019 ◽  
Vol 12 (5) ◽  
pp. e229119 ◽  
Author(s):  
Oyintayo Ajiboye ◽  
Michelle Manesh ◽  
Nisar Asmi ◽  
Benjamin Mba
Keyword(s):  
Muscle Weakness ◽  
Oropharyngeal Dysphagia ◽  
Rapid Progression ◽  
Atypical Presentation ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Autoimmune Antibodies ◽  
Autoimmune Myopathy ◽  
High Index Of Suspicion ◽  
Statin Use

Necrotising autoimmune myopathy (NAM) is characterised by a common phenotype of profound symmetrical proximal muscle weakness, elevated creatine kinase levels, irritable myopathy on electromyography and histological findings of myocyte necrosis and regeneration without remarkable inflammation. NAM is associated with autoimmune antibodies including anti-3-hydroxy-3-methylglutaryl-coenzyme receptor, which is strongly associated with statin use. We report a case of statin-associated NAM with an atypical presentation of severe oropharyngeal dysphagia and no remarkable proximal muscle weakness at initial presentation but with rapid progression to severe quadriparesis in weeks. This case expands the spectrum of presentation patterns of this rare disease and highlights the need for a high index of suspicion in patients with a remote history of statin use.

scholarly journals Severe proximal myopathy secondary to Hashimoto’s thyroiditis

2019 ◽  
Vol 12 (7) ◽  
pp. e230427
Author(s):  
William Jervis ◽  
Najeeb Shah ◽  
Shiva Kumar Mongolu ◽  
Thozhukat Sathyapalan
Keyword(s):  
Creatine Kinase ◽  
Differential Diagnosis ◽  
Muscle Weakness ◽  
Creatine Kinase Level ◽  
Complete Resolution ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Rare Differential Diagnosis ◽  
Thyroxine Replacement ◽  
Proximal Myopathy

Muscular symptoms in hypothyroidism are common, including myalgia, fatigue and cramps; however, a significantly raised creatine kinase and muscle weakness are rare. Differential diagnosis of patients presenting with muscle weakness and a raised creatine kinase is wide, and hypothyroidism is rarely considered. We report this case of a 30-year-old female presenting with proximal muscle weakness as her primary symptom, hypothyroid symptoms of 3-month duration and a significantly raised creatine kinase. After ruling out other causes of a raised creatine kinase, thyroxine replacement was commenced, which led to complete resolution of her proximal weakness, myalgia and normalisation of creatine kinase level. This case illustrates severe proximal myopathy can be secondary to hypothyroidism, symptoms can resolve with thyroxine replacement and emphasises the importance of measuring thyroid function in patients with proximal weakness/myalgia and a significantly raised creatine kinase.

scholarly journals Diagnosis of dermatomyositis and polymyositis: a study of 102 cases

2000 ◽  
Vol 58 (3B) ◽  
pp. 789-799 ◽  
Author(s):  
ROSANA HERMINIA SCOLA ◽  
LINEU CESAR WERNECK ◽  
DANIEL MONTE SERRAT PREVEDELLO ◽  
EDIMAR LEANDRO TODERKE ◽  
FÁBIO MASSAITI IWAMOTO
Keyword(s):  
Weight Loss ◽  
Creatine Kinase ◽  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Malignant Neoplasm ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Systemic Involvement ◽  
Perifascicular Atrophy

Patients with dermatomyositis (DM) or polymyositis (PM) were studied retrospectively. The patients were divided into four groups: definite PM 24, probable PM 19, definite DM 34 and mild-early DM 25 cases. PM patients complained more often proximal muscle weakness [p <0.01]. DM patients complained more arthralgia [p <0.05], dysphagia [p <0.03] and weight loss [p <0.04]. Five patients had a malignant neoplasm and 9 had other connective-tissue disease. DM presented higher ESR than PM [p <0.002]. PM presented more significant increase in creatine kinase (CK) [p <0.02] and in alanine aminotransferase (ALT) [p <0.001] levels. Electromyography showed myopathic pattern in 76%. Muscle biopsy was the definitive test. Perifascicular atrophy was more frequent in definite DM than in mild-early DM group [p <0.03]. CONCLUSION: A small association with connective-tissue diseases and neoplasms was found. DM and PM are clinically different. DM presents systemic involvement affecting the skin, developing more severe arthralgia, dysphagia and weight loss and presenting higher values of ESR. PM presents a restricted and more significant involvement of muscles generating more weakness complaints and higher levels of serum muscle enzymes.

Immune-mediated necrotising myopathy in asymptomatic patients with high creatine kinase

2020 ◽  
Vol 13 (10) ◽  
pp. e235457
Author(s):  
Izadora Fonseca Zaiden Soares ◽  
Victoria Fernandez Comprido ◽  
Bianca Raquel Ruoh Harn Scovoli Hsu ◽  
Alzira Alves de Siqueira Carvalho
Keyword(s):  
Creatine Kinase ◽  
Muscle Biopsy ◽  
Signal Recognition Particle ◽  
Disease Onset ◽  
Delayed Diagnosis ◽  
Signal Recognition ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Asymptomatic Patients ◽  
Immune Mediated

Subacute symmetrical proximal muscle weakness and persistent elevated creatine kinase levels are typical of immune-mediated necrotising myopathy (IMNM). These conditions are accompanied by copious myofibre necrosis, degeneration and regeneration with minimal to no inflammation on muscle biopsy. We report two cases (case 1 and case 2) of asymptomatic IMNM from different families with hyperCKaemia associated with positive anti-signal recognition particle (SRP) and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies, respectively, and we also reviewed the literature. There are only a few previous descriptions of patients with asymptomatic IMNM.The disease onset could be insidious and lead to delayed diagnosis and treatment. We recommend testing for the anti-HMGCR and anti-SRP antibodies in patients with idiopathic hyperCKaemia because they could show no symptoms of this disorder.

Statin-induced necrotising autoimmune myopathy: a rare complication of statin therapy

2021 ◽  
Vol 14 (4) ◽  
pp. e240865
Author(s):  
Muhammad Tauseef Ghaffar ◽  
Avinash Radhakrishna ◽  
Imran Ali ◽  
Byran Whelan
Keyword(s):  
Muscle Weakness ◽  
Rare Complication ◽  
Response To Treatment ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Hmg Coa Reductase ◽  
Muscle Groups ◽  
Autoimmune Myopathy ◽  
Coa Reductase ◽  
Statin Use

Statin-induced necrotising autoimmune myopathy (SINAM), a rare complication of statin use, presents with significant proximal muscle weakness and raised creatine kinase (CK) levels (50–100 times). This is different from other musculoskeletal conditions caused by statin use. Anti-hydroxy-methyl-glutaryl-coenzyme A reductase (HMG-CoA) reductase antibody is usually positive in SINAM and it generally indicates good response to immunosuppressive medications. We report a case of a 52-year-old man who presented with a 2-month history of significant upper and lower extremity proximal muscle weakness and a CK level of >10 000. He was started on atorvastatin for myocardial infarction 3 years ago. MRI pelvis, including proximal thigh, showed diffuse muscle oedema to all muscle groups. Muscle biopsy was suggestive of necrotising myopathy. His HMG-CoA reductase antibody was also positive. His treatment regimen consisted of immunosuppressants, including steroids. He also required extensive physiotherapy and showed response to treatment when reviewed in the outpatient clinic 9 months later.

scholarly journals Rituximab in the treatment of immune-mediated necrotizing myopathy: a review of case reports and case series

2021 ◽  
Vol 14 ◽  
pp. 175628642199891
Author(s):  
Anji Xiong ◽  
Guancui Yang ◽  
Zhuoyao Song ◽  
Chen Xiong ◽  
Deng Liu ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Case Reports ◽  
Signal Recognition Particle ◽  
High Dose ◽  
Signal Recognition ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Coa Reductase

Immune-mediated necrotizing myopathy (IMNM) is a group of immune-related myopathies characterized by progressive proximal muscle weakness, extremely high serum creatine kinase (CK) levels, and necrotic muscle fibers with a relative lack of inflammation. Treatment of IMNM is challenging, with most cases refractory to high-dose steroids in combination with multiple immunotherapies. The role of rituximab (RTX) for IMNM has been explored in isolated case reports and small series. The aim of this article was to perform a literature review of patients with IMNM treated with RTX and to evaluate RTX efficacy and safety. A total of 34 patients with IMNM were reviewed: 52.9% (18/34) with anti-signal recognition particle (SRP) antibodies and 47.1% (16/34) with anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibodies. Patient age at onset varied from 11 years to 81 years (mean 41 years). The majority of patients presented as a severe proximal muscle weakness and the peak level of CK varied from 3900 IU/L to 56,000 IU/L (mean 18,440 IU/L). Prior to RTX administration, all patients were treated with high-dose steroids and most were treated with multiple immunotherapies. The reason for initiating RTX was that 64.7% (22/34) of patients showed no improvement after previous treatments, and 35.3% (12/34) of patients relapsed when attempting to wean steroids or other immunosuppressive agents. With regard to RTX efficacy, 61.8% (21/34) of patients presented a response to RTX. Our data may support the use of RTX as an effective treatment strategy against IMNM resistant to steroids and multiple immunotherapies. Meanwhile, RTX as a first-line therapy could be a choice in IMNM, particularly in African Americans with anti-SRP antibody-positive subsets. ANA, antinuclear antibody; CK, creatine kinase; HMGCR, 3-hydroxy-3-methylglutaryl-CoA reductase; IMNM, immune-mediated necrotizing myopathy; MAC, membrane attack complex; MHC-I, major histocompatibility complex-I; RTX, rituximab; SRP, signal recognition particle.

scholarly journals Isolated Oropharyngeal Dysphagia as the Initial Presentation of Anti-SRP Immune-Mediated Necrotizing Myopathy

2020 ◽  
Author(s):  
Pat Korathanakhun ◽  
Thanyalak Amornpojnimman
Keyword(s):  
Muscle Weakness ◽  
Clinical Course ◽  
Signal Recognition Particle ◽  
Oropharyngeal Dysphagia ◽  
Signal Recognition ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Electrodiagnostic Study

A 51-year-old male initially presented with a progressive course of isolated oropharyngeal dysphagia prior to the clinical course of painful symmetrical proximal muscle weakness without sensory deficit which rendered him to wheelchairbound status within 5 months. The physical examination revealed symmetrical proximal muscle weakness without sensory symptoms. The initial serum creatine kinase was extremely high and the electrodiagnostic study revealed a myopathic pattern. A muscle biopsy of the left biceps suggested a diagnosis of immune-mediated necrotizing myopathy (IMNM) and the serum anti-signal recognition particle (SRP) autoantibody was finally detected. This case presented a rare form of anti-SRP IMNM in which isolated oropharyngeal dysphagia preceded the onset of proximal muscle weakness.

Necrotizing autoimmune myopathy

2012 ◽  
Vol 153 (38) ◽  
pp. 1502-1507 ◽  
Author(s):  
Levente Bodoki ◽  
Melinda Vincze ◽  
Tibor Hortobágyi ◽  
Zoltán Griger ◽  
Karolina Cseri ◽  
...  
Keyword(s):  
Muscle Weakness ◽  
Rare Entity ◽  
Separate Entity ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Systemic Autoimmune Diseases ◽  
Necrotizing Autoimmune Myopathy ◽  
Autoimmune Myopathy ◽  
Very High

Idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by symmetrical proximal muscle weakness. One of them is the subgroup of necrotizing autoimmune myopathy, which has recently been recognized as a separate entity. In addition to the typical symmetrical muscle weakness, it is characterized by very high creatine kinase levels, myopathic triad in the electromyography, and myocyte necrosis without significant inflammation. The paper aims to review this rare entity, which has to be diagnosed and treated quickly in every case. Orv. Hetil., 2012, 153, 1502–1507.

scholarly journals Statin and fibrate associed myopathy: study of eight patients

2004 ◽  
Vol 62 (2a) ◽  
pp. 257-261 ◽  
Author(s):  
Alzira A. Siqueira Carvalho ◽  
Ürsula Waleska Poti Lima ◽  
Raul Alberto Valiente
Keyword(s):  
Creatine Kinase ◽  
Muscle Weakness ◽  
Muscle Biopsy ◽  
Clinical Course ◽  
Lipid Lowering ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Inflammatory Infiltration ◽  
Lipid Lowering Drugs ◽  
Ragged Red Fibers

Lipid-lowering drugs have been occasionally associated with neuromuscular symptoms and muscle biopsy changes. We reported the clinical course and the muscle biopsy in eight patients with hyperlipoproteinemia, treated with lipid -lowering drugs (statins/fibrates). Five patients had myalgias while; in two cases there was proximal muscle weakness. All patients became asymptomatic after the withdrawal of the drug, although creatine kinase remained elevated. We performed muscle biopsy in six cases from three months to two years after suspension of the drug. We found variation in fibers diameters in all cases, with necrosis of fibers in five cases, inflammatory infiltration in one case, the presence of vacuolated fiber in one patient and ragged-red fibers in three subjects. We concluded that although the muscle biopsy findings were not specific, the prolonged use of statins and or fibrates might induce a chronic myopathy even in the absence of symptoms.

scholarly journals Statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like features: A case report

2020 ◽  
Vol 8 ◽  
pp. 2050313X2098412
Author(s):  
Darosa Lim ◽  
Océane Landon-Cardinal ◽  
Benjamin Ellezam ◽  
Annie Belisle ◽  
Annie Genois ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Muscle Weakness ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Intravenous Immunoglobulins ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Therapeutic Implications ◽  
Necrotizing Myopathy ◽  
Immune Mediated

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) immune-mediated necrotizing myopathy is a subtype of idiopathic inflammatory myopathy which may be associated with statin exposure. It presents with severe proximal muscle weakness, high creatine kinase levels and muscle fiber necrosis. Treatment with intravenous immunoglobulins and immunosuppressants is often necessary. This entity is not commonly known among dermatologists as there are usually no extramuscular manifestations. We report a rare case of statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like cutaneous features. The possibility of anti-HMGCR immune-mediated necrotizing myopathy should be considered in patients with cutaneous dermatomyositis-like features associated with severe proximal muscle weakness, highly elevated creatine kinase levels and possible statin exposure. This indicates the importance of muscle biopsy and specific autoantibody testing for accurate diagnosis, as well as significant therapeutic implications.

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