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.

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.

Inclusion body myositis — a case based clinicopathological update

Open Medicine ◽  
2014 ◽  
Vol 9 (1) ◽  
pp. 80-85
Author(s):  
Levente Bodoki ◽  
Melinda Vincze ◽  
Zoltán Griger ◽  
Tamás Csonka ◽  
Balázs Murnyák ◽  
...  
Keyword(s):  
Inclusion Body ◽  
Inclusion Body Myositis ◽  
Treatment Options ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Older Patient ◽  
Close Collaboration ◽  
Systemic Autoimmune Diseases ◽  
Case Based

AbstractInclusion body myositis is a slowly progressive myopathy affecting predominantly the middle-aged and older patient population. It is a major form of the idiopathic inflammatory myopathies which are chronic systemic autoimmune diseases characterized by symmetrical proximal muscle weakness. Unfortunately, there is no effective therapy yet; however, the early diagnosis is essential to provide treatment options which may significantly slow the progression of the disease. In our case-based clinicopathological study the importance of the close collaboration between the clinician and the neuropathologist is emphasised.

Idiopathic inflammatory myopathies: polymyositis and dermatomyositis

2011 ◽  
pp. 385-404
Author(s):  
Alan J. Hakim ◽  
Gavin P.R. Clunie ◽  
Inam Haq
Keyword(s):  
Inclusion Body ◽  
Clinical Features ◽  
Muscle Weakness ◽  
Inclusion Body Myositis ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Breakdown

Epidemiology and diagnosis 386 Clinical features of polymyositis and dermatomyositis 388 Investigation of polymyositis and dermatomyositis 392 Autoantibodies in myositis 394 Treatment of polymyositis and dermatomyositis 398 Inclusion-body myositis 401 Polymyositis and dermatomyositis in children 402 • The idiopathic inflammatory myopathies are characterized by proximal muscle weakness and evidence of autoimmune-mediated muscle breakdown. These disorders include:...

scholarly journals Juvenile Dermatomyositis and the Inflammatory Myopathies

2020 ◽  
Vol 40 (03) ◽  
pp. 342-348
Author(s):  
Collin Swafford ◽  
E. Steve Roach
Keyword(s):  
Muscle Weakness ◽  
Muscle Injury ◽  
Juvenile Dermatomyositis ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Rimmed Vacuoles ◽  
Immune Mediated ◽  
Primary Disorder ◽  
Tissue Changes

AbstractThe inflammatory myopathies comprise disorders of immune-mediated muscle injury. The histopathology and clinical features help distinguish them. Juvenile dermatomyositis (JDM) is the most common form of myositis in children and adolescents. Children with JDM present with proximal muscle weakness and characteristic rashes. The presentation is similar in children and adults, but JDM is a primary disorder and the adult form often is concerning for a paraneoplastic syndrome. Proximal muscle weakness occurs with dermatomyositis, polymyositis, and immune-mediated necrotizing myopathy, but the latter two conditions have no dermatologic findings or distinct tissue changes which set them apart from dermatomyositis. Inclusion body myositis, also included in the inflammatory myopathies, presents with more distal involvement, and microscopically exhibits identifiable rimmed vacuoles. We review key features of these disorders, focusing in more detail on JDM because it is more often encountered by the child neurologist.

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.

A Case of Immune-Mediated Necrotizing Myopathy With Associated Skeletal Muscle Involvement by Sarcoid Granulomata: A Rare Association

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S69-S69
Author(s):  
Shalla Akbar ◽  
Sandhya Dasaraju ◽  
Osama Elkadi
Keyword(s):  
Skeletal Muscle ◽  
Muscle Weakness ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Muscle Involvement ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Skeletal Muscle Involvement

Abstract Skeletal muscle involvement by noncaseating granulomata occurs in a variety of conditions, including sarcoidosis, infections, and rarely in association with primary inflammatory myopathies such as inclusion body myositis (IBM) and dermatomyositis (DM). Sarcoid myopathy is typically asymptomatic; however, a picture of acute myositis with proximal muscle weakness has been described. Immune-mediated necrotizing myopathy (IMNM) is a subgroup of inflammatory myopathies typically presenting with proximal muscle weakness and markedly elevated muscle enzymes, mostly occurring in the setting of statin treatment. IMNM is associated with positive autoantibodies, but a subset of cases is antibody negative. Here we describe a case of myopathy occurring in association with sarcoidosis with combined features of granulomatous and necrotizing myopathy. The patient was a 54-year-old African American male with medical history significant for statin use 3 years ago, which was discontinued due to myalgia and elevated muscle enzymes and biopsy-proven sarcoidosis diagnosed on a pulmonary lymph node biopsy. He presented with progressive worsening of bilateral proximal weakness involving the upper and lower extremities. Electromyogram showed features of active myopathy with no evidence of peripheral neuropathy. Myositis panel was negative for the following antibodies: anti-Jo1, Mi-2, anti-Ku, PL-7, PL-12, OJ, EJ, and SRP. However, there was elevation of aldolase, CRP, and CK-MB. Biopsy of thigh and deltoid muscle showed necrotic muscle fibers, myophagocytosis with associated minimal inflammation, and multiple well-formed nonnecrotizing granulomas with multinucleated giant cells. Myopathic features include increased internalized nuclei, round atrophic fibers, and scattered split fibers. Specific features of IBM or DM were not present. Conclusion Myopathies developing or worsening after discontinuation of statin are rare. The association of necrotizing myopathy with sarcoidosis is not well described in the literature. Additional studies are warranted to elucidate this association.

scholarly journals ANCA-associated vasculitis and severe proximal muscle weakness

2021 ◽  
pp. 1-3
Author(s):  
Jordan S. Dutcher ◽  
Albert Bui ◽  
Tochukwu A. Ibe ◽  
Goyal Umadat ◽  
Eugene P. Harper ◽  
...  
Keyword(s):  
Muscle Weakness ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Anca Associated Vasculitis

Juvenile dermatomyositis: a case of delayed recognition with unusual complication of nephrocalcinosis

2021 ◽  
Vol 14 (4) ◽  
pp. e241152
Author(s):  
Geminiganesan Sangeetha ◽  
Divya Dhanabal ◽  
Saktipriya Mouttou Prebagarane ◽  
Mahesh Janarthanan
Keyword(s):  
Muscle Weakness ◽  
Juvenile Dermatomyositis ◽  
Inflammatory Myopathy ◽  
Unusual Complication ◽  
Ultrasound Screening ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Calcinosis Cutis ◽  
First Case ◽  
Medullary Nephrocalcinosis

Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in children and is characterised by the presence of proximal muscle weakness, heliotrope dermatitis, Gottron’s papules and occasionally auto antibodies. The disease primarily affects skin and muscles, but can also affect other organs. Renal manifestations though common in autoimmune conditions like lupus are rare in JDM. We describe a child whose presenting complaint was extensive calcinosis cutis. Subtle features of proximal muscle weakness were detected on examination. MRI of thighs and a muscle biopsy confirmed myositis. Nephrocalcinosis was found during routine ultrasound screening. We report the first case of a child presenting with rare association of dermatomyositis, calcinosis cutis and bilateral medullary nephrocalcinosis.

scholarly journals Juvenile dermatomyositis - A case report with review on oral manifestations and oral health considerations

2018 ◽  
Vol 44 (1) ◽  
pp. 52-61
Author(s):  
Pritesh Ruparelia ◽  
Oshin Verma ◽  
Vrutti Shah ◽  
Krishna Shah
Keyword(s):  
Oral Health ◽  
Muscle Weakness ◽  
Juvenile Dermatomyositis ◽  
Oral Manifestations ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Inflammatory Myositis ◽  
Life Threatening

Juvenile Dermatomyositis is the most common inflammatory myositis in children, distinguished by proximal muscle weakness, a characteristic rash and Gottron’s papules. The oral lesions most commonly manifest as diffuse stomatitis and pharyngitis with halitosis. We report a case of an 8 year old male with proximal muscle weakness of all four limbs, rash, Gottron’s papules and oral manifestations. Oral health professionals must be aware of the extraoral and intraoral findings of this rare, but potentially life threatening autoimmune disease of childhood, for early diagnosis, treatment, prevention of long-term complications and to improve the prognosis and hence, the quality of life for the patient.

scholarly journals Dermatomyositis-specifikus autoantitesttel rendelkező és autoantitest-negatív betegeink klinikai jellemzőinek és laboratóriumi paramétereinek összehasonlítása

2015 ◽  
Vol 156 (36) ◽  
pp. 1451-1459 ◽  
Author(s):  
Levente Bodoki ◽  
Dóra Budai ◽  
Melinda Nagy-Vincze ◽  
Zoltán Griger ◽  
Zoe Betteridge ◽  
...  
Keyword(s):  
Muscle Weakness ◽  
Matrix Protein ◽  
Skin Lesions ◽  
Clinical Findings ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Nuclear Matrix Protein ◽  
Specific Antibodies ◽  
Antibody Positivity ◽  
Specific Autoantibody

Introduction: Myositis is an autoimmune disease characterised by proximal muscle weakness. Aim: The aim of the authors was to determine the frequency of dermatomyositis-specific autoantibodies (anti-Mi-2, anti-transcriptional intermediary factor 1 gamma, anti-nuclear matrix protein 2, anti-small ubiquitin-like modifier activating enzyme, anti-melanoma differentiation-associated gene) in a Hungarian myositis population and to compare the clinical features with the characteristics of patients without myositis-specific antibodies. Method: Antibodies were detected using immunoblot and immunoprecipitation. Results: Of the 330 patients with nyositis, 48 patients showed dermatomyositis-specific antibody positivity. The frequency of antibodies in these patients was lower than those published in literature Retrospective analysis of clinical findings and medical history revealed that patients with dermatomyositis-specific autoantibody had more severe muscle weakness and severe skin lesions at the beginning of the disease. Conclusions: Antibodies seem to be useful markers for distinct clinical subsets, for predicting the prognosis of myositis and the effectiveness of the therapy. Orv. Hetil., 2015, 156(36), 1451–1459.

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