Atypical presentation of necrotising autoimmune myopathy

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.

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

BMJ Case Reports ◽

10.1136/bcr-2020-240865 ◽

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.

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

BMJ Case Reports ◽

10.1136/bcr-2019-232391 ◽

2020 ◽

Vol 13 (2) ◽

pp. e232391 ◽

Cited By ~ 1

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.

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

Journal of Health Science and Medical Research ◽

10.31584/jhsmr.2020760 ◽

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.

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Necrotizing autoimmune myopathy

Orvosi Hetilap ◽

10.1556/oh.2012.29450 ◽

2012 ◽

Vol 153 (38) ◽

pp. 1502-1507 ◽

Cited By ~ 1

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.

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ANCA-associated vasculitis and severe proximal muscle weakness

Baylor University Medical Center Proceedings ◽

10.1080/08998280.2020.1871177 ◽

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

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

BMJ Case Reports ◽

10.1136/bcr-2020-241152 ◽

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.

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

International Journal of Orofacial Myology ◽

10.52010/ijom.2018.44.1.4 ◽

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.

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

Orvosi Hetilap ◽

10.1556/650.2015.30221 ◽

2015 ◽

Vol 156 (36) ◽

pp. 1451-1459 ◽

Cited By ~ 3

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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