scholarly journals The Significance of Autoantibodies in Juvenile Dermatomyositis

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Dominika Kwiatkowska ◽  
Adam Reich
Keyword(s):  
Juvenile Dermatomyositis ◽  
Immunosuppressive Agents ◽  
Treatment Strategies ◽  
Case Series ◽  
Autoimmune Disorder ◽  
Current Treatment ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Cutaneous Lesions

Juvenile dermatomyositis is a chronic and rare autoimmune disorder classified into the spectrum of idiopathic inflammatory myopathies. Although this entity is mainly characterized by the presence of pathognomonic cutaneous lesions and proximal muscle weakness, the clinical manifestation can be highly heterogeneous; thus, diagnosis might be challenging. Current treatment recommendations for juvenile dermatomyositis, based mainly upon case series, include the use of corticosteroids, immunomodulatory, and immunosuppressive agents. Recently, several specific autoantibodies have been shown to be associated with distinct clinical phenotypes of classic dermatomyositis. There is a need to further evaluate their relevance in the formation of various clinical features. Furthermore, while providing more personalized treatment strategies, one should consider diversity of autoantibody-related subgroups of juvenile dermatomyositis.

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 Anasarca as the presenting symptom of juvenile dermatomyositis: a case series

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Emily E. Schildt ◽  
Deirdre De Ranieri
Keyword(s):  
Juvenile Dermatomyositis ◽  
Early Recognition ◽  
Case Series ◽  
Atypical Presentation ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Rare Presentation ◽  
Case Presentation ◽  
Patients Âgés ◽  
Rare Manifestation

Abstract Background Juvenile Dermatomyositis (JDM) is an autoimmune disease that typically presents with classic skin rashes and proximal muscle weakness. Anasarca is a rare manifestation of this disease and is associated with a more severe and refractory course, requiring increased immunosuppression. Early recognition of this atypical presentation of JDM may lead to earlier treatment and better outcomes. Case presentation We present two female patients, ages 11 years old and 4 years old, who presented to the ED with anasarca and were subsequently diagnosed with JDM. Both patients required ICU-level care and significant immunosuppression, including prolonged courses of IV methylprednisolone, IVIG, and Rituximab. Conclusions Anasarca is a rare presentation of Juvenile Dermatomyositis, but it is important for clinicians to recognize this manifestation of the disease. Early recognition and treatment will lead to better outcomes in these children and hopefully decrease the need for prolonged hospitalization and ICU level care.

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 Transition From Full Mu Opioid Agonists to Buprenorphine in Opioid Dependent Patients—A Critical Review

2021 ◽  
Vol 12 ◽  
Author(s):  
Michael Soyka
Keyword(s):  
Partial Agonist ◽  
Treatment Strategies ◽  
Case Series ◽  
Current Treatment ◽  
Opioid Maintenance Treatment ◽  
Opioid Agonist ◽  
Mu Receptor ◽  
Dosing Strategies ◽  
Delta Receptor ◽  
Higher Doses

Methadone, a full opioid agonist at the mu-, kappa-, and delta-receptor, and buprenorphine, a partial agonist at the mu receptor, are first-line medications in opioid maintenance treatment. Transition from methadone to buprenorphine may precipitate withdrawal, and no accepted algorithm for this procedure has been developed. Current treatment strategies recommend transfer from methadone to buprenorphine predominantly in patients at low doses of methadone (30–40 mg/day). There are some reports indicating that transition from higher doses of methadone may be possible. A number of dosing strategies have been proposed to soften withdrawal symptoms and facilitate transfer including use of other opioids or medications and especially microdosing techniques for buprenorphine. The case series and studies available thus far are reviewed.

Multiple Sclerosis

2017 ◽  
Author(s):  
Pavan Bhargava ◽  
Peter A. Calabresi
Keyword(s):  
Multiple Sclerosis ◽  
Immunosuppressive Agents ◽  
Treatment Strategies ◽  
Current Treatment ◽  
Phase 2 ◽  
Future Directions ◽  
Disease Modifying Therapies ◽  
Future Challenges ◽  
Brain And Spinal Cord ◽  
The Brain

Multiple sclerosis is a chronic demyelinating neurological disorder of the brain and spinal cord, with both inflammatory and degenerative components. Current treatment strategies utilize immunomodulatory and immunosuppressive agents to reduce the inflammatory disease activity and retard accumulation of disability. Future challenges for treatment include identifying agents that will promote remyelination and axonal protection to help impact progressive forms of multiple sclerosis. This chapter discusses currently available disease modifying therapies, agents currently in phase 2/3 trials, and future directions in the treatment of multiple sclerosis.

Juvenile Dermatomyositis: A Clinical Overview

1990 ◽  
Vol 12 (4) ◽  
pp. 117-124
Author(s):  
Lauren M. Pachman
Keyword(s):  
Medical Care ◽  
Causal Relationship ◽  
Medical Therapy ◽  
Muscle Weakness ◽  
Genetic Factors ◽  
Juvenile Dermatomyositis ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Coxsackievirus B ◽  
Clinical Overview

The child who develops the symptoms of the specific rash, proximal muscle weakness, and fatigue should seek medical care promptly. With the advances in physical and medical therapy, many of the consequences of the disease can now be ameliorated. There are suggestive data that JDMS and PM may each have a different pathophysiology, but more evidence is needed. The next few years should be exciting as there is increased effort to determine if there is, in fact, a causal relationship between Coxsackievirus B or other enterovirus and genetic factors that alter the susceptibility to or severity of the course of the disease—JDMS.

Polymyositis and Dermatomyositis

2019 ◽  
pp. 187-189
Author(s):  
Josephina A. Vossen
Keyword(s):  
Muscle Biopsy ◽  
Clinical Examination ◽  
Immune Suppression ◽  
Imaging Modality ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Muscle Enzyme

Chapter 42 discusses polymyositis and dermatomyositis, which are idiopathic inflammatory myopathies characterized by muscle inflammation, proximal muscle weakness, and elevated muscle enzymes. Idiopathic inflammatory myopathies represent a heterogeneous group of muscle diseases. Diagnosis is based on clinical examination, muscle enzyme laboratory values, electromyography (EMG), and muscle biopsy. MRI is the most important imaging modality used in diagnosis and management. Radiographs and CT may detect calcinosis. Treatment is primarily by immune suppression.

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.

scholarly journals Case Report: Elevated CPK, an indicator of idiopathic inflammatory myopathy?

F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 164
Author(s):  
Hina N. Khan ◽  
Usman Jilani ◽  
Shitij Arora
Keyword(s):  
Case Report ◽  
Rare Disease ◽  
Treatment Response ◽  
Creatine Phosphokinase ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Current Treatment ◽  
Incidence Rates ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle

Polymyositis is a rare disease with incidence rates at about 1 per 100,000 people annually. In this case report we will review a case of proximal muscle weakness with an elevated creatine phosphokinase that was initially misdiagnosed twice as rhabdomyolysis. Therefore, emphasizing that idiopathic inflammatory myopathy is a potential cause of myasthenia that must be considered in the differential. The case will also describe the current treatment and treatment response in polymyositis.

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