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Unlabelled: Polymyositis (PM) and dermatomyositis (DM) are chronic inflammatory muscle disorders characterized by muscle weakness and fatigue. They are histopathologically defined by inflammatory cell infiltrates in the skeletal muscle. In a recent meta-analysis, the incidence of cardiovascular complications in patients with PM/DM ranged from 9 to 72 %, with heart failure being the most commonly reported heart disease.

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Juvenile Dermatomyositis: Updates in Pathogenesis and Biomarkers, Current Treatment, and Emerging Targeted Therapies.

Paediatr Drugs

January 2025

National Institute of Arthritis Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.

Juvenile dermatomyositis is a rare systemic inflammatory autoimmune disease involving muscle, skin, and vessels. Most patients do not fully respond to initial therapy, instead having a chronic refractory or polycyclic disease course. Pathogenesis is not completely understood, but immune cell dysregulation, particularly of B cells, mitochondrial dysfunction, changes in neutrophils and neutrophil extracellular traps (NETs), and increased type I and type II interferon (IFN) signaling have been described.

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Introduction And Importance: Polymyositis is an inflammatory process, primarily affecting proximal muscles, characterized by elevated muscle enzymes and distinctive electromyography patterns.

Case Presentation: The authors present a case of a 33-year-old male patient experiencing complications of polymyositis, including pharyngeal and laryngeal involvement leading to dysphagia and dysphonia. Steroids and intravenous immunoglobulin (IVIG) therapy proved ineffective.

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Article Synopsis
  • Clinical amyopathic dermatomyositis is a skin condition that lacks muscle symptoms and is often associated with anti-melanoma differentiation-associated gene 5 antibodies, particularly in Japanese patients, leading to poor outcomes in those with lung complications.
  • A 52-year-old male presented with severe respiratory issues and skin rashes, diagnosed with dermatomyositis-related interstitial pneumonia, but treatment with steroids and immunosuppressants was largely ineffective.
  • Despite some initial improvement after switching medications, the patient ultimately faced worsening respiratory failure due to invasive pulmonary aspergillosis, resulting in a fatal outcome.
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Article Synopsis
  • MDA-5 antibody-positive dermatomyositis (MDA5-DM) often leads to rapidly progressive interstitial lung disease (RP-ILD) and can be complicated by cancer, though such instances are rare compared to other forms of dermatomyositis.
  • Traditional treatment involves addressing cancer first, but surgeries and chemotherapies can be risky due to respiratory issues.
  • A case study of a 48-year-old woman with MDA5-DM and bilateral breast cancer showed improvement with tofacitinib and plasma exchange therapy, ultimately allowing her to undergo surgery; she has remained cancer-free and symptom-free for three years since treatment.
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