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Autoimmune conditions
Published in Ibrahim Natalwala, Ammar Natalwala, E Glucksman, MCQs in Neurology and Neurosurgery for Medical Students, 2022
Ibrahim Natalwala, Ammar Natalwala, E Glucksman
Which of the following statements regarding autoimmune inflammatory myopathies such as polymyositis and dermatomyositis are true and which are false? Muscle weakness is characteristically distal and symmetrical with most people having an element of myalgia.Muscle biopsies containing cytotoxic T-cells encircling non-necrotic myofibres are pathognomic for polymyositis.A heliotrope rash is specifically related to inclusion body myositis.Autoimmune myopathies are associated with increased risk of cancer.Dermatomyositis responds well to steroid therapy.
Selected topics
Published in Henry J. Woodford, Essential Geriatrics, 2022
Polymyositis causes a slowly progressive, symmetrical, proximal weakness. It is usually painless. It may be associated with dysphagia, polyarthritis or arthralgia. There can also be cardiopulmonary involvement causing pneumonitis, conduction defects, pericarditis or myocarditis. A number of people will have another underlying condition. Around 20% have a connective tissue disease (e.g. systemic lupus erythematosus or rheumatoid arthritis). It is more common in women than men. Dermatomyositis causes a rash in addition to muscular symptoms and has a more frequent association with malignancy. Gottron's papules are areas of macular erythematous scaling over the knuckles and extensor surfaces of the knees and elbows. It can also cause a heliotrope periorbital rash, or less commonly macular erythema over the anterior chest or shoulders. It may be associated with malignancy, especially in older people, most commonly ovarian, gastrointestinal, lung or breast cancer.69
Tumors of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Polymyositis/dermatomyositis: treatment of the underlying malignancy and immunosuppressive therapy with corticosteroids, azathioprine, cyclophosphamide, methotrexate, and other potent immunosuppressive agents is usually effective in controlling the disease.
Patients with dermatomyositis shared partially similar transcriptome signature with COVID-19 infection
Published in Autoimmunity, 2023
Yiying Yang, Jie Song, Hongjun Zhao, Huali Zhang, Muyao Guo
Dermatomyositis is an inflammatory autoimmune disease, affecting skin, muscle, and blood vessels. The pathogenesis is complicated, involved in multiple factors, including genetic, epigenetic, and environmental factors [28]. This study was expected to find key genes and signal pathway in the skin of DM. A total of 380 DEGs of GSE46239 were identified, consisting of 271 up-regulated genes and 109 down-regulated genes. And we found that the skin of DM patients and the skin of COVID-19 patients shared a common transcriptional profile. Then, we used R package cluster Profiler to perform GO and KEGG analysis for the DEGs of GSE46239. A PPI network of DEGs in GSE46239 was constructed and 173 genes and 829 edges, and two significant modules were chosen from the PPI network. Next, 10 hub genes were identified by using plug-in cytoHubba, namely MX1, ISG15, IFIT3, IFIT1, RSAD2, IFIT2, IFI6, XAF1, IRF9, MX2. Finally, we verified these genes in the muscle and skin tissue of patients with DM.
Rescue Treatment with Infliximab for a Bilateral, Severe, Sight Threatening Frosted Branch Angiitis Associated with Concomitant Acute Onset of Presumed Dermatomyositis
Published in Ocular Immunology and Inflammation, 2023
Piergiorgio Neri, Shaikha Aljneibi, Francesco Pichi
Dermatomyositis is a systemic disease presenting a multiorgan involvement and affecting prevalently skin and muscles. The onset of such a condition might harm the integrity of multiple structures, including the eyes. The eye involvement is mainly represented by cotton wool spots and retinal hemorrhages,1–3 albeit more recently frosted branch angiitis has been described.4 A vitreous involvement might also be observed. If not promptly treated, the visual outcome might be very poor with a permanent impairment of patient’s best corrected visual acuity (BCVA). In this report, we describe a case of severe frosted branch angiitis associated with acute onset dermatomyositis, unresponsive to traditional immunesuppressive therapy, which rapidly responded to intravenous infliximab.
Evaluating the use of JAK inhibitors in inflammatory connective tissue diseases in pediatric patients: an update
Published in Expert Review of Clinical Immunology, 2022
Jane Chuprin, Lindsay McCormack, Jillian M. Richmond, Mehdi Rashighi
Juvenile Dermatomyositis (JDM) is a rare, autoimmune inflammatory disease, with an annual incidence of 2–3 per one million children in the United Kingdom and 3.2 per one million children in the United States [14–16]. The disease may be multisystemic, with potential involvement of the skin (Figures 2A,B), striated muscles, lungs, gastrointestinal tract, and other organs [17,18]. While treatments for JDM have improved over recent decades, high-dose steroids and steroid-sparing immunomodulating agents continue to be mainstay therapies. As Ravelli et al. note, 40% of patients with JDM experience disease that is refractory to steroids and/or disease-modifying antirheumatic drugs (DMARDs), with long-term potential consequences of cutaneous scarring, lipodystrophy, muscle dysfunction, severe calcinosis, and contractures [18,19]. Similar to the pathogenesis of dermatomyositis in adult patients, Type I interferon (IFN) signaling has recently been implicated in the pathogenesis of JDM in pediatric patients [20–22]. Smaller studies and case reports have described positive outcomes for chronic dermatomyositis in adults following therapy with tofacitinib and ruxolitinib [23–28]. JAKi have now been described in several case series and reports regarding children with JDM [20].