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Autologous Hematopoietic Stem Cell Transplantation for Idiopathic Inflammatory Myositis
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Yu Oyama, Walter G. Barr, Richard K. Burt
Juvenile dermatomyositis (JDM) presents with skin rash, muscle weakness, myalgias and later, patients may develop vasculitis, joint contractures, calcinosis and oral and skin ulcers. Elevated ESR, CPK, LDH, ANA, myogenic pattern of EMG and histologic findings similar to adult PM/DM are seen. Patients generally respond to corticosteroids and immunosuppressive therapy better than adults, but there are some patients who need long-term corticosteroids or other immunosuppressive therapies due to chronic continuous disease activity. Severe disability or even deaths have been reported.36,37
Rheumatology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Clarissa Pilkington, Kiran Nistala, Helen Lachman, Paul Brogan
Juvenile dermatomyositis (JDM) affects 2–3 cases per million children per year. The mean age of onset is 7 years; 25% of cases occur before the age of 4 years. There is no association with malignancy (unlike adult cases).
Principles of Clinical Diagnosis
Published in Susan Bayliss Mallory, Alanna Bree, Peggy Chern, Illustrated Manual of Pediatric Dermatology, 2005
Susan Bayliss Mallory, Alanna Bree, Peggy Chern
Pachman LM, Hayford JR, Chung A, et al. Juvenile dermatomyositis at diagnosis: Clinical characteristics of 79 children. J Rheumatol 1998; 25: 1198–204 Pachman LM. Juvenile dermatomyositis: immunogenetics, pathophysiology, and disease expression. Rheum Dis N Amer 2002; 28: 579–602
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].
Fatal outcome of anti-MDA5 juvenile dermatomyositis in a paediatric COVID-19 patient: a case report
Published in Modern Rheumatology Case Reports, 2021
Cristian Quintana-Ortega, Agustín Remesal, Marta Ruiz de Valbuena, Olga de la Serna, María Laplaza-González, Elena Álvarez-Rojas, Clara Udaondo, Rosa Alcobendas, Sara Murias
An 11-year-old Spanish female presented with skin rash and dry cough and was admitted to a local hospital. Four months before admission, she noticed palmar erythema, periorbital rash and papular skin lesions on elbows and knees. In addition, the patient referred weight loss, wrist arthritis and generalised arthromyalgias without proximal muscle weakness. Gottron’s papules with calcinosis, heliotrope rash and painful palmar papules were found (Figures 1 and 2). Initial laboratory findings are summarised in Table 1. Immunology study revealed a positive ANA (tittle 1/160) and extended myositis antibody determination (Myositis 12 IgG DOT for BlueDiver Instrument, Alphadia, Wavre, Belgium) showed positive anti Ro/SS-A and anti-MDA5 antibodies. She was diagnosed with juvenile dermatomyositis. Respiratory function tests revealed a moderate restrictive ventilatory impairment. Spirometry data according to Global Lung Function Initiative (GLI) reference values were: forced expiratory volume in one second (FEV1) 1.06 L (44.24%, Z-score −4.45); forced vital capacity (FVC) 0.97 L (35.56%, Z-score −5.89); FEV1/FVC 109% (Z-score 5.52) [7]. Lung volume measurements using plethysmography were: total lung capacity (TLC) 1.60 L (45%); residual volume (RV) 0.65 L (71%); RV/TLC 40.25%.
Increased circulating Tfh17 and PD-1+Tfh cells are associated with autoantibodies in Hashimoto’s thyroiditis
Published in Autoimmunity, 2018
Jiwei Zhao, Yanxia Chen, Qing Zhao, Jie Shi, Wei Yang, Zhongliang Zhu, Wenge Yu, Jinju Guan, Yingxiang Song, Hui Wu, Weidong Jin, Yonglie Zhou, Jinlin Liu
Morita et al. [5] previously revealed that human blood CD4+CXCR5+ T cells can be divided into Tfh1, Tfh2, and Tfh17 cell subsets, among which only the Tfh2 and Tfh17 cell subsets can efficiently induce naïve B cells to produce immunoglobulins. Juvenile dermatomyositis, a systemic autoimmune disease, displayed a profound skewing with respect the percentages of Tfh2 and Tfh17 cells; this skewing was correlated with disease activity [5]. Therefore, we wondered whether Tfh2 and Tfh17 cells play a role in HT. We used CXCR3 and CCR6 to define Tfh1 (CXCR3+Tfh), Tfh2 (CXCR3−CCR6− Tfh), and Tfh17 (CCR6+ Tfh) cells, as previously reported [5], and analysed the percentages of these Tfh subsets in HT patients. Surprisingly, the percentages of Tfh1 and Tfh2 cells were significantly decreased, while the percentage of Tfh17 cells was significantly increased in HT patients compared with those in HCs (all p < .0001) (Figure 5(A,B)). Thus, Tfh17 cells might play a significant role in the pathogenesis of HT.