We report the clinical course of three patients with refractory juvenile dermatomyositis (JDM) who were treated with tacrolimus. All three children had extensive skin disease and severe muscle weakness and were corticosteroid dependent. All three patients showed impressive improvement of mainly the cutaneous lesions. Furthermore, overall disease activity decreased, all children became more physically active, and corticosteroid treatment could be tapered. However, none of the patients showed recovery of muscle strength, which was most likely due to irreversible muscle damage related to the long-standing myositis and/or high-dose steroid treatment. Patients were followed up for 7 to 9 months after the introduction of tacrolimus. No adverse effects were seen. These cases demonstrate that tacrolimus has beneficial effects in children with refractory JDM, especially in those with severe cutaneous manifestations.
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http://dx.doi.org/10.1007/s10067-008-0973-2 | DOI Listing |
Indian J Pediatr
December 2024
Department of Pediatrics, Dr. D.Y. Patil Medical College, Hospital and Research Center, Pune, Maharashtra, India.
Lancet Rheumatol
December 2024
Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA; Pharming Healthcare, Warren, NJ, USA. Electronic address:
Background: Systemic juvenile idiopathic arthritis-related lung disease (sJIA-LD) is a severe complication in patients with treatment-refractory systemic juvenile idiopathic arthritis (sJIA). The objective of this study was to evaluate the effect of allogeneic haematopoietic stem-cell transplantation (HSCT) in a cohort of children with sJIA-LD.
Methods: This international, retrospective cohort study was performed in nine hospitals across the USA and Europe in children with sJIA-LD who had received allogeneic HSCT.
Exp Neurol
December 2024
Neuroscience Department, U.S. Army Medical Research Institute of Chemical Defense (USAMRICD), Aberdeen Proving Ground, MD, United States of America. Electronic address:
Exposure to organophosphorus nerve agents irreversibly inhibits acetylcholinesterase and may lead to cholinergic crisis and seizures. Although benzodiazepines are the standard of care after nerve agent-induced status epilepticus, when treatment is delayed for up to 30 min or more, refractory status epilepticus can develop. Adult male rodents are often utilized for evaluation of therapeutic efficacy against nerve agent exposure.
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November 2024
Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, China.
Background: Currently, no established integrated treatment regimen exists for anti-melanoma differentiation-associated gene 5 (anti-MDA5)-positive juvenile dermatomyositis (JDM) complicated by rapidly progressive interstitial lung disease (RP-ILD). We present a case of refractory anti-MDA5-positive JDM with RP-ILD that was successfully treated using a combination of tocilizumab and plasma exchange, along with a review of the relevant literature.
Methods: A literature review was conducted to gain insights into the clinical features and treatment strategies for managing refractory anti-MDA5-positive JDM complicated by RP-ILD.
Front Pediatr
November 2024
Department of Pediatrics, Golisano Children's Hospital, University of Rochester, Rochester, NY, United States.
Juvenile dermatomyositis is a systemic autoimmune disease characterized by progressive proximal muscle weakness, pathognomonic rashes, and often the presence of myositis-specific antibodies. Consensus treatment plans for pediatric patients with juvenile dermatomyositis recommend steroids and methotrexate as initial therapy. Patients with anti-transcription intermediary factor 1 gamma (anti-TIF-1γ) antibodies tend to have more refractory disease requiring more aggressive treatment with intravenous immunoglobulin, which is typically well tolerated.
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