In this study we have investigated the efficacy and safety of cyclosporin A (CyA) in a group of pediatric patients with juvenile chronic arthritis (JCA, 9 cases) and polymyositis-dermatomyositis (PM-DM, 3 cases). Of the 9 JCA patients, 7 had the systemic and 2 the polyarticular form of the disease. All of the patients received CyA after the failure of corticosteroids and/or cytotoxic drugs. CyA was administered for 9 to 48 months at a mean dose of 5 mg/kg/day. Ten patients received corticosteroids with CyA. The results of CyA treatment were satisfactory overall, both in the PM-DM patients and in the JCA patients, as assessed by clinical and laboratory evaluation. CyA-related side effects included alopecia, hypertension, hypertrichosis, tremors, and hirsutism. One JCA patient developed polyserositis with hypoproteinemia of unknown origin while receiving CyA. CyA blood levels did not correlate with clinical efficacy and/or side effects. This study suggests that CyA represents a promising agent for the treatment of JCA and childhood PM-DM.
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Clin Rheumatol
January 2025
Division of Pediatric Rheumatology and Nephrology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey.
Objective: Chronic non-bacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease associated with other chronic inflammatory diseases such as familial Mediterranean fever (FMF), juvenile idiopathic arthritis (JIA), spondylarthropathies, inflammatory bowel disease (IBD), and pyoderma gangrenosum. We aimed to describe the clinical and follow-up characteristics of patients with CNO and to compare findings between patients with and without comorbidities.
Methods: The clinical records of patients with CNO who were followed up in our pediatric rheumatology clinic between 2018 and 2023 were reviewed.
Turk J Pediatr
December 2024
Department of Pediatrics, Nilratan Sircar Medical College and Hospital, Kolkata, West Bengal, India.
Objectives: To evaluate the role of serum procalcitonin (PCT) as a diagnostic tool to differentiate bacterial sepsis from flare-ups during febrile episodes in children with known rheumatic disorders compared to other inflammatory markers like C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR).
Methods: Previously diagnosed patients with known rheumatic disorders presenting in emergency or outpatient departments with febrile episodes were included in the study. Blood samples were collected upon admission to test for signs of infection, including serum PCT levels with routine laboratory and radiological tests.
Acta Dermatovenerol Croat
November 2024
Prof. Marija Jelušić, MD, PhD, Department of Paediatrics, University of Zagreb, School of Medicine, Division of Clinical Immunology, Rheumatology and Allergology, Centre of Reference for Paediatric and Adolescent Rheumatology of Ministry of Health of the Republic Croatia, University Hospital Centre Zagreb, Kispaticeva 12, 10 000 Zagreb, Croatia;
Juvenile dermatomyositis with emphasized vasculopathy is rare, but the most severe form of the disease, with a poor prognosis with relapsing and chronic course or, in some cases, lethal outcome. We present a case of a 19-year-old Caucasian female, who developed severe acute juvenile dermatomyositis with emphasized multisystem vasculopathy, including retinal vasculopathy and maculopathy (cotton-wool spots, retinal hemorrhages, macular edema) at the age of 8. Due to no response to standard treatment protocols and rapid worsening of clinical symptoms and laboratory findings, a TNF inhibitor (infliximab) was introduced after the third week of treatment resulting in complete normalisation of muscle enzyme levels and complete resolution of eye changes within the next 2 weeks with a gradual general recovery.
View Article and Find Full Text PDFRheumatol Immunol Res
December 2024
Rheumatology, Rehabilitation, and Physical medicine department, Faculty of Medicine, Menoufia University, Shibin El Kom Egypt.
Background And Objectives: Juvenile Idiopathic Arthritis (JIA) and Rheumatoid arthritis (RA) are autoimmune chronic inflammatory disorders of undetermined cause. Uveitis is one of the commonest and most dangerous extra-articular manifestations of JIA and RA presenting chronic anterior uveitis with non-specific biomarkers for its early detection. We evaluated the role of serum 14-3-3 Eta protein to assess its potential role as a novel biomarker for the early detection of uveitis in Egyptian JIA and RA patients as well as its correlation with disease activity.
View Article and Find Full Text PDFJ Pediatr Psychol
December 2024
Cassie and Friends: A Society for Children with Juvenile Arthritis and Other Rheumatic Diseases, Vancouver, BC, Canada.
Objective: Youth with juvenile idiopathic arthritis (JIA) experience elevated rates of internalizing symptoms, although more research is required to understand this phenomenon. Perfectionism, a multidimensional personality trait that involves dimensions such as striving for flawlessness (self-oriented perfectionism) and feeling that others demand perfection (socially-prescribed perfectionism), is a well-known risk factor for internalizing symptoms that has received minimal attention in pediatric populations. Preregistered hypotheses explored the relationships between youth and parent perfectionism and symptoms of depression and anxiety in youth with JIA, as mediated by (a) youth/parent negative self-evaluations and (b) youth self-concealment.
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