Autologous Stem Cell Transplantation for Refractory Juvenile Idiopathic Arthritis (JIA)
Richard K. Burt, Alberto M. Marmont in Stem Cell Therapy for Autoimmune Disease, 2019
A growing number of functional outcome scales have become available for paediatric rheumatology research. Instruments to measure health status and function specifically developed for juvenile arthritis include the Childhood Arthritis Impact Measurement Scales (CHAIMS),18 the Childhood Health Assessment Questionnaire (CHAQ),19 the Juvenile Arthritis Functional Assessment Report (JAFAR),20 the Childhood Arthritis Health Profile (CAHP),21 the Juvenile Arthritis Quality of Life Questionnaire (JAQQ),22 and the Juvenile Arthritis Self Report Index (JASI).23 Of these, the instrument tested most widely is the CHAQ. For evaluation after HSCT, we used the core set of outcome variables for clinical trials in childhood arthritis as proposed by Giannini and the Pediatric Rheumatology International Trials Organisation group (PRINTO)24-27 (Table 3). PRINTO rheumatological outcome measures include a physician’s global assessment of disease activity, the child health assessment questionnaire, CHAQ (a parent/patient assessment of overall well-being, the functional ability and disease severity) (Table 4), the number of joints with active arthritis (Fuchs Swelling Index, FSI), the number of joints with limited range of motion, (EPM-ROM) (Table 5) and the erythrocyte sedimentation rate (ESR). Giannini proposed a 30% improvement from baseline of 3 out of 6 variables, with no more than one of the remaining variables worsening more than 30%.24 The evolution of the disease was followed at 3 month intervals.
Micronutrients in Prevention and Improvement of the Standard Therapy in Arthritis
Kedar N. Prasad in Micronutrients in Health and Disease, 2019
JRA is the most common childhood arthritis. This is considered an autoimmune disease in which immune cells attack healthy cells of the joint causing inflammatory reactions that release toxic chemical species, which cause progressive damage to the joints and their surrounding tissues causing redness, swelling, pain, and stiffness. Some children with JRA outgrow the illness. As a matter of fact, the symptoms of JRA disappear in over about 50% of affected children. JRA may cause chronic fever and anemia and can affect the heart, lungs, eyes, and nervous system. These arthritic episodes may last for several weeks and then may recur with less severe symptoms. This disease can impair bone development and weaken fine motor skills. The incidence of JRA in girls is higher than in boys. Transient JRA may follow certain infections.
Principles of Clinical Diagnosis
Susan Bayliss Mallory, Alanna Bree, Peggy Chern in Illustrated Manual of Pediatric Dermatology, 2005
Psoriatic arthritis Develops in 1% of children with psoriasis; represents 8–20% of all childhood arthritis; peak age of onset is 9–12 years old50% will have only one joint involved at initial presentationSubgroups:
Initiating disease-modifying anti-rheumatic drugs rapidly reduces purchases of analgesic drugs in juvenile idiopathic arthritis
Published in Scandinavian Journal of Rheumatology, 2021
K Rebane, K Aalto, M Haanpää, K Puolakka, LJ Virta, H Kautiainen, H Pohjankoski
In JIA, glucocorticoids are used either as intra-articular injections or systemically, and they may provide rapid relief from inflammatory pain (13). In the present study, we have information only about oral glucocorticoids, because intra-articular injections are given in hospitals or medical centres and are not reimbursed and registered by the SII. About one-quarter of the patients used oral glucocorticoids during the first 3 months post-index, which is a rather small proportion. Results from the UK Childhood Arthritis Prospective Study (CAPS) of 1051 patients with new-onset JIA reveal that 86% received glucocorticoids over the initial 3 years (29). Tiller et al (24) found that approximately half of Australian children with newly diagnosed JIA were treated with oral glucocorticoids during the first year, and intra-articular glucocorticoids were given to 62%. A long-term Nordic study (including Finland) reported that 74% received intra-articular glucocorticoids in the course of 8 years (30).
Current status and prospects of IL-6–targeting therapy
Published in Expert Review of Clinical Pharmacology, 2022
Masashi Narazaki, Tadamitsu Kishimoto
After the efficacy of IL-6 inhibitory therapy was established for Castleman disease and RA, IL-6 inhibitory therapy was attempted for some refractory inflammatory diseases. sJIA is a chronic childhood arthritis with symptoms including fever, skin rash, pericarditis, hepatosplenomegaly, and growth retardation. It is often treated with glucocorticoids, but the long-term use of glucocorticoids can potentially cause growth impairment. IL-6 levels are correlated with the severity of disease manifestation of sJIA. A randomized phase 3 trial of TCZ in children with refractory sJIA showed drastic improvement in disease activity [95]. TCZ also restored body growth and improved bone homeostasis [96,97]. TCZ was approved for sJIA treatment in 2008 in Japan and in 2011 in the EU and the United States.
Adalimumab in the treatment of pediatric patients with chronic noninfectious anterior uveitis
Published in Expert Review of Ophthalmology, 2021
Jeanne Gunzinger, Phoebe Moore, Athimalaipet Ramanan, Andrew Dick
Clinicians aim to distinguish between drug-induced remission and disease remission to assess the risk of disease flare on cessation of therapy. Ability to make this distinction would allow clinicians to tailor therapies to individual patient response and more safely predict ability to tolerate treatment cessation. Measurement of serum biomarkers that indicate underlying disease activity (including CD163 MRP8, MRP14, S100 proteins, IL‐18, and IL-6) is gaining traction in the management of systemic JIA [69–72]. While not currently utilized in ophthalmology, this area of research may prove beneficial in tailoring treatment to individual patient needs. To achieve this, the CLUSTER Consortium (including industry) is a multidisciplinary group interrogating childhood arthritis, JIA-uveitis through trial outcome data, multi-omic analysis of peripheral immune profiles and bioinformatics [73]. CLUSTER’s aim is to discover, replicate, and validate biomarkers to predict response to treatment, to define novel therapeutic targets, and to discover disease and treatment response measures to enable stratification of patients with childhood arthritis and JIA-U to guide treatment.
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