Pharmacological and non-pharmacological treatments for refractory paediatric Still's disease: a scoping review
These videos have been matched automatically. Contact us if they are not relevant.
These videos have been matched automatically. Contact us if they are not relevant.
View abstract on PubMed
Summary
This summary is machine-generated.Treatment options for refractory Still's disease, including macrophage activation syndrome, are emerging. New therapies like emapalumab and JAK inhibitors show promise, but more research is needed for these challenging pediatric cases.
Area Of Science
- Pediatric Rheumatology
- Immunology
- Systemic Juvenile Idiopathic Arthritis
Background
- Systemic juvenile idiopathic arthritis (sJIA), or Still's disease, has improved with IL-1 and IL-6 antagonists.
- A subset of patients develops refractory disease, presenting as persistent arthritis, macrophage activation syndrome (MAS), or interstitial lung disease.
Purpose Of The Study
- To conduct a scoping review summarizing evidence for treating refractory pediatric Still's disease.
- To identify and analyze treatment strategies for the distinct subtypes of refractory sJIA.
Main Methods
- Searched databases for studies on refractory Still's disease and its subtypes.
- Included controlled trials, uncontrolled trials, and case series/reports of patients with disease onset before age 16.
- Extracted data on study characteristics, survival, remission, corticosteroid reduction, and adverse effects.
Main Results
- Thirty articles met inclusion criteria; no controlled studies were found for all refractory subtypes.
- A pilot study showed positive results for emapalumab in refractory MAS.
- Combination biologics and Janus kinase (JAK) inhibitors demonstrated benefits for refractory arthritis; allogeneic stem cell transplantation was reported with significant risks.
Conclusions
- Various treatments like emapalumab, JAK inhibitors, rituximab, and combination biologics are reported for refractory Still's, but evidence is limited.
- Further research with consistent outcome reporting and innovative trial designs is crucial for these challenging patient subgroups.
These videos have been matched automatically. Contact us if they are not relevant.
These videos have been matched automatically. Contact us if they are not relevant.
Related Concept Videos
In pediatric medicine, understanding the renal function and drug elimination nuances is crucial for administering safe and effective treatments. Newborns, in particular, display markedly slower renal functions than adults, profoundly affecting how drugs are cleared from their bodies. This slower drug clearance requires clinicians to extend the dosing intervals for many medications to prevent drug accumulation and toxicity while ensuring therapeutic efficacy.One key area where these adjustments...
Understanding the physiological differences in the pediatric population is crucial for effective pharmacotherapy. Neonates, infants, and children exhibit significant variations in gastric pH, gastric emptying time, intestinal transit time, and biliary function. These variations profoundly affect oral drug absorption, necessitating a nuanced approach to pediatric dosing.Neonates present with a unique physiological profile, having a gastric pH greater than 4 and faster and more irregular gastric...
In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses...
Antipsychotic drugs are a crucial treatment method for acute and chronic psychoses, bipolar illness, and behavioral disorders. The selection of these drugs depends on several factors, including the state of the disease, clinical judgment, possible drug interactions, and the patient's sensitivity to adverse effects. In immediate scenarios, such as delirium and dementia, short-term treatment with low doses of high-potency typical or atypical agents can effectively manage symptom exacerbation.
Upon diagnosis, managing Inflammatory Bowel Disease (IBD) involves addressing several crucial aspects. The primary goals include resting the bowel, correcting malnutrition, and providing symptomatic relief. Resting the bowel may consist of medications to reduce inflammation and promote healing. Correcting malnutrition is essential, often requiring dietary adjustments and nutritional supplements. Symptomatic relief aims to ease pain, diarrhea, and other discomforts in IBD.
Pharmacologic...
The advent of drug therapy has profoundly shaped modern mental health care, providing targeted treatments for a range of psychological disorders. Psychotherapeutic drugs, classified into antianxiety, antidepressant, and antipsychotic medications, address symptoms across anxiety disorders, mood disorders, and schizophrenia. While these medications have transformed patient outcomes, they require careful management due to their potential side effects and limitations.
Antianxiety Medications