Pediatrics – Rheumatology
EULAR/PReS Guidelines for the Diagnosis and Management of Still’s Disease
20 Oct, 2024 | 17:41h | UTCIntroduction:
Still’s disease, encompassing systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD), is a systemic inflammatory disorder characterized by spiking fevers, rash, arthralgia or arthritis, and elevated inflammatory markers. Historically treated as separate entities, sJIA and AOSD are now recognized as the same disease continuum. To unify and optimize diagnosis and management across all ages, the European Alliance of Associations for Rheumatology (EULAR) and the Paediatric Rheumatology European Society (PReS) have developed comprehensive, evidence-based recommendations.
Key Recommendations:
- Unified Terminology: sJIA and AOSD are the same disease and should be collectively termed “Still’s disease” to standardize diagnosis and treatment. (Recommendation: strong)
- Rapid Diagnosis Using Operational Definitions: Key clinical features include spiking fever ≥39°C lasting ≥7 days, transient rash coinciding with fever spikes, arthralgia or arthritis, and elevated inflammatory markers (CRP, ESR, neutrophils, ferritin). Arthritis is supportive but not essential for diagnosis. (Strong)
- Diagnostic Biomarkers: Elevated serum interleukin-18 (IL-18) and S100 proteins strongly support the diagnosis and should be measured when available. (Moderate)
- Exclude Alternative Diagnoses: Carefully consider infections, malignancies, other immune-mediated inflammatory diseases, and monogenic autoinflammatory disorders to avoid misdiagnosis. (Strong)
- Treatment Goals: The ultimate goal is drug-free remission, defined as clinically inactive disease (CID) maintained for at least 6 months. CID entails absence of disease-related symptoms and normalization of ESR and CRP. Intermediate targets at specific time points guide treatment adjustments. (Strong)
- Early Use of IL-1 and IL-6 Inhibitors: To avoid prolonged glucocorticoid use, prioritize early initiation of interleukin-1 (IL-1) or interleukin-6 (IL-6) inhibitors upon diagnosis. (Strong)
- Glucocorticoid Tapering: Aim to achieve CID without glucocorticoids within 6 months. Maintain CID for 3–6 months before initiating tapering of biologic DMARDs. (Strong)
- Monitor for Complications: Be vigilant for life-threatening complications, including macrophage activation syndrome (MAS) and lung disease (LD), which require prompt recognition and management. (Strong)
- MAS Management: Consider MAS in patients with persistent fever, splenomegaly, elevated ferritin, cytopenias, abnormal liver function tests, coagulopathy, and hypertriglyceridemia. High-dose glucocorticoids are essential; anakinra, ciclosporin, and interferon-γ inhibitors may be added. (Strong)
- LD Screening and Management: Screen patients for LD via clinical assessment and pulmonary function tests; high-resolution CT scans are indicated for symptomatic patients. The presence of LD is not a contraindication for IL-1 or IL-6 inhibitors. (Strong)
Conclusion: Implementing these unified, evidence-based recommendations is expected to improve the diagnosis and management of Still’s disease across all ages, leading to earlier intervention, optimized treatment strategies, reduced complications, and enhanced patient outcomes, including achieving drug-free remission.
RCT | Baricitinib demonstrates efficacy in treatment-resistant juvenile idiopathic arthritis
2 Aug, 2023 | 13:45h | UTCBaricitinib in juvenile idiopathic arthritis: an international, phase 3, randomised, double-blind, placebo-controlled, withdrawal, efficacy, and safety trial – The Lancet (link to abstract – $ for full-text)
Commentary on Twitter
NEW in @TheLancet—Baricitinib was efficacious with an acceptable safety profile in patients with #juvenile idiopathic #arthritis in a phase 3 withdrawal trialhttps://t.co/vZyXUwBiDZ #JIA
Plus linked Comment: https://t.co/VWofLvL427 pic.twitter.com/MGVoUz4xIv
— The Lancet Rheumatology (@TheLancetRheum) July 10, 2023
Consensus Paper | Methotrexate for inflammatory skin disease in pediatric patients
7 Jul, 2023 | 16:16h | UTC
Consensus Statement | Evaluation and management of deficiency of adenosine deaminase 2
30 Jun, 2023 | 14:53h | UTCInvited Commentary: Finding a Quorum in Deficiency of Adenosine Deaminase 2 Management – JAMA Network Open
Podcast | Childhood-Onset SLE
6 Apr, 2023 | 13:12h | UTC#77: Childhood-Onset SLE – Is It Sometimes Lupus? – The Cribsiders
RCT | Hydroxychloroquine in children with proliferative lupus nephritis
30 Mar, 2023 | 14:11h | UTC
RCT | Genicular nerve block vs. intra-articular triamcinolone in juvenile idiopathic arthritis
10 Feb, 2023 | 13:36h | UTC
RCT | Methylprednisolone vs. immunoglobulins equally effective in PIMS temporally associated with SARS-CoV-2
8 Feb, 2023 | 12:42h | UTC
SR | Intravenous immunoglobulin for the treatment of Kawasaki disease
7 Feb, 2023 | 13:58h | UTCIntravenous immunoglobulin for the treatment of Kawasaki disease – Cochrane Library
Summary: Immunoglobulin treatment for Kawasaki disease – Cochrane Library
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Systematic Review: Corticosteroids for the treatment of Kawasaki disease in children.
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7 Feb, 2023 | 13:56h | UTCRelated:
Intravenous immunoglobulin for the treatment of Kawasaki disease – Cochrane Library
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2ry analysis of a RCT | Vitamin D supplementation did not influence growth, body composition, and pubertal development in children.
30 Nov, 2022 | 13:49h | UTCInfluence of Vitamin D Supplementation on Growth, Body Composition, and Pubertal Development Among School-aged Children in an Area With a High Prevalence of Vitamin D Deficiency: A Randomized Clinical Trial – JAMA Pediatrics (free for a limited period)
Commentary: Vitamin D Supplementation Does Not Influence Growth in Children – HealthDay
Recommendations for the screening, monitoring, and treatment of juvenile idiopathic arthritis-associated uveitis.
3 Nov, 2022 | 14:01h | UTC
RCT | Secukinumab in enthesitis-related arthritis and juvenile psoriatic arthritis.
15 Aug, 2022 | 12:05h | UTC
EULAR/PRES recommendations for vaccination of pediatric patients with autoimmune inflammatory rheumatic diseases.
24 Jun, 2022 | 11:17h | UTC
EULAR/ACR points to consider for diagnosis, management and monitoring of the interleukin-1 mediated autoinflammatory diseases.
24 Jun, 2022 | 11:23h | UTC
Consensus Paper: Diagnosis and treat-to-target management of macrophage activation syndrome in children.
24 Jun, 2022 | 11:13h | UTC
Cohort Study: Association of maternal autoimmune diseases with risk of mental disorders in offspring in Denmark.
20 Apr, 2022 | 09:19h | UTC
Commentary on Twitter
https://twitter.com/JAMANetworkOpen/status/1515057370715594753
RCT: Long-term efficacy and safety of up to 108 weeks of Ixekizumab in pediatric patients with moderate to severe plaque psoriasis.
18 Apr, 2022 | 09:49h | UTC
Commentary on Twitter
In this RCT, by 108 weeks of use of ixekizumab for pediatric psoriasis, PASI 75/90/100 were achieved in 92%/79%/55%, respectively, of children, with clearance of nails in 68%, scalp in 76%, genital lesions in 88%, and palm/sole involvement in 90%. https://t.co/vk73Z3MKi4
— JAMA Dermatology (@JAMADerm) April 13, 2022
Cohort Study: Predictors of severe illness in children with multisystem inflammatory syndrome after SARS-CoV-2 infection.
12 Apr, 2022 | 09:57h | UTCCommentary: Older Kids More Vulnerable to MIS-C: Study – HealthDay
Acute phase of Kawasaki disease: a review of national guideline recommendations.
12 Apr, 2022 | 08:32h | UTC
Systematic Review: Epidemiology of musculoskeletal manifestations in pediatric inflammatory bowel disease.
7 Apr, 2022 | 07:55h | UTC
BSR guideline on management of pediatric, adolescent and adult patients with idiopathic inflammatory myopathy.
4 Apr, 2022 | 01:05h | UTC
Pharmacologic interventions for Kawasaki disease in children: A network meta-analysis of 56 randomized controlled trials.
1 Apr, 2022 | 08:45h | UTCCommentary: Network meta-analysis rates treatments for Kawasaki disease – medwire News
Guideline: Treatment of unspecific back pain in children and adolescents.
29 Mar, 2022 | 09:52h | UTC
ACR/Vasculitis Foundation Guideline for the management of Kawasaki Disease.
9 Mar, 2022 | 10:01h | UTC