juvenile rheumatoid arthritis; juvenile idiopathic arthritis; Still's disease
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Introduction
Rheumatoid arthritis (RA) in children. An adult form of Still's disease is virtually identical to the childhood variety.
Epidemiology
- children; also adult-onset Still's disease
Genetics
- susceptibility associated with genetic variations in: IL6, MMIF
Clinical manifestations
- relative to adult RA
- fever more intense (at least 39.0 C); fever spikes daily
- transient (evanescent) rash
- arthritis, arthralgias
- recurrent pharyngitis
- pericarditis more common, pleuritis
- myalgias
- cardiac valvular disease more common
- lymphadenopathy common
- hepatosplenomegaly common
- RA interferes with bone growth
- underdevelopment of mandible
- general
- severe, debilitating, extra-articular features
- occasionally fatal complications despite medical treatment
- many children still experience early joint destruction, necessitating surgical replacement
Laboratory
- complete blood count
- leukocytosis > 10,000/uL, >= 80% granulocytes
- erythrocyte sedimentation rate (ESR) > 100 mm/h
- serum C-reactive protein > 2x upper limit of normal
- liver function tests
- serum ferritin elevated, > 1000 ug/L (as high as 6000 ug/L)
- serology
- rheumatoid factor negative
- antinuclear antibody negative[2]
Differential diagnosis
Management
- NSAIDs are 1st line therapy
- corticosteroids may be helpful if refractory to NSAIDs
- escalation of therapy as indicated[4]
More general terms
Additional terms
References
- ↑ DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 867
- ↑ 2.0 2.1 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2018.
- ↑ OMIM https://mirror.omim.org/entry/604302
- ↑ 4.0 4.1 Beukelman T et al 2011 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis: Initiation and Safety Monitoring of Therapeutic Agents for the Treatment of Arthritis and Systemic Features Arthritis Care & Research 2011, 63:465-482 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/21452260 <Internet> http://www.rheumatology.org/practice/clinical/guidelines/ACR_2011_jia_full_manuscript.pdf
- ↑ Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still's disease. Ann Rheum Dis. 2006 May;65(5):564-72. Epub 2005 Oct 11. PMID: https://www.ncbi.nlm.nih.gov/pubmed/16219707
- ↑ Franchini S, Dagna L, Salvo F et al Efficacy of traditional and biologic agents in different clinical phenotypes of adult-onset Still's disease. Arthritis Rheum. 2010 Aug;62(8):2530-5 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20506370
- ↑ Kontzias A, Efthimiou P. Adult-onset Still's disease: pathogenesis, clinical manifestations and therapeutic advances. Drugs. 2008;68(3):319-37. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18257609
- ↑ National Institute of Arthritis and Muscluloskeletal and Skin Diseases (NIAMS) Juvenile Rheumatoid Arthritis https://www.niams.nih.gov/health-topics/juvenile-arthritis
Ophthalmologic examinations in children with juvenile rheumatoid arthritis (American Academy of Pediatrics) http://aappolicy.aappublications.org/cgi/content/full/pediatrics;117/5/1843
Patient information
juvenile rheumatoid arthritis patient information