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juvenile rheumatoid arthritis; juvenile idiopathic arthritis; Still's disease

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: 1) relative to adult RA - fever more intense (at least 39.0 C); fever spikes daily - transient (evanescent) rash - 2-5 mm erythematous papules appearing at height of fever - nodules rare - distribution: trunk & proximal extremities - 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 2) general - severe, debilitating, extra-articular features - occasionally fatal complications despite medical treatment - many children still experience early joint destruction, necessitating surgical replacement Laboratory: 1) complete blood count - leukocytosis > 10,000/uL, >= 80% granulocytes 2) erythrocyte sedimentation rate (ESR) > 100 mm/h 3) serum C-reactive protein > 2x upper limit of normal 4) liver function tests - serum ALT, serum AST, serum LDH generally elevated 5) serum ferritin elevated, > 1000 ug/L (as high as 6000 ug/L) 6) serology a) rheumatoid factor negative b) antinuclear antibody negative [2] Differential diagnosis: - infection - lymphoma - leukemia Management: 1) NSAIDs are 1st line therapy 2) corticosteroids may be helpful if refractory to NSAIDs 3) escalation of therapy as indicated [4] a) methotrexate b) TNF-alpha inhibitor c) anakinra d) see rheumatoid arthritis

Interactions

disease interactions

Related

adult-onset Still's disease

General

rheumatoid arthritis (RA)

Database Correlations

OMIM 604302

References

  1. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 867
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2018.
  3. OMIM :accession 604302
  4. 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 PMID: 21452260 http://www.rheumatology.org/practice/clinical/guidelines/ACR_2011_jia_full_manuscript.pdf
  5. 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: 16219707
  6. 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: 20506370
  7. Kontzias A, Efthimiou P. Adult-onset Still's disease: pathogenesis, clinical manifestations and therapeutic advances. Drugs. 2008;68(3):319-37. PMID: 18257609
  8. 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