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adult-onset Still's disease

Adult form of Still's disease is virtually identical to the childhood variety, except that the skeleton (including joints) are fully developed. Epidemiology: - adult form: young adults; more common in females Genetics: - susceptibility associated with genetic variations in: IL6, MMIF Clinical manifestations: 1) relative to adult RA a) fever more intense (at least 39.0 C); fever spikes daily - returns to subnormal temperature [2] b) transient (evanescent) pink rash - non-pruritic, salmon-colored maculopapular rash - 2-5 mm erythematous papules appearing at height of fever - cutaneous nodules rare - distribution: trunk & proximal extremities - rash may resolve with resolution of fever c) non-erosive inflammatory arthritis, migratory arthritis, arthralgias [2] d) recurrent pharyngitis e) serositis, pericarditis more common, pleuritis f) myalgias g) fatigue, malaise h) cardiac valvular disease more common i) lymphadenopathy common j) hepatosplenomegaly common 2) general a) severe, debilitating, extra-articular features b) occasionally fatal complications despite medical treatment Diagnostic criteria: 1) major criteria a) daily spiking temperatures >= 39.0 C (99%) - quotidian fever in which temperature spikes once daily, then returns to subnormal temperature [2] b) arthragia/arthritis > 2 weeks (85%) c) non-pruritic, evanescent salmon-colored maculopapular rash on trunk or extremities (85%) d) leukocytosis > 10,000/uL, >= 80% granulocytes (90%) 2) minor criteria a) sore throat, pharyngitis (66%) b) lymphadenopathy &/or splenomegaly (65%/50%) c) increased serum AST, serum ALT or serum LDH (70%) d) antinuclear antibody & rheumatoid factor negative * diagnosis requires >= 5 criteria with >= 2 major criteria [2] * diagnosis is clinically based & requires exclusion of infection, malignancy or other rheumatologic disease [2] 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 12000 ug/L) - marker of macrophage activation 6) serology a) rheumatoid factor negative b) antinuclear antibody negative [2] c) antistreptolysin O negative d) anti-DNase B antibody negative [8] 7) urinalysis: proteinuria Differential diagnosis: - infection - lymphoma - leukemia - vasculitis - systemic lupus erythematosis - symmetric arthritis - fixed vs evanescent (short duration) skin rash - antinuclear antibody positive (almost always) - rheumatoid arthritis: no high fever or rash - ANCA-associated vasculitis: purpuric rash - cryoglobulinemia: purpuric rash 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 - anakinra plus glucocorticoid taper [8] d) canakinumab [2] e) see rheumatoid arthritis

Interactions

disease interactions

Related

juvenile rheumatoid arthritis; juvenile idiopathic arthritis; 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) 11,15,16,17,18. American College of Physicians, Philadelphia 1998,2009,2012,2015,2018. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  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. Hunt DP, Scheske JA, Dudzinski DM, Arvikar SL. CASE RECORDS of the MASSACHUSETTS GENERAL HOSPITAL. Case 22-2015. A 20-Year-Old Man with Sore Throat, Fever, Myalgias, and a Pericardial Effusion. N Engl J Med. 2015 Jul 16;373(3):263-71 PMID: 26176384 http://www.nejm.org/doi/full/10.1056/NEJMcpc1501310
  9. Pouchot J, Arlet JB. Biological treatment in adult-onset Still's disease. Best Pract Res Clin Rheumatol. 2012 Aug;26(4):477-87 PMID: 23040362
  10. Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Seve P. Adult-onset Still's disease. Autoimmun Rev. 2014 Jul;13(7):708-22. Review. PMID: 24657513 Free Article
  11. NEJM Knowledge+ Rheumatology
  12. Giacomelli R, Ruscitti P, Shoenfeld Y A comprehensive review on adult onset Still's disease. J Autoimmun. 2018 Sep;93:24-36 PMID: 30077425 Review.