adult-onset Still's disease

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Introduction

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

Clinical manifestations

Diagnostic criteria

* diagnosis requires >= 5 criteria with >= 2 major criteria[2]

* diagnosis is clinically based & requires exclusion of infection, malignancy or other rheumatologic disease[2]

Laboratory

Differential diagnosis

Management

More general terms

Additional terms

References

  1. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 867
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 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 https://mirror.omim.org/entry/604302
  4. 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
  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: https://www.ncbi.nlm.nih.gov/pubmed/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: https://www.ncbi.nlm.nih.gov/pubmed/20506370
  7. 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
  8. 8.0 8.1 8.2 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 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/26176384 <Internet> 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: https://www.ncbi.nlm.nih.gov/pubmed/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: https://www.ncbi.nlm.nih.gov/pubmed/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: https://www.ncbi.nlm.nih.gov/pubmed/30077425 Review.

Patient information

Adult-onset Still's disease patient information

Database