Annals of the Rheumatic Diseases 2006;65:564-572
REVIEW
Diagnosis and management of adult onset Stills disease
1 Department of Medicine, Division of Allergy, Immunology, and Rheumatology, New Jersey Medical School, UMDNJ, Newark, NJ 07101, USA
2 Department of Medicine, Weill Medical College of Cornell University, NY, NY 10021, USA
Correspondence to:
Dr P Efthimiou
90 Bergen Street, DOC#4700, Newark, NJ 07103, USA; efthimpv{at}umdnj.edu
Background: Adult onset Stills disease (AOSD) is a rare systemic inflammatory disorder of unknown aetiology that is responsible for a significant proportion of cases of fever of unknown origin and can also have serious musculoskeletal sequelae.
Objective: To assess and synthesise the evidence for optimal diagnosis and management of AOSD.
Methods: The key terms, adult onset Stills disease, AOSD, adult Stills disease, ASD, Stills disease were used to search Medline (19662005) and PubMed (19662005) for all available articles in the English language. Clinically relevant articles were subsequently selected. Bibliographies, textbooks, and websites of recent rheumatology conferences were also assessed.
Results: Data on diagnosis and treatment of AOSD are limited in the medical literature and consist mainly of case reports, small series, and modest scale retrospective studies. Diagnosis is clinical and requires exclusion of infectious, neoplastic, and other autoimmune diseases. Laboratory tests are non-specific and reflect heightened immunological activity. Treatment comprises non-steroidal anti-inflammatory drugs, corticosteroids, immunosuppressive drugs (methotrexate, leflunomide, gold, azathioprine, ciclosporin A, cyclophosphamide), and intravenous gammaglobulin. The recent successful application of biological agents (anti-tumour necrosis factor, anti-interleukin (IL)1, anti-IL6), often in combination with traditional immunosuppressive drugs, has been very promising.
Conclusions: AOSD often poses a diagnostic and therapeutic challenge and clinical guidelines are lacking. The emergence of validated diagnostic criteria, discovery of better serological markers, and the application of new biological agents may all provide the clinician with significant tools for the diagnosis and management of this complex systemic disorder.
Abbreviations: ANA, antinuclear antibodies; AOSD, adult onset Stills disease; ASD, adult Stills disease; CRP, C reactive protein; ESR, erythrocyte sedimentation rate; IFN
, interferon
; IL, interleukin; JIA, juvenile idiopathic arthritis; IVIG, intravenous gammaglobulin; LFTs, liver function tests; MTX, methotrexate; NSAID, non-steroidal anti-inflammatory drug; RF, rheumatoid factor; TNF
, tumour necrosis factor
; TRAPS, TNF receptor associated periodic syndrome; WBC, white blood cell
Keywords: adult onset Stills disease; biological agents; anti-cytokine treatment
![]()
CiteULike
Complore
Connotea
Del.icio.us
Digg
Reddit
Technorati What's this?
This article has been cited by other articles:
-
Limsukon, A., Jones, H. D., Feinstein, J.
(2009). A 60-Year-Old Japanese Man With Fevers, Myalgias, Pharyngitis, and Right Knee Pain. Chest
136: 1428-1431
[Full Text] -
ZENG, T., ZOU, Y.-Q., WU, M.-F., YANG, C.-D.
(2009). Clinical Features and Prognosis of Adult-onset Still's Disease: 61 Cases from China. The Journal of Rheumatology
36: 1026-1031
[Abstract] [Full Text] -
LEE, S.-W., PARK, Y.-B., SONG, J.-S., LEE, S.-K.
(2009). The Mid-Range of the Adjusted Level of Ferritin Can Predict the Chronic Course in Patients with Adult Onset Still's Disease. The Journal of Rheumatology
36: 156-162
[Abstract] [Full Text] -
Limsukon, A., Jones, H. D., Feinstein, J.
(2008). A FULMINANT CASE OF AN ADULT ONSET STILL'S DISEASE WITH PULMONARY CAPILLARITIS AND PLEUROPERICARDITIS. Chest Meeting
134: c6001-c6001
[Abstract]
eLetters:
Read all eLetters
- Thank you
- Lia T. Umikashvili
- Ann Rheum Dis Online, 4 Jun 2007 [Full text]
Register for free content
The full back archive is now available for all BMJ Journals. Institutional subscribers may access the entire archive as part of their subscription. Personal subscribers will also have access to all content when logged in. Non-subscribers who register have free access to all articles published before 2006 right back to volume 1 issue 1. Register here to access the free archive of all BMJ Journals.
Don't forget to sign up for content alerts so you keep up to date with all the articles as they are published.
