Adult Onset Still’s Disease in a Patient with Fever of Unknown Origin: A Rare Case
Abstract
Adult onset Still’s disease (AOSD) is a rare clinical entity with unknown etiology, characterized by arthritis, fever, evanescent rash, and other systemic presentations. AOSD generally does not overlap with other rheumatic diseases. AOSD is a diagnosis by exclusion, and it is one of the common causes of pyrexia of unknown origin (PUO) which can be life-threatening if mistreated. Although steroids are the first line of therapy, about 20%-30% of patients are refractory, intolerant, and or relapse during tapering or upon discontinuation of steroids. There are no clinical guidelines in treating such patients, which is challenging.
Keywords: Still’s disease, arthritis, fever, auto-inflammatory
Keywords:
Still’s disease, arthritis, fever, auto-inflammatoryDOI
https://doi.org/10.22270/jddt.v14i1.6201References
Bywaters EG. Still's disease in the adult. Annals of the rheumatic diseases. 1971;30(2):121. https://doi.org/10.1136/ard.30.2.121 PMid:5315135 PMCid:PMC1005739
POUCHOT J, SAMPALIS JS, BEAUDET F, CARETTE S, DÉCARY F, SALUSINSKY-STERNBACH MA, HILL RO, GUTKOWSKI A, HARTH M, MYHAL D, SENÉCAL JL. Adult Still's disease: manifestations, disease course, and outcome in 62 patients. Medicine. 1991;70(2):118-36. https://doi.org/10.1097/00005792-199103000-00004 PMid:2005777
Castañeda S, Blanco R, González-Gay MA. Adult-onset Still's disease: advances in the treatment. Best Practice & Research Clinical Rheumatology. 2016;30(2):222-38. https://doi.org/10.1016/j.berh.2016.08.003 PMid:27886796
Kurasawa M, Kotani K, Kurasawa G, Shida K, Yamada S, Tago T. Adult-onset Still's disease in a patient over 80 years old successfully treated with low-dose methotrexate therapy. Age and ageing. 2007;36(1):104-6. https://doi.org/10.1093/ageing/afl128 PMid:17158115
Bywaters EG. Still's disease in the adult. Annals of the rheumatic diseases. 1971;30(2):121. https://doi.org/10.1136/ard.30.2.121 PMid:5315135 PMCid:PMC1005739
Zeng T, Zou YQ, Wu MF, Yang CD. Clinical features and prognosis of adult-onset Still's disease: 61 cases from China. The Journal of rheumatology. 2009 ;36(5):1026-31. https://doi.org/10.3899/jrheum.080365 PMid:19273456
Kokkinos A, Iliopoulos A, Greka P et al: Successful treatment of refractory adult-onset Still's disease with infliximab. A prospective, non-comparative series of four patients. Clin Rheumatol, 2004; 23(1): 45-49. https://doi.org/10.1007/s10067-003-0775-5 PMid:14749983
Naniwa T, Tamechika S, Iwagaitsu S, Maeda S, Togawa H. Successful use of higher-dose etanercept for multirefractory systemic flare of adult-onset Still's disease with liver failure with no response to tocilizumab therapy. Case Reports in Rheumatology.;2013. https://doi.org/10.1155/2013/923497 PMid:24455384 PMCid:PMC3877607
Bywaters EG. Still's disease in the adult. Annals of the rheumatic diseases. 1971;30(2):121. https://doi.org/10.1136/ard.30.2.121 PMid:5315135 PMCid:PMC1005739
Castañeda S, Blanco R, González-Gay MA. Adult-onset Still's disease: advances in the treatment. Best Practice & Research Clinical Rheumatology. 2016;30(2):222-38. https://doi.org/10.1016/j.berh.2016.08.003 PMid:27886796
Kaneko Y, Kameda H, Ikeda K, Ishii T, Murakami K, Takamatsu H, Tanaka Y, Abe T, Takeuchi T. Tocilizumab in patients with adult-onset still's disease refractory to glucocorticoid treatment: a randomised, double-blind, placebo-controlled phase III trial. Annals of the rheumatic diseases. 2018;77(12):1720-9. https://doi.org/10.1136/annrheumdis-2018-213920 PMid:30279267
Kelly J, Chowienczyk P, Gibson T. Sore throat and hyperferritinaemia. Journal of the Royal Society of Medicine. 2001;94(8):400-1. https://doi.org/10.1177/014107680109400807 PMid:11461984 PMCid:PMC1281634
Fautrel B, Zing E, Golmard JL, Le Moel G, Bissery A, Rioux C, Rozenberg S, Piette JC, Bourgeois P. Proposal for a new set of classification criteria for adult-onset still disease. Medicine. 2002 May 1;81(3):194-200. https://doi.org/10.1097/00005792-200205000-00003 PMid:11997716
WOUTERS JM, van de PUTTE LB. Adult-onset Still's disease; clinical and laboratory features, treatment and progress of 45 cases. QJM: An International Journal of Medicine. 1986 Nov 1;61(2):1055-65. https://doi.org/10.1093/oxfordjournals.qjmed .
Appenzeller S, Castro GR, Costallat LT, Samara AM, Bértolo MB. Adult-onset Still disease in southeast Brazil. JCR: Journal of Clinical Rheumatology. 2005;11(2):76-80. https://doi.org/10.1097/01.rhu.0000158544.38663.0a PMid:16357707
Huang SH, DeCoteau WE. Adult-onset Still's disease: an unusual presentation of rubella infection. Canadian Medical Association Journal. 1980;122(11):1275. PMID: 7388725
Lakshman H, Athwal PS, Gondi A, Dhillon S, Towfiq BA, Towfiq BA. A Case of Adult-Onset Still's Disease with Positive Antinuclear Antibodies. Cureus. 2020 ;12(10). https://doi.org/10.7759/cureus.10761
Published



How to Cite
Issue
Section
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution-NonCommercial 4.0 International (CC BY-NC 4.0). that allows others to share the work with an acknowledgment of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgment of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access).