Adult-onset Still disease is a rare multisystem inflammatory disorder.
Still disease in adults is rare affecting around 1.5 per 100,000 people, it occurs in a bimodal distribution with one peak around the age of 15-25 years old and another around the age of 35-45 years old 1. It affects females more than males 2.
Adult-onset Still disease classically is described as a triad of:
- high fevers
- salmon-colored rash
Less common are:
Elevated inflammatory markers (e.g. CRP), leukocytosis, thrombocytosis and elevated ferritin are common 3.
The cause of adult-onset Still disease is unknown but genetic, environmental and infectious factors may play a role 3,4. Elevated levels of various cytokines are seen in adult-onset Still disease. Interleukin-18-induced activation of macrophages and neutrophils appears to be important in Still disease. Interleukin-1-β also seems to be important in Still disease causing neutrophil production and diapedesis 5.
The Yamaguchi criteria is probably the most commonly used and sensitive scoring system for diagnosing adult-onset Still disease 6.
Adult-onset Still disease is a seronegative disease meaning a negative rheumatoid factor and anti-nuclear antibodies are commonly found.
Pericapitate and trapezoid-metacarpal joint space narrowing without radiocarpal involvement is characteristic 7. This pattern is rarer in rheumatoid arthritis.
Treatment and prognosis
Steroids are commonly used to achieve remission of acute disease. Disease modifying anti-rheumatic drugs may be used to maintain remission 3.
Three main patterns of disease have been described:
- single acute episode before a period of lifelong remission
- acute symptomatic episodes separated by months-years of no symptoms
- chronic articular disease causing joint destruction 3
History and etymology
Adult-onset Still disease was described first in 1971, by the renowned British rheumatologist Eric G L Bywaters (1910-2003) 8, who thought the disease resembled systemic juvenile idiopathic arthritis (pediatric Still disease) 1.
- 1. Bywaters EG. Still's disease in the adult. (1971) Annals of the rheumatic diseases. 30 (2): 121-33. Pubmed
- 2. Owlia MB, Mehrpoor G. Adult-onset Still's disease: a review. (2009) Indian journal of medical sciences. 63 (5): 207-21. doi:10.4103/0019-5359.53169 - Pubmed
- 3. Gopalarathinam, Rajesh, Orlowsky, Eric, Kesavalu, Ramesh, Yelaminchili, Sreeteja. Adult Onset Still’s Disease: A Review on Diagnostic Workup and Treatment Options. (2018) Case Reports in Rheumatology. 2016: 6502373. doi:10.1155/2016/6502373 - Pubmed
- 4. Perez C, Artola V. Adult Still's disease associated with Mycoplasma pneumoniae infection. (2001) Clinical infectious diseases : an official publication of the Infectious Diseases Society of America. 32 (6): E105-6. doi:10.1086/319342 - Pubmed
- 5. Chen DY, Lan JL, Lin FJ, Hsieh TY. Proinflammatory cytokine profiles in sera and pathological tissues of patients with active untreated adult onset Still's disease. (2004) The Journal of rheumatology. 31 (11): 2189-98. Pubmed
- 6. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T. Preliminary criteria for classification of adult Still's disease. (1992) The Journal of rheumatology. 19 (3): 424-30. Pubmed
- 7. Björkengren AG, Pathria MN, Sartoris DJ, Terkeltaub R, Esdaile JM, Weisman M, Resnick D. Carpal alterations in adult-onset Still disease, juvenile chronic arthritis, and adult-onset rheumatoid arthritis: comparative study. (1987) Radiology. 165 (2): 545-8. doi:10.1148/radiology.165.2.3659381 - Pubmed
- 8. Dixon A. Obituary: Eric Bywaters 1910-2003. Rheumatology. 2003; 42:1025-1027.