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Adult-onset Still's disease

Definition
ASD is defined as a rare, polygenetic, autoinflammatory disorder.
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Pathophysiology
The exact pathophysiology of ASD is unknown, but it is characterized by overactive immune responses and excessive production of pro-inflammatory cytokines such as TNF-α, IL-1, IL-6, interleukin-8, and IL-18.
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Epidemiology
The incidence of ASD worldwide is estimated at 0.16-0.4 per 100,000 person-years.
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Disease course
Clinically, ASD presents with a variety of symptoms. Common symptoms include fever, rash, arthralgia, arthritis, throat pain, lymphadenopathy, and myalgia. Less common symptoms include pleuritis, pericarditis, and abdominal pain. Arthralgias are very common (> 80%) and arthritis is common (> 50%). The most commonly involved regions are the knees, ankles, and wrists. Systemic complications may develop that include lung involvement and perimyocarditis, which are associated with an unfavorable prognosis.
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Prognosis and risk of recurrence
The prognosis of ASD is variable and can be influenced by several factors. For instance, an elevated ESR and refractoriness to corticosteroids have been associated with poor prognosis.
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Key sources
The following summarized guidelines for the evaluation and management of adult-onset Still's disease are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023), the German Society of Rheumatology (DGRh 2022), the European League Against Rheumatism (EULAR 2022), the National Institute for Health and Care Excellence (NICE 2021), the Adult-onset Still's Disease Consensus Group (AOSD-CG 2019), and the Ministry of Health, Labour and Welfare of Japan (MHLW 2018).
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Guidelines

1.Screening and diagnosis

Definition: as per DGRh 2022 guidelines, recognize that ASD is a rare, polygenetic, autoinflammatory disorder.
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  • Clinical presentation

  • Diagnostic criteria

2.Diagnostic investigations

Laboratory testing
As per DGRh 2022 guidelines:
Measure serum ferritin levels for diagnosis and follow-up of patients with ASD and to assess disease activity in conjunction with markers of inflammation, such as CRP. Recognize that a markedly elevated serum ferritin level (≥ 5× ULN) and, if available, markedly reduced fraction of glycosylated ferritin (< 20%) further support the diagnosis of ASD.
B
Consider measuring IL-18 levels to substantiate the diagnosis and disease activity in patients with ASD.
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  • Evaluation for complications

  • Pretreatment evaluation

3.Medical management

General principles
Ensure rheumatological expertise in diagnosing and treating ASD.
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Follow the principles of participatory decision-making within a holistic therapeutic approach including pharmacological therapy alongside accompanying measures such as analgetic and physical therapy, rehabilitative measures, functional training, and the involvement of patient support groups for the treatment of ASD.
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  • NSAIDs

  • Corticosteroids

  • DMARDs

  • Biologics

4.Preventative measures

Routine immunizations: consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in ≥ 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications.
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