What is Still’s disease?
Still’s disease is a rare, autoinflammatory disease that ranges from systemic juvenile idiopathic arthritis (SJIA) occurring in childhood to adult-onset Still’s disease (AOSD) in adulthood.1,2
The overlapping clinical features suggest a disease continuum with different ages of onset – both SJIA and AOSD have common pathophysiology, symptoms and response to treatment.1–4
SJIA Disease onset age <16 years
AOSD Disease onset age >16 years
Diagnosing Still’s disease is challenging, because diagnosis is normally based on clinical evaluation, patient history, presence of characteristic findings and excluding of other disorders, rather than specific tests that differentiate Still’s from similar disorders.5
Both juvenile and adult types of the Still’s disease are characterised by inflammatory polyarthritis, daily fever, and a transient salmon-pink maculopapular rash.6,7
What causes Still’s disease?
IL-1β is a critical driver of autoinflammatory diseases such as Still’s disease8,9 where an excessive release of activated IL-1β can cause an inflammatory cascade and a feedback loop that induces the production of more of it.10–12
It is not known what causes overproduction of IL-1β in both the juvenile and adult onset of Still’s disease.6,7
Still’s is believed to be a reactive syndrome in which various environmental factors, including infectious agents, vaccinations, antibiotics, vitamin D deficiency, stress and trauma, may act as triggers in a genetically predisposed host.6,7
Who does Still’s disease affect?
In England, Still’s disease affects between 400 and 800 adults and approximately 1000 children.5
More females are affected by Still’s disease than males.7
The first peak of AOSD is between 15 and 25 years of age and the second is between 36 and 46 years.7
Most patients report the onset of disease between 16 and 35 years of age.7
SJIA affects children under 16 years old, with onset usually between 3 and 5 years old.5
SJIA resolves before adulthood in half of all patients.5
How does Still’s disease manifest?
Symptoms of Still’s disease can develop rapidly or over time and are highly variable between individuals.5
75 to 95% patients experience:5,7
Daily fever which usually peaks in the late afternoon or early evening.
Joint pain, muscle pain and swelling (commonly in the knees, wrists and ankles).
Pink rash.
Other common symptoms7
lymphadenopathy
myalgia
splenomegaly
pharyngitis
Less commonly-observed symptoms7
hepatomegaly
pleurisy
pericarditis
abdominal pain
SJIA6
- Patients experience arthritis of the joints and extra-articular structures, including eyes, skin, and internal organs
- SJIA can lead to disability and can be fatal in some cases
- There are four subtypes of SJIA: oligoarticular (persistent or extended), polyarticular (RF-negative or RF-positive), systemic (sJIA), psoriatic arthritis and enthesitis-related arthritis, and undifferentiated
AOSD5
- There are two subtypes of AOSD: systemic and arthritis-predominant
- In the systemic form, the main symptoms are acute onset characterised by fever, weight loss and other systemic manifestations
- The arthritis-predominant form is characterised by slow onset and symptoms mostly affect the joints
- A potentially life-threatening complication of AOSD is Macrophage Activation Syndrome, although this is not common5
What is the management goal for patients with Still’s disease?
The goal of treatment for patients with Still’s disease
To induce and maintain remission of symptoms (NICE, 2019)5
AOSD, adult-onset Still’s disease; DMARD, disease-modifying antirheumatic drug; IL-1β, interleuckin-1 beta; NICE, National Institute for Health and Care Excellence; NSAID, non-steroidal anti-inflammatory drug; RF, rheumatoid factor; SJIA, systemic juvenile idiopathic arthritis.
References
- Föll D, et al. J Rheumatology 2020;79:639–648.
- Nigrovic PA. Arthritis Rheumatol 2014;66:1405.
- Vastert SJ, et al. Rheumatology (Oxford) 2019;58:vi9–22.
- Feist E, et al. Clin Exp Rheumatol 2018;36:668–675.
- NICE. Single Technology Appraisal: Anakinra for treating Still’s disease, Final scope. Appendix B. 2019. Available at: https://www.nice.org.uk/guidance/ta685/documents/final-scope [Accessed June 2022].
- Zaripova LN, et al. Pediatric Rheumatology 2021;19:135.
- Bhargava J, Panginikkod S. Still Disease. [Updated 2021 Jul 10]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available at: https://www.ncbi.nlm.nih.gov/books/NBK538345/ [Accessed June 2022].
- Church LD, McDermott MF. Nat Clin Pract Rheumatol 2008;4(1):34–42.
- McGeough MD, et al. J Immunol 2012;189(6):2707–2711.
- Lachmann HJ, et al. Arthritis Rheum 2011;63(2):314–324.
- Dinarello CA, et al. Nat Rev Drug Discov 2012;11(8):633–652.
- Jesus AAD, Mansky AG. Oral Dis 2016;22(7):591–604.