Adult Still's disease

Adult Still's disease is a diagnosis of exlusion in a patient with systemic upset, fever, rash and arthralgia (1).

• is a rare systemic autoinflammatory disease characterized by arthritis, spiking fever, skin rash and elevated ferritin levels (2)
• reason behind the nomenclature of this condition is that adult-onset Still’s disease (AoSD) shares certain symptoms with Still’s disease in children, currently named systemic-onset juvenile idiopathic arthritis (2)

Typically the patient is a young adult, 75% are between 16 and 35, although patients over 60 have been reported. The sexes are affected equally.

The disease is rare, with a prevalence of about 1 in 100,000 (1).

Early initiation of biologics is preferred over conventional treatment such as methotrexate (3).

There is an important role for early use of IL-1 and IL-6 blockade (3).

Reference:

1. Evans, RH et al. Pyrexia of unknown origin. (Grand Round). BMJ 1997;314: 583-6.
2. Tomaras S, Goetzke CC, Kallinich T, Feist E. Adult-Onset Still's Disease: Clinical Aspects and Therapeutic Approach. J Clin Med. 2021 Feb 12;10(4):733.
3. Leavis HL et al. Management of adult-onset Still's disease: evidence- and consensus-based recommendations by experts. Rheumatology (Oxford). 2024 May 3;63(6):1656-1663.