From the Guidelines
The optimal therapeutic strategy for Still's disease relies on early use of interleukin (IL-1 or IL-6 inhibitors associated with short duration glucocorticoid (GC) to achieve clinically inactive disease (CID) and remission. Still's disease, also known as adult-onset Still's disease (AOSD) or systemic juvenile idiopathic arthritis (sJIA) in children, is a rare inflammatory disorder characterized by high spiking fevers, salmon-pink rash, and joint pain. Treatment typically begins with nonsteroidal anti-inflammatory drugs (NSAIDs) like naproxen (500mg twice daily) or ibuprofen (600-800mg three times daily) for mild cases. For moderate to severe disease, corticosteroids such as prednisone (0.5-1mg/kg/day) are often necessary, as evidenced by studies showing that most patients with AOSD will need treatment with corticosteroids at some point in their disease course 1.
Some key points to consider in the management of Still's disease include:
- The use of IL-1 inhibitors, such as anakinra 100mg daily subcutaneously, which has been shown to be effective in refractory cases 1
- The use of IL-6 inhibitors, such as tocilizumab 8mg/kg IV every 4 weeks, which has been shown to be effective in achieving remission 1
- The importance of early diagnosis and aggressive treatment to prevent complications such as joint destruction, macrophage activation syndrome, and amyloidosis
- The need for regular monitoring of disease activity and medication side effects, including complete blood counts, liver function tests, and inflammatory markers (ESR, CRP, ferritin)
According to the most recent guidelines, the therapeutic targets for Still's disease are clinically inactive disease (CID) and remission, which is defined as CID maintained for at least 6 months 1. The guidelines also emphasize the key role of expert centers for difficult-to-treat patients and the importance of a multidisciplinary approach to management. Overall, the goal of treatment is to achieve remission and prevent long-term complications, and the choice of therapy should be individualized based on the severity of disease and the patient's response to treatment.
From the Research
Definition and Treatment of Still's Disease
- Still's disease, also known as Adult-Onset Still's Disease (AOSD), is a rare systemic inflammatory condition characterized by persistent high spiking fever, evanescent rash, and polyarthritis 2.
- The treatment of AOSD varies from glucocorticoids to disease-modifying antirheumatic drugs (DMARDs), both conventional synthetic and biological (bDMARDs) 3.
First-Line Treatment Options
- Glucocorticoids and non-steroidal anti-inflammatory drugs (NSAIDs) represent the first line of therapy for AOSD, with add-on therapy with second-line drugs reserved for steroid-dependent patients and in life-threatening cases 4.
- Conventional DMARDs can be effective in some severe and steroid-dependent cases of AOSD; however, anti-cytokine agents represent an effective and overall more suitable alternative in this specific subset of patients 4.
Biologic Agents and Treatment
- IL-1 and IL-6 inhibitors are frequently used in the treatment of AOSD, as they have been shown to be effective in treating systemic and articular inflammation 3, 5.
- IL-1 blockade has an excellent safety profile and represents the first choice of biologic treatment in AOSD patients 4.
- Tocilizumab, a humanized anti-IL-6 receptor antibody, has been shown to be effective in treating AOSD patients refractory to corticosteroids and methotrexate therapy 6.
Future Perspectives and Unmet Needs
- Innovative therapeutic strategies have suggested an early administration of bDMARDs to increase the rate of clinical response and drug-free remission 3.
- New targets have been proposed, including IL-18, IFN-γ, and JAK/STAT pathway, which could be applied to Still's disease and its life-threatening evolution 3.
- A better patient stratification may help in arranging specific studies to improve the long-term outcome of Still's disease 3.