From the Guidelines
The most effective treatment for Still's disease involves the early use of IL-1 or IL-6 inhibitors, such as anakinra or tocilizumab, in combination with short-duration glucocorticoids, to achieve clinically inactive disease and remission. This approach is based on the recent recommendations from the EULAR/PRES guidelines for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease 1. The guidelines emphasize the importance of early treatment with IL-1 or IL-6 inhibitors to control disease activity and limit exposure to glucocorticoids, which can have severe side effects with long-term use.
The treatment strategy for Still's disease should be individualized based on disease severity, with the following key principles:
- Early use of IL-1 or IL-6 inhibitors, such as anakinra 100mg daily or tocilizumab 8mg/kg IV every 2-4 weeks, to achieve clinically inactive disease and remission 1
- Short-duration glucocorticoids, such as prednisone 0.5-1mg/kg/day, to control disease activity and prevent complications 1
- Monitoring of safety and disease activity, with regular assessments of laboratory parameters, such as liver function tests and serum ferritin levels 1
- Consideration of biologic therapies, such as TNF inhibitors, for refractory cases or patients with severe disease 1
The guidelines also emphasize the importance of expert centers for difficult-to-treat patients, and the need for a multidisciplinary approach to manage complications, such as macrophage activation syndrome and severe lung disease 1. Overall, the goal of treatment is to achieve clinically inactive disease and remission, while minimizing the risk of complications and improving quality of life for patients with Still's disease.
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 remains largely empirical and is based on the administration of immunosuppressive drugs, including nonsteroidal anti-inflammatory drugs (NSAIDs), glucocorticoids (GCs), and disease-modifying antirheumatic drugs (DMARDs) 3, 4.
First-Line and Second-Line Therapies
- First-line therapy for AOSD typically includes NSAIDs and GCs, with GCs being the primary treatment administered at the beginning of the disease 3, 4.
- Second-line therapy involves the use of conventional synthetic DMARDs, such as methotrexate (MTX), when GCs do not fully control the disease and/or to reduce the dosage of concomitant GCs 3.
- Calcineurin inhibitors (CNIs) are used in severe cases of AOSD, and biologic DMARDs may be administered when there is a lack of clinical response to non-biological pharmacotherapies 3, 4.
Biologic Agents and Targeted Therapies
- Biologic agents, such as IL-1 and IL-6 blockers, have been shown to be effective in treating systemic and articular inflammation in AOSD patients 4, 2.
- Anti-TNF therapy has also been used in refractory cases of AOSD, with favorable responses reported in some patients 5.
- Intravenous immunoglobulin (IVIG) has been investigated as a potential alternative to classical steroid therapy in patients with AOSD refractory to NSAIDs, with some studies suggesting its efficacy 6.
Efficacy and Safety of Therapies
- Non-biological pharmacotherapies, including GCs, MTX, and CNIs, have shown efficacy in inducing a clinical response, managing life-threatening complications, and may be well tolerated in combination with biologic DMARDs 3.
- Biologic agents, such as IL-1 and IL-6 blockers, have been shown to be effective and overall more suitable alternatives in severe and recalcitrant cases of AOSD 4.
- However, further specific studies are needed to fully clarify the specific role of these drugs in clinical practice and to provide a more tailored treatment for AOSD patients 3, 4.