What is Adult-Onset Still's Disease (AOSD)?

From the Guidelines

The most effective treatment for Still's disease is early use of interleukin (IL-1 or IL-6 inhibitors associated with short duration glucocorticoid (GC), with a goal of achieving clinically inactive disease (CID) and remission. This approach is supported by the most recent evidence from the EULAR/PRES recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease 1. The use of IL-1 inhibitors, such as anakinra, has been shown to be effective in treating refractory cases of Still's disease, with rapid improvement in symptoms and laboratory values 1. Additionally, IL-6 inhibitors, such as tocilizumab, have also been used successfully in treating Still's disease, particularly in cases with high levels of IL-6 1.

Some key points to consider in the treatment of Still's disease include:

• Early diagnosis and aggressive treatment are crucial to prevent complications like joint destruction, macrophage activation syndrome, and amyloidosis
• The use of nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids may be necessary for mild to moderate cases, but biologic therapies targeting specific inflammatory pathways are often required for refractory cases
• Patients should be monitored regularly for disease activity and medication side effects, including complete blood counts, liver function tests, and inflammatory markers (ESR, CRP, ferritin)
• The disease is thought to result from dysregulation of the innate immune system, with excessive production of pro-inflammatory cytokines like IL-1, IL-6, and TNF-α

In terms of specific treatment regimens, the following may be considered:

• IL-1 inhibitors, such as anakinra 100mg daily subcutaneously
• IL-6 inhibitors, such as tocilizumab 8mg/kg IV every 4 weeks
• Glucocorticoids, such as prednisone 0.5-1mg/kg/day, for short durations
• Disease-modifying antirheumatic drugs (DMARDs), such as methotrexate 15-25mg weekly, as steroid-sparing agents.

From the Research

Treatment Options for Still's Disease

• The primary treatment for Adult-Onset Still's Disease (AOSD) includes glucocorticoids and non-steroidal anti-inflammatory drugs (NSAIDs) as the first line of therapy 2.
• Conventional disease-modifying anti-rheumatic drugs (DMARDs) and biologic agents can be used as add-on therapy for patients with severe and recalcitrant clinical manifestations 2, 3.
• Biologic agents, such as IL-1 and IL-6 inhibitors, have shown effectiveness in treating systemic and articular inflammation in AOSD patients 2, 4.

Efficacy of Biologic Agents

• IL-1 inhibitors are more effective for systemic manifestations, while IL-6 inhibitors are effective for both joint disease and systemic features 4.
• Anti-TNF agents may be useful for patients with the pure rheumatoid subgroup 4.
• The overall rate of clinical response to biologic drugs in AOSD patients is 0.85, and the overall rate of complete remission is 0.66 5.

Patient Characteristics and Treatment

• Patients with chronic disease course and younger age are more likely to be treated with biologic DMARDs (bDMARDs) 6.
• The physicians' prescribing motivations for bDMARDs include inadequate response to conventional therapy, corticosteroid-sparing effect, and occurrence of macrophage activation syndrome (MAS) 6.
• Approximately 60-70% of AOSD patients develop a chronic form, and up to 20-30% of patients who are refractory to conventional therapy may require biologic agents 4.