What is the treatment for a Systemic Juvenile Idiopathic Arthritis (SJIA) or Adult-Onset Still's Disease (AOSD) flare?

From the Guidelines

To treat a Systemic Juvenile Idiopathic Arthritis (SJIA) or Adult-Onset Still's Disease (AOSD) flare, start with an IL-1 or IL-6 inhibitor as early as possible, such as anakinra (100mg subcutaneously daily) or tocilizumab (8mg/kg IV every 2-4 weeks), and consider high-dose glucocorticoids (GCs) for patients with high disease severity. The treatment approach is based on the latest recommendations from the EULAR/PReS guidelines for the diagnosis and management of Still's disease, comprising SJIA and AOSD 1.

Key Principles of Treatment

• The goal of treatment is to achieve clinically inactive disease (CID) and remission, with a focus on early use of IL-1 or IL-6 inhibitors and short-duration GCs 1.
• The treatment algorithm proposes a two-part approach, with IL-1 or IL-6 inhibitors as first-line therapy, and GCs for patients with high disease severity or impending macrophage activation syndrome (MAS) 1.
• The use of biologic agents, such as IL-1 inhibitors like anakinra or IL-6 inhibitors like tocilizumab, is recommended for severe or refractory flares, and disease-modifying antirheumatic drugs (DMARDs) like methotrexate may be added for long-term management.

Supportive Measures

• Rest, hydration, and fever management with acetaminophen are important supportive measures during a flare.
• Prompt treatment is crucial to prevent serious complications, including MAS.
• The treatment approach targets the underlying immune dysregulation, particularly the overproduction of pro-inflammatory cytokines like IL-1, IL-6, and TNF-alpha that drive the characteristic fever, rash, joint pain, and systemic inflammation seen in Still's disease.

Expert Center Referral

• Patients who fail to achieve CID off GC after IL-1/IL-6 inhibitor rotation should be considered as difficult-to-treat and referred to an expert center for further management 1.

From the FDA Drug Label

The FDA drug label does not answer the question.

From the Research

Treatment Options for Systemic Juvenile Idiopathic Arthritis (SJIA) or Adult-Onset Still's Disease (AOSD) Flare

• The first line of therapy for AOSD includes glucocorticoids and non-steroidal anti-inflammatory drugs (NSAIDs) 2
• Conventional disease modifying anti-rheumatic drugs (DMARDs) can be effective in some severe and steroid-dependent cases of AOSD, but anti-cytokine agents are often a more suitable alternative 2
• IL-1 and IL-6 blockade have been shown to be effective in treating systemic and articular inflammation of AOSD patients 2, 3, 4, 5

Biologic Agents

• Tocilizumab, a humanized anti-IL-6 receptor antibody, has been shown to be effective in treating AOSD patients refractory to corticosteroids and DMARDs 3, 4
• IL-1 blockade has an excellent safety profile and is often the first choice of biologic treatment for AOSD 2

Treatment Approach

• Early treatment with conventional DMARDs and biologic agents blocking causal cytokines is advocated in patients with severe and recalcitrant clinical manifestations 2
• A treatment approach that targets proinflammatory cytokines, such as interleukin-1β, -18, -6, and tumor necrosis factor-α, may be effective in managing AOSD 5, 6