What is the treatment for Still's disease (Juvenile Rheumatoid Arthritis)?

From the Guidelines

The treatment for Still's disease, also known as Juvenile Rheumatoid Arthritis, should prioritize the use of IL-1 or IL-6 inhibitors as early as possible, with the goal of achieving clinically inactive disease (CID) and minimizing the use of glucocorticoids (GCs). This approach is supported by the most recent and highest quality study, which emphasizes the importance of early initiation of IL-1 or IL-6 inhibitors in achieving high levels of rapid GC-free CID 1.

The primary treatment involves anti-inflammatory medications, starting with high-dose nonsteroidal anti-inflammatory drugs (NSAIDs) like naproxen (500mg twice daily) or indomethacin (25-50mg three times daily). For more severe cases, corticosteroids such as prednisone (0.5-1mg/kg/day) are often necessary, but their use should be limited to avoid prolonged systemic GC use 1.

Disease-modifying antirheumatic drugs (DMARDs) like methotrexate (15-25mg weekly) or hydroxychloroquine (200-400mg daily) may be added for steroid-sparing effects, but their efficacy in Still's disease is limited 1. For refractory cases, biologic agents targeting specific inflammatory pathways are used, including IL-1 inhibitors (anakinra 100mg daily subcutaneously), IL-6 inhibitors (tocilizumab 8mg/kg IV every 4 weeks), or TNF inhibitors (etanercept 50mg weekly subcutaneously) 1.

Treatment duration varies based on disease activity, with some patients requiring lifelong therapy while others achieve remission. Regular monitoring of inflammatory markers (ESR, CRP), liver function, and complete blood counts is essential during treatment. This comprehensive approach targets the underlying immune dysregulation in Still's disease, reducing the characteristic high fevers, rash, joint pain, and systemic inflammation that characterize this rare inflammatory disorder.

Key considerations in the treatment of Still's disease include:

• Early initiation of IL-1 or IL-6 inhibitors to achieve rapid GC-free CID 1
• Limiting the use of GCs to avoid prolonged systemic use 1
• Monitoring for severe complications, such as macrophage activation syndrome (MAS) and lung disease 1
• Adjusting treatment based on disease activity and response to therapy 1

By prioritizing the use of IL-1 or IL-6 inhibitors and minimizing the use of GCs, patients with Still's disease can achieve improved outcomes and reduced morbidity.

From the FDA Drug Label

Methotrexate is indicated in the management of selected adults with severe, active rheumatoid arthritis (ACR criteria), or children with active polyarticular-course juvenile rheumatoid arthritis, who have had an insufficient therapeutic response to, or are intolerant of, an adequate trial of first-line therapy including full dose non-steroidal anti-inflammatory agents (NSAIDs). The efficacy of ACTEMRA for the treatment of active SJIA was assessed in WA18221 (NCT00642460), a 12-week randomized, double blind, placebo-controlled, parallel group, 2-arm study Patients treated with or without MTX, were randomized (ACTEMRA:placebo = 2:1) to one of two treatment groups: 75 patients received ACTEMRA infusions every two weeks at either 8 mg per kg for patients at or above 30 kg or 12 mg per kg for patients less than 30 kg and 37 were randomized to receive placebo infusions every two weeks

The treatment for Still's disease (Juvenile Rheumatoid Arthritis) includes:

• Methotrexate (PO): indicated in the management of selected adults with severe, active rheumatoid arthritis, or children with active polyarticular-course juvenile rheumatoid arthritis 2
• Tocilizumab (IV): efficacy assessed in a 12-week randomized, double-blind, placebo-controlled study for the treatment of active Systemic Juvenile Idiopathic Arthritis (SJIA) 3 Key points:
• Methotrexate may be used in combination with other therapies, such as NSAIDs and/or low-dose steroids
• Tocilizumab may be used with or without methotrexate
• The dosage of tocilizumab varies based on patient weight, with 8 mg/kg or 12 mg/kg infusions every two weeks for patients at or above 30 kg or less than 30 kg, respectively 3

From the Research

Treatment Options for Still's Disease (Juvenile Rheumatoid Arthritis)

• Non-steroidal anti-inflammatory drugs (NSAIDs) are commonly used to treat Still's disease 4, 5, 6
• Corticosteroids are also used to treat the disease, sometimes in combination with immunosuppressive agents 4, 5, 6
• Disease-modifying anti-rheumatic drugs (DMARDs) such as methotrexate are used to treat the disease 4, 5, 7
• Biologic agents such as tumor necrosis factor (TNF)-α, interleukin (IL)-1, and IL-6 antagonists have been shown to be effective in treating patients with Still's disease who are refractory to corticosteroids or conventional DMARDs 4, 5, 7, 8
• Tocilizumab, a humanized anti-IL-6 receptor antibody, has been shown to be effective in treating adult-onset Still's disease (AOSD) patients who are refractory to corticosteroids and methotrexate therapy 7
• Paracetamol may be an effective agent in adult-onset Still's disease, as demonstrated in a case report 6

Biologic Therapies

• IL-1 antagonists have been shown to be effective in treating AOSD 4, 5, 8
• IL-6 antagonists, such as tocilizumab, have been shown to be effective in treating AOSD 7, 8
• TNF-α antagonists have been shown to be effective in treating AOSD 4, 5, 8

Treatment Goals

• The goal of treatment is to reduce inflammation, relieve symptoms, and prevent long-term damage 4, 5, 7, 8
• Treatment should be individualized based on the severity of the disease and the patient's response to therapy 4, 5, 7, 8