From the Guidelines
The treatment for pediatric Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS) primarily involves IL-1 inhibitors, as recommended by the 2021 EULAR/American College of Rheumatology points to consider for diagnosis, management, and monitoring of IL-1 mediated autoinflammatory diseases 1. The goal of treatment is to reduce inflammation, prevent organ damage, and improve quality of life.
- IL-1 inhibitors, such as anakinra, are considered first-line therapy due to their ability to block excessive IL-1 production, which is a key driver of inflammation in TRAPS.
- The dose of anakinra is typically started at 1-2 mg/kg/day subcutaneously, with adjustments made based on clinical response, as seen in other IL-1 mediated autoinflammatory diseases 1.
- For severe or refractory cases, alternative IL-1 inhibitors like canakinumab may be used, or the dose of anakinra may be increased up to 5 mg/kg/day.
- In addition to IL-1 inhibitors, symptomatic relief during acute flares can be achieved with NSAIDs and short courses of corticosteroids, such as prednisolone 0.5-1 mg/kg/day for 3-5 days.
- Regular monitoring for disease activity, inflammatory markers, and medication side effects is crucial to ensure effective treatment and prevent long-term complications, such as amyloidosis.
- Treatment should be individualized based on disease severity, frequency of attacks, and organ involvement, taking into account the patient's overall quality of life and potential risks associated with each treatment option.
From the Research
Treatment Overview
The treatment for pediatric Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS) typically involves managing the symptoms and preventing the progression of the disease.
- The initial treatment often includes corticosteroids to reduce inflammation and manage acute attacks 2, 3.
- However, long-term use of corticosteroids can have significant side effects, and other treatments may be necessary to control the disease.
Biological Agents
Biological agents, such as anti-interleukin-1 (IL-1) antagonists, have been shown to be effective in treating TRAPS.
- Anakinra, an IL-1 receptor antagonist, has been used to treat patients with TRAPS, including children, and has been shown to be effective in reducing symptoms and preventing disease relapses 4.
- Tocilizumab, an anti-IL-6 receptor monoclonal antibody, has also been used to treat TRAPS, particularly in cases where other treatments have failed 5.
- Etanercept, a TNF receptor superfamily 1B fusion protein, has been studied as a potential treatment for TRAPS, but its effectiveness is still being evaluated 6.
Management and Prognosis
The management of TRAPS typically involves a combination of medications and regular monitoring to prevent disease relapses and reduce the risk of complications, such as secondary inflammatory amyloidosis.
- Regular clinical and biological monitoring is essential in the follow-up of TRAPS patients to adjust treatment as needed and prevent long-term damage 3.
- The prognosis of TRAPS is overall good, but the risk of developing secondary inflammatory amyloidosis is a significant concern, particularly in patients with structural mutations leading to conformation abnormalities of the TNFRSF1A receptor 3.