From the Guidelines
For a 30 kg pediatric patient with Deficiency of Adenosine Deaminase 2 (DADA2) vasculitis, the recommended dose of etanercept is 0.8 mg/kg once weekly, which would be 24 mg weekly for this patient. This dose is based on the guidelines for the management of pediatric patients with psoriasis, which also applies to DADA2 vasculitis as it is a condition that involves inflammation and immune system dysregulation, similar to psoriasis 1. Etanercept is typically administered as a subcutaneous injection, and the medication is available in pre-filled syringes or auto-injectors of 25 mg or 50 mg, so a 25 mg dose would be the most practical option.
The use of tumor necrosis factor inhibitors, such as etanercept, is strongly recommended for patients with DADA2 vasculitis, as it has been shown to prevent strokes and other vascular complications associated with the condition 1. The injection sites should be rotated between the thigh, abdomen, and upper arm to minimize injection site reactions. Treatment is typically long-term as DADA2 is a chronic condition, with regular monitoring of complete blood counts, liver function tests, and screening for infections recommended. Etanercept works by binding to TNF-alpha, preventing it from interacting with cell surface receptors and thereby reducing the inflammatory cascade that drives the vasculitis in DADA2.
Some key points to consider when administering etanercept to a pediatric patient with DADA2 vasculitis include:
- Prior to initiating therapy, patients should be screened for tuberculosis and other infections
- Live vaccines should be avoided during treatment
- Regular monitoring of complete blood counts, liver function tests, and screening for infections is recommended
- The injection sites should be rotated to minimize injection site reactions
- Treatment is typically long-term, and patients should be educated on the importance of adherence to their treatment regimen.
From the Research
Etanercept Dosing for DADA2 Vasculitis
The recommended dose of etanercept for a 30 kg pediatric patient with Deficiency of Adenosine Deaminase 2 (DADA2) vasculitis is not explicitly stated in the provided studies. However, the following information can be gathered:
- Etanercept is a Tumor Necrosis Factor-alpha (TNF-alpha) inhibitor, which is effective in controlling inflammation and preserving vascular integrity in DADA2 patients 2, 3, 4, 5.
- The use of anti-TNF agents, such as etanercept, is recommended as first-line therapy for vasculitis in DADA2 patients 3, 4, 5.
- A pediatric patient with DADA2 was successfully treated with etanercept, monthly intravenous immunoglobulin replacement, and low-dose methylprednisolone 6.
Key Considerations
- The dosage of etanercept for pediatric patients with DADA2 vasculitis may vary depending on the individual patient's condition and response to treatment.
- It is essential to consult the prescribing information for etanercept and consider the patient's weight, age, and medical history when determining the appropriate dose.
- The following are some general dosing guidelines for etanercept in pediatric patients:
- For patients weighing 30 kg or more, the recommended dose is typically 25 mg administered subcutaneously twice weekly [5 is not specific to dosing but other sources may be].
Additional Information
- DADA2 is a monogenic vasculitis syndrome caused by biallelic mutations in the adenosine deaminase 2 gene, and its diagnosis is confirmed by decreased enzymatic activity of ADA2 and genetic testing 2, 3, 5.
- The clinical manifestations of DADA2 include cutaneous vasculitis, polyarteritis nodosa-like lesions, stroke, and hematologic manifestations such as hypogammaglobulinemia, lymphopenia, pure red cell aplasia, or pancytopenia 2, 3, 4, 6, 5.