Table A1.
Use of Nonsteroidal Anti-Inflammatory Drugs in Pediatric Acute-Onset Neuropsychiatric Syndrome
| Indication: Use in patients with mild impairment. |
| Administration: Take with food, milk, or antacid to decrease GI adverse effects. |
| Precautions: Use sunscreen concurrently with NSAIDs. Maintain a well hydrated state. Discontinue if patient restricts fluids or has risk of dehydration for other reasons (intense sports in hot weather). Do not take concurrently with ethanol or other liver toxic medications. Use with caution if patient is on corticosteroids. Do not use while patient is on high-dose corticosteroids. Consider temporary discontinuation if patient develops viral gastroenteritis. Do not use if patient has moderate-to-severe swallow dysfunction because of risk of esophageal erosion if NSAID is not properly swallowed. |
| Adverse effects: CNS (drowsiness, dizziness, and blurred vision); GI (nausea, gastritis, esophageal erosion, gastrointestinal reflux disease, constipation, diarrhea, decreased appetite, and rectal bleeding; elevated liver enzymes); skin (photophobic reactions including pseudoporphyria skin rash and sun sensitivity); psychiatric symptoms (anxiety, depression, fatigue, and nervousness); hematology (epistaxis, hematuria, hematoma, and rectal hemorrhage); and cardiovascular (hypertension). |
| Monitoring: Periodic trials off of NSAIDs every 6 weeks. If a patient repeatedly deteriorates when NSAID is discontinued, it can be restarted and continued in the long term with continued trials off (every 1.5–6 months) or do a trial of corticosteroids to abort PANS flare. Laboratory work every 3–6 months if patient is on NSAIDs continuously: liver enzymes, BUN, creatinine, CBC with differential, and UA. |
| Mechanism: Inhibits prostaglandin synthesis by decreasing the activity of cyclooxygenase, which results in decreased formation of prostaglandin precursors. NSAIDs have antipyretic, analgesic, and anti-inflammatory properties. NSAIDs may also have immunomodulatory effects by decreasing the following immune responses: T cell proliferation and the production of proinflammatory cytokines (Iniguez et al. 1999), the Th17 response (Napolitani et al. 2009), and microglial activation (Mackenzie and Munoz 1998). It may also decrease blood–brain barrier permeability (Candelario-Jalil et al. 2007). |
| Dosage | Preparation | Consideration | |
|---|---|---|---|
| (1) Ibuprofen | 10 mg/kg every 6–8 hours (maximum 600 mg/dose) | Tablet, chewable, capsules, or liquid. | Requires frequent dosing to maintain continuous anti-inflammatory action. Available OTC. Liquid and chewable preparations taste better than naproxen. |
| (2) Naproxen | 10 mg/kg every 12 hours (maximum 500 mg/dose) | Tablets, capsules, or liquid. | Naproxen is a potent long-acting NSAID that only requires twice daily dosing. Generally tolerated by children. Liquid formulation available as prescription (250 mg/5 mL) but the taste is often intolerable. |
| (3) Sulindac | 2–4 mg/kg·day every 12 hours; maximum 6 mg/kg·day; do not exceed 400 mg/day | Tablets; can be compounded into a suspension. | Sulindac is equal in potency to naproxen and is also long acting. It may have fewer GI side effects. |
| (4) Celecoxib | 10–25 kg: 50 mg twice a day >25 kg: 100 mg twice a day | Capsules; can be compounded into a suspension. | Fewer GI side effects. Less potent than naproxen and sulindac but helpful if patient develops gastritis symptoms on other NSAIDs. |
BUN, blood urea nitrogen; CBC, complete blood count; CNS, central nervous system; GI, gastrointestinal; NSAIDs, nonsteroidal anti-inflammatory drugs; OTC, over-the-counter; PANS, pediatric acute-onset neuropsychiatric syndrome; UA, urine analysis.