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Risk factors for poor response to initial first-line immunotherapy and subsequent immunotherapy on prognosis in
Yuhang Li1, Hanyu Luo1, Zhiwei Yu1
1Department of Neurology, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Neurodevelopment and Cognitive Disorders, Children's Hospital of Chongqing Medical University, 2 Zhongshan Rd, Chongqing, 400013, China.
Insights
Pediatric anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis patients with decreased consciousness, autonomic dysfunction, speech problems, severe disability (mRS score ≥4), or under 3 years old are at higher risk for poor response to initial immunotherapy. Subsequent immunotherapy improves outcomes for these patients.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune neurological disorder.
- First-line immunotherapy is crucial, but response rates vary, necessitating identification of predictive factors.
Purpose of the Study:
- To determine factors associated with poor response to initial immunotherapy in pediatric anti-NMDAR encephalitis.
- To evaluate the impact of subsequent immunotherapy on outcomes in non-responsive patients.
Main Methods:
- A monocentric prospective cohort study included 152 pediatric patients diagnosed with anti-NMDAR encephalitis between January 2017 and December 2021.
- Neurological severity was assessed using the modified Rankin Scale (mRS).
Main Results:
- Nearly half (48.7%) of patients showed a poor response to initial immunotherapy.
- Factors predicting poor response included decreased consciousness, autonomic dysfunction/central hypoventilation, speech dysfunction, high mRS score (≥4) before treatment, and age ≤3 years.
- Good responders had significantly better outcomes at 12 months (100% vs. 74.3%).
- Subsequent immunotherapy in poor responders led to improved outcomes compared to those not receiving further treatment.
Conclusions:
- Pediatric anti-NMDAR encephalitis patients presenting with specific clinical features (decreased consciousness, autonomic/speech dysfunction, severe disability, young age) face a higher risk of poor response to initial immunotherapy and worse prognosis.
- Subsequent immunotherapy offers a viable strategy to enhance long-term outcomes for patients with poor initial response.
Objectives:
To identify factors associated with poor response to initial first-line immunotherapy in pediatric patients with anti-NMDAR encephalitis.
Methods:
This monocentric prospective cohort study included pediatric anti-NMDAR encephalitis between January 2017 and December 2021. The modified Rankin Scale (mRS) score was used to assess neurological severity.
Results:
This study included 152 patients, 74 of them (48.7%) had a poor response to initial first-line immunotherapy. Decreased consciousness (p = 0.001, OR = 6.889), autonomic dysfunction/central hypoventilation (p = 0.003, OR = 4.704), speech dysfunction (p = 0.007, OR = 4.272), mRS score before immunotherapy > = 4 points (p < 0.001, OR = 10.968), and age < = 3 years (p = 0.044, OR = 5.169) significantly affected the response to initial first-line immunotherapy. At 12 months, the good responders demonstrated a significantly better outcome than did the poor responders (100% vs. 74.3%, p < 0.001), although the recurrence rate was comparable between the two group (11.5% vs. 14.9%, p = 0.544). Of the 74 poor responders, 45 patients underwent subsequent immunotherapy, and they exhibited a greater proportion of favorable outcomes compared to the group without subsequent immunotherapy at 12 months.
Conclusion:
Pediatric anti-NMDAR encephalitis with decreased consciousness, autonomic dysfunction/central hypoventilation, speech dysfunction, mRS score > = 4 points before immunotherapy, and age < = 3 years had a greater risk of poor response to initial first-line immunotherapy, and poorer prognosis. Subsequent immunotherapy can improve long-term prognosis for poor responders.
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