Pediatric Acute-onset Neuropsychiatric Syndrome: Markers of Inflammation/autoimmunity at Clinical Presentation and Eventual Development of Arthritis and Other Autoimmune Diseases

Meiqian Ma¹, Erin Masteron² and Jennifer Frankovich¹, ¹Stanford University School of Medicine, Palo Alto, CA, ²University of Washington School of Public Health, Seattle, WA

Meeting: ACR Convergence 2023

Keywords: autoimmune diseases, Juvenile idiopathic arthritis, mental health, neuropsychiatric disorders, Ultrasound

Session Information

Date: Tuesday, November 14, 2023

Title: (2039–2060) Pediatric Rheumatology – Clinical Poster III: Potpourri

Session Type: Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Certain psychiatric conditions are associated with systemic inflammatory processes. We aimed to evaluate whether a pediatric condition characterized by a sudden onset of severe neuropsychiatric symptoms, pediatric acute-onset neuropsychiatric syndrome (PANS), is related to inflammatory disease.

Methods: This retrospective, observational analysis is based on longitudinal clinical data from patients with PANS from the Stanford Immune Behavioral Health (IBH) Clinicfrom 2012-2021.We reviewed patient medical records since birth to evaluate immune activation markers during psychiatric flare episodes, arthritis andautoimmune disease diagnoses. We describe laboratory, physical exam, and musculoskeletal ultrasoundresults and estimate the cumulative risk of developing arthritis and autoimmunediseases using product limit (Kaplan-Meier) survival probability.

Results: We analyzed data from 193 children who had a sudden onset of severe neuropsychiatric symptoms at approximately 7.5 ± 3.5 years of age.These patients had been followed in the IBH Clinic for an average of47.9 ± 25.5 months (mean age: 13.9 ± 4.7 yearsat data collection). Among those tested for immune activation markers, 55% had results indicative of autoimmunity, 12% of immune dysregulation, and 45% of vasculopathy (Table 1). By 14 years of age, the estimated cumulative incidence of juvenile-onset arthritis was 28% (95% CI: 21%, 36%) and of autoimmune disease was 8% (95% CI: 4%, 12%).We report novel findings among children with arthritis, including capsular thickening (55%), DIP tenderness (82%), and spinous process tenderness (80%) (Table 2).

Conclusion: Patients with PANS show signs of immune activation during psychiatric flare episodes and have a heightened risk of arthritis and other autoimmune diseases compared to the general pediatric population. These findings suggestPANS may be a systemic inflammatory condition rather than an isolated psychiatric or neuroinflammatory disorder.

Table 1. Non-specific laboratory markers and physical signs of immune activation measured at the first flare captured in clinic among Stanford University Immune Behavioral Health Clinic patients with sudden-onset, severe neuropsychiatric symptoms, N=193

Table 2. Juvenile-onset arthritis, musculoskeletal characteristics, and other autoimmune/autoinflammatory diseases in Stanford University Immune Behavioral Health Clinic patients with sudden-onset, severe neuropsychiatric symptoms, N=193

Disclosures: M. Ma: None; E. Masteron: None; J. Frankovich: None.

To cite this abstract in AMA style:

Ma M, Masteron E, Frankovich J. Pediatric Acute-onset Neuropsychiatric Syndrome: Markers of Inflammation/autoimmunity at Clinical Presentation and Eventual Development of Arthritis and Other Autoimmune Diseases [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/pediatric-acute-onset-neuropsychiatric-syndrome-markers-of-inflammation-autoimmunity-at-clinical-presentation-and-eventual-development-of-arthritis-and-other-autoimmune-diseases/. Accessed .

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