Pediatric rheumatology

Development of Autoimmune Diseases Among Children With Pediatric Acute-Onset Neuropsychiatric Syndrome

JAMA Netw OpenPublished Retrospective cohort study

Authors' conclusion

This study found that patients with PANS show signs of immune activation and vasculopathy during psychiatric symptom flares and have an increased risk of developing arthritis and other autoimmune diseases compared with the general pediatric population. The most common arthritis subtype was enthesitis-related arthritis.

Study context

Population
Consecutive children with PANS followed up within the Stanford Immune Behavioral Health Clinic, September 1, 2012, to December 31, 2021112 boys [58.0%]
Participants
193 children
Outcome
Immune activation and vasculopathy markers during a flare; cumulative risk of arthritis and other autoimmune diseases
Methodological detailsconsecutive patients, single clinic, Kaplan-Meier survival probability
  • Consecutive patients
  • Single clinic
  • Kaplan-Meier survival probability

Main finding

As reported in the abstract

Nonspecific markers of autoimmunity (97 of 179 tested)
54.2%
Nonspecific signs of immune dysregulation or inflammation (22 of 184 tested)
12.0%
Signs of vasculopathy (69 of 193)
35.8%
Estimated cumulative incidence of arthritis by 14 years of age
28.3% (95% CI, 20.8%-36.3%)
Estimated cumulative incidence of another autoimmune disease by 14 years of age
7.5% (95% CI, 4.0%-12.4%)

Observational data: associations, not effects.

Further findings

Clinical findings in subgroup with arthritis

Joint capsule thickening (22 of 40)
55.0%
Distal interphalangeal joint tenderness (45 of 55)
81.8%
Spinous process tenderness (44 of 55)
80.0%

Arthritis subtypes among the 55 patients with arthritis

Enthesitis-related arthritis (37)
67.3%
SpA (27)
49.1%
PsA (10)
18.2%

Original publication

Development of Autoimmune Diseases Among Children With Pediatric Acute-Onset Neuropsychiatric Syndrome. JAMA Netw Open. Published 1 July 2024. doi:10.1001/jamanetworkopen.2024.21688

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